case_id
stringlengths 12
14
| gender
stringclasses 4
values | age
int8 0
98
| case_text
stringlengths 216
57.5k
| keywords
stringlengths 8
843
⌀ | image_file
imagewidth (px) 136
1.66k
⌀ | caption
stringclasses 584
values |
|---|---|---|---|---|---|---|
PMC6333586_01
|
Male
| 17
|
A 17-year-old male presented with a one-week history of abdominal pain and dysuria. He described the abdominal pain as sharp and localized to the infraumbilical and right inguinal regions. He reported a decrease in appetite. He lost approximately 15 kg over the preceding five months. He had no fever. He had normal bowel movements without blood or mucus. His past medical history was significant for recurrent anterior abdominal wall abscesses. The initial abscess occurred five months prior and he was treated with intravenous (IV) antibiotics at an outside facility. At that institution, computed tomography (CT) of the abdomen was reported as demonstrating significant bowel wall thickening of the TI with adjacent inflammatory stranding. After the antibiotic course was completed, a repeat CT showed resolution of the abdominal wall abscess but persistence of the bowel wall abnormality. Two months later, he developed a second episode of abdominal wall abscess formation that required percutaneous surgical drainage and antibiotic therapy (amoxicillin and metronidazole). Aspirated serous fluid contained few leukocytes and was sterile. After this second episode, he underwent esophagogastroduodenoscopy (EGD) and colonoscopy at the outside institution. The EGD was normal and colonoscopy revealed erythema with ulcerations in the TI. Histopathology confirmed severe chronic inflammation of TI. He was prescribed an oral 5-aminosalicylic acid for inflammatory bowel disease. He was not compliant with taking this medication. His abdominal pain slightly improved for a brief period of time. A follow-up colonoscopy was planned at the other institution but he was admitted to our hospital with the current presenting symptoms.
On admission, his vital signs were within normal limits. Physical examination revealed a soft, non-distended abdomen with mild tenderness in the infraumbilical and right inguinal regions. A small area of erythema and induration, approximately 2 cm in diameter, was present just inferior to the umbilicus. There was minimal guarding but no rigidity or rebound. No umbilical discharge was noted. Laboratory evaluation demonstrated elevated inflammatory markers and normal hemoglobin, platelet count, albumin, and urinalysis. The CT of the abdomen revealed a rim enhancing ovoid shaped fluid collection in the midline of the suprapubic region (just below the umbilicus) measuring 10.5x3x2.5 cm (Fig. 1A). This abscess cavity contained multiple small foci of gas tracking from the umbilicus to the superior-ventral aspect of the urinary bladder wall. Contiguous with the ovoid fluid collection and just lateral to the right rectus abdominis muscle, there was a smaller abscess approximately 1.5 cm in diameter. The CT also revealed significant thickening of the wall of the distal ileum that was in close contiguity with the urachal remnant suggesting the presence of an enterourachal fistula (Fig. 1B). The prior CT performed at the outside hospital at the time of initial presentation was reviewed at our institution and revealed a patent urachus at the same location of the current ovoid shaped abscess (Fig. 1C). He was started on IV piperacillin-tazobactam. He underwent EGD and colonoscopy. EGD was normal. Colonoscopy revealed severe erythema and ulceration in the TI and colon appeared normal. Biopsies from TI revealed chronic severe ileitis with architectural distortion. The colonic biopsies were normal. Magnetic resonance enterography (MRE) confirmed the CT scan findings and clearly demonstrated a fistula from the TI to the urachal remnant abscess (Fig. 1D). Quantiferon-TB gold test was negative and chest radiograph was normal. He was diagnosed with fistulizing CD based on the endoscopic, histologic, and MRE findings. Induction therapy for CD with prednisone was initiated. Percutaneous drainage of the abscess was performed. The aspirated fluid was purulent with neutrophilic predominance and no organism was cultured. He completed two weeks of IV piperacillin-tazobactam and two weeks of oral ciprofloxacin and metronidazole therapy. At the completion of the antibiotic course, a CT scan with sinogram (contrast administered through the drainage catheter) showed no residual fluid collection and no significant filling of the abscess cavity with contrast. The drainage catheter was removed. Repeat MRE showed interval improvement of the TI thickening, complete resolution of the anterior abdominal abscesses, and a blind-ended fistula from the TI without communication to the urachal remnant. His inflammatory markers slowly normalized. Prednisone was gradually weaned and he was started on azathioprine for maintenance therapy. He was referred for a colorectal surgical consultation and underwent laparoscopic exploration of the abdomen. The old abscess cavity was identified and noted to be tightly adherent to the anterior abdominal wall and in close proximity to the inflamed TI. Ileocecectomy with primary anastomosis and complete resection of the abscess cavity was performed. The enterourachal fistula could not be identified and likely obliterated with the medical management. Resection of the urachal remnant could not be simultaneously done due to dense adhesions. Histopathology of the resected specimen confirmed active CD. A repeat colonoscopy ten months after the surgery revealed the CD in remission. He had no recurrence of abscesses during the follow up. A complete resection of the urachal remnant has been planned.
|
crohn disease, inflammatory bowel diseases, intestinal fistula, urachus
| Not supported with pagination yet
| null |
PMC5516656_01
|
Male
| 8
|
Henoch-Schonlein purpura (HSP), which is the most common form of childhood vasculitis, affects mostly skin, joints, gastrointestinal system (GIS), kidneys, and in addition more rarely can have effect on central nervous system (CNS), heart and scrotum. Its pathogenesis and causal factors have not been accurately identified yet. Viral and bacterial infections, drugs, vaccines, food allergy and even insect bites have been considered in the etiology of HSP. Streptococcus, vaccines, viral infections (varicella, measles, rubella, hepatitis A, B), tuberculosis, mycoplasma, Bartonella, helicobacter pylori are stated as the triggering factors in the literature. Epstein-Barr virus (EBV) infection associated with HSP cases have been reported rarely. Herewith we report the case of an 8-year old boy with primary active Epstein Barr Virus infection triggering HSP.
|
epstein-barr virus, henoch-schönlein purpura, children
| Not supported with pagination yet
| null |
PMC3891081_01
|
Female
| 28
|
A 28-year-old woman in her 24th week of pregnancy visited our hospital experiencing febrile sensation and palpitation. The woman was diagnosed with hyperthyroidism 10 years earlier and had taken thionamide periodically. She had not taken any medication recently because she did not display any signs or symptoms of hyperthyroidism. At the 20th week of gestation, she had a relapse of hyperthyroidism and resumed taking the thionamide (propylthiouracil 300 mg) that she had received at a local clinic. At that time, her blood count was within the normal range.
At her initial visit to the hospital, her blood pressure was 110/70 mmHg, pulse rate 110/min,respiratory rate 23/min and body temperature 39C. Her height and body weight were 163 cm and 55 kg. On physical examination, the patient was alert, her heart rhythm was regular and there was no sign of a murmur. She had a diffuse goiter and mild ophthalmopathy, and there was no evidence of any upper respiratory tract infection.
Her full blood count showed total WBCs at 1,600/mm3 with neutrophils 213/mm3 and lymphocytes 963/mm3, platelets 233x103/mm3 and hemoglobin 9.1 g/dL. Level of free T4 was 5.7 ng/mL (0.7~1.9), total T3 was 6.51 ng/mL (0.6~1.7), and TSH was<0.005 mU/mL (0~4.7). TBII (TSH-binding inhibitory immunoglobulin) was 61% (< 14.9%). AST/ALT were 49/47 IU/L and BUN/Creatinine were 12/1.0 mg/dL. An EKG showed sinus tachycardia and chest X-rays were normal. A fetal ultrasound examination revealed a normal sized fetus for the gestation period. The fetal heart rate was 160 beats/min and a non-stress test showed a reactive response.
Following the discontinuation of propylthiouracil, we began intravenous antibiotics (cephalosporin) and hydration (5% sodium dextrose fluid). Due to the possibility of teratogenecity, we excluded the use of G-CSF (granulocyte-colony stimulating factor). The patient's neutrophil count recovered to normal range with supportive care and her fever resolved after 10 days, yet her symptoms of hyperthyroidism were aggravated. At this point it was difficult to choose the optimal treatment method which would save both the mother and fetus. After considering all treatment options, it was decided that a total thyroidectomy rather than a subtotal thyroidectomy was the better option in this case, in order to prevent any future relapse. We also prescribed a beta-adrenergic blocker (propranolol 80 mg/day) and a Lugol solution (5% Lugol solution 1.2 mL/day) because a thyroidectomy without preoperative management was viewed to be too risky due to the high risk of thyrotoxic crisis. The treatment consisting of the beta-adrenergic blocker and the Lugol solution led to a rapid decline ofserum free T4 and total T3 concentration back to their normal ranges within 2 week (Figure 1). At 28 weeks of gestation, the patient underwent a total thyroidectomy without complications. Thyroxine replacement began on the first day following the operation and the patient's thyroid hormone levels were normalized after levothyroxine (synthyroid) 0.2 mg replacement.
The maternal TBII level at 38 weeks of gestation decreased to 23% and labor was induced at 40 weeks. A 3,370 g healthy male infant was born without any clinical features of thyrotoxicosis.
| null | Not supported with pagination yet
| null |
PMC10244928_01
|
Male
| 13
|
Our index case is a 13-year-old male from a rural area in who acquired HIV through vertical transmission. The mother tested negative during antenatal care, but she was not tested at delivery. He was diagnosed with HIV and pulmonary tuberculosis in 2010, started tuberculosis treatment and later on ART. As per the national guidelines, abacavir, 3TC and ritonavir-boosted lopinavir (LPV/r) syrups were initiated but it is unclear if he received ritonavir super-boosting during his tuberculosis treatment to overcome the inducing effect of rifampicin. He was diagnosed with tuberculosis again in 2013, again without documentation that LPV/r was super-boosted with ritonavir during treatment. Since ART initiation, he has never been virally suppressed, despite ongoing enhanced adherence support.
Figure 1 shows the HIV viral load (VL) and CD4 count timeline after ART initiation with no evidence of viral suppression defined as < 50 copies/mL. HIV VL is in log10 copies/mL (right vertical axis) and CD4 count is in cells/microL (left vertical axis). HIV VL >= 1000 copies/mL (>= 3 log10 copies/mL) is defined as virological failure.
In 2018 HIV drug resistance (DR) genotyping was performed, showing only M184V mutation with no protease resistance mutations, which suggested suboptimal adherence. He was switched to zidovudine (AZT) and 3TC, and was started on LPV/r paediatric tablets. The patient received continuous enhanced adherence counselling by the attending doctor and via the social worker. In January 2020, LPV/r was switched to DTG in an attempt to simplify the regimen and decrease the pill burden; however, he remained on AZT/3TC as he weighed 28 kg. The VL at the time of the switch was 529 copies/mL and hence a HIV DR test was not performed. However, he remained unsuppressed and admitted to often forgetting his medication as he lacked a treatment supporter.
There were major social issues impacting adherence; he is the product of rape by his mother's older cousin. Being a teen mom, and not having disclosed the rape issue to her mother proved to have affected the patient's mother, as well as her ability to provide optimum care for her child. She was the primary caregiver living with the maternal grandmother and was responsible for bringing him for his hospital appointments, but would sometimes not be available to bring him, and occasionally the patient's biological father would accompany the patient to hospital but identified himself as the patient's uncle. In 2017, the father became ill, and the grandmother (not knowing the issues around the rape) took him into their home, resulting in family conflict and missed hospital appointments. In 2019, both the patient's biological parents died due to AIDS-related complications. Upon discovering for the first time at her daughter's funeral that her nephew had raped her daughter and infected her with HIV, the patient's grandmother struggled to accept the situation. As a result, the grandmother avoided involving herself in the healthcare of her grandson including supervising him when taking treatment, or accompanying him to hospital, making addressing the adherence issues and disclosure difficult, requiring a home visit by the social workers.
Due to poor adherence and patient not suppressing for two years on the DTG-based regimen, despite enhanced adherence counselling, HIV DR was done in April 2022 which showed M184V again and the development of InSTI mutations (major T66TI, G118R, E138K, minor M50I), conferring high-level resistance to DTG (refer to Table 1).
The third-line regimen commenced in May 2022 was TDF, emtricitabine and ritonavir-boosted darunavir once daily. The viral load done after one month was 473 copies/mL with a 2 log10 drop.
|
dtg, hiv, adolescence, dolutegravir, paediatrics, resistance
| Not supported with pagination yet
| null |
PMC4318046_01
|
Male
| 38
|
A 38-year-old male, diagnosed as HIV-positive, presented to our department with multiple painful, erythematous skin lesions over extremities, face and abdomen of 2 days duration along with the history of evening rise of temperature with chills, malaise, body pains, joint pains, and ankle edema. The patient had history of nasal stuffiness and epistaxis. He was not a known case of leprosy. He was on ART zidovudine, lamivudine, nevirapine (ZLN) for 4 weeks prior to the onset of skin lesions. Further, history of sexual exposure with multiple heterosexual partners was noted. No history of diabetes mellitus, hypertension, or tuberculosis.
On general examination, he was pale, thin built, ill nourished and febrile. On examination, there was no generalized lymphadenopathy. There was diffuse infiltration of ear lobe [Figure 1]. Multiple erythematous, tender papules, and nodules were present on the face [Figure 2] and also on trunk and extremities [Figure 3]. Sensations of pain and touch were reduced over both the feet. Ulnar, radial cutaneous, lateral popliteal, and posterior tibial nerves on both sides were thickened and tender. Hair, nails and mucous membranes were normal. Systemic Examination revealed no abnormalities.
Hematological Examination revealed microcytic hypochromic anemia (Hb: 10 g%), and neutrophilic leukocytosis (TLC: 12,800/mm3) differential count N75L20M3E2, and raised ESR (68 mm in-first hour). VDRL was non-reactive, and HBsAg was negative. The findings of complete urine examination were normal. Blood glucose levels, renal function and liver function tests were normal. Chest X-ray and ultrasonography of abdomen were normal. HIV-1 was reactive, the viral load was 1,07,400 copies/mL and CD4 count 94 cells/cu. mm before starting ART. At the time of diagnosis of IRIS, which is 1 month after starting of ART, CD4 count was 151 cells/cu. mm. Slit skin smear examination for M. leprae revealed bacteriological index of 4 along with a morphological index of 20%. Biopsy taken from the nodule on the left hand [Figure 3] demonstrated mild hyperkeratosis in epidermis, grenz zone. Dermis shows edema with collection of macrophages [Figure 4]. Vessels showed acute and chronic perivascular inflammatory infiltrate [Figure 5] consistent with ENL.
On the basis of the history, clinical examination, and investigative findings, a diagnosis of IRIS presenting as ENL in a patient of HIV with unmasking of lepromatous leprosy was made.
The patient was continued on ART and started on MBMDT (rifampicin:-600 mg, dapsone:- 100 mg, and clofazamine:-50 mg). He was given oral steroids which were gradually tapered over a period of 3 months. After 9 months of MBMDT and ART, no further episodes of ENL were reported and his CD4 count is 367. The patient is doing fine and attending to his duties.
|
erythema nodosum leprosum, immune reconstitution inflammatory syndrome, lepromatous leprosy
| Not supported with pagination yet
| null |
PMC4840429_01
|
Male
| 75
|
A 75-year-old white male, weighing 65 kg, presented to our center with complaints of a 3-months history of fatigue, evening rise in body temperature, night sweats, unexplained weight loss of 5 kg, worsening dyspnea, cough and mucopurulent sputum. He was a current smoker (30 pack/years) and worked in a stone quarry up to 12 years before; he had a history of chronic catarrhal bronchitis, benign prostatic hypertrophy and an intestinal polypectomy. He denied performing spirometry and taking systemic or inhaled corticosteroids; a chest x-ray of 3 years before showed rarefaction of bronchovascular tree.
On examination, he was afebrile and reduced vesicular murmur and widespread rhonchi were heard on auscultation of the chest. Remainder of the physical examination was unremarkable.
Blood tests showed neutrophilic leukocytosis (white blood cells 16500/mL, 70% neutrophils), mild anemia (hemoglobin 11,4 g/dl), moderate increase in serum inflammatory markers (erythrocyte sedimentation rate 72 mm/h, C-reactive protein 20 mg/l). Plasma immunoglobulin, liver and renal function were in the normal range and HIV antibody testing was negative.
A spirometry test was performed, revealing a severe obstructive ventilation defect, post b2 agonist Forced Vital Capacity of 72% and Forced Expiratory Volume in one second of 46% predicted. The chest X-ray showed, bilaterally in the apical and subclavian regions, sclerosis and multiple nodules with cavitations (Fig. 1). The chest high resolution computed tomography (HRCT) was reported as following: "Multiple nodular opacities are recognized in the upper lobes, in the apical segments of the lower lobes and in subpleural location; small and regular central cavitations are there in some of these lesions. Multiple focal points of "tree in bud" and bronchiectasis, with prevalent cystic type, in both lung apices are also present. No significant mediastinal lymphadenopathy. These findings are consistent with chronic TB" (Fig. 2).
Three sputum samples on three different days were collected and submitted for the search of Mycobacteria spp. Specimen were digested and decontaminated according to Kubica procedure; sputum smears for acid-fast bacilli examination using Kinyoun staining and culture on solid (Lowenstein-Jensen) and liquid medium (Mycobacteria Growth Indicator Tube) were then prepared. The microscopic examination showed filamentous, slim, branched (mycelium and hyphae), blue-to-fuchsia colored (acid-alcohol variable) elements (Fig. 3). Nocardia spp. was suspected and confirmed by Gram staining (not shown) and blood agar culture (Fig. 4). Culture on Lowenstein-Jensen medium is showed in Fig. 5. All the previous exams were negative for M. tuberculosis.
Genotypic identification was performed by amplification and sequencing of a DNA fragment coding a rRNA 16S (Applied Biosystem, USA). Sequences were compared with the reference ones present in the GenBank database through BLAST and Nocardia cyriacigeorgica with 100% identity was identified.
Minimal inhibitory concentrations were determined on Mueller Hinton Agar following a 24 h incubation at 37 C (Etest, BioMerieux). Results were revised and interpreted according to CLSI criteria: amikacin 0.25 mug/mL; amoxicillin plus clavulanic acid (8 mug/mL); ceftriaxone (1 mug/mL); cefepime (2 mug/mL); ciprofloxacin (4 mug/mL); clarythromycin (4 mug/mL); imipenem (0.38 mug/mL); linezolid (0.50 mug/mL); tobramycin (0.047 mug/mL); trimethoprim plus sulfonamide (0.006 mug/mL).
Patient was then started on trimethoprim-sulfamethoxazole (160/800 one tablet twice per day) for 6 months. Following this, a rapid improvement of symptoms, regression of the purulent sputum and of the neutrophilic leukocytosis had been achieved. A second chest radiograph was performed 2 months later, showing an improvement of radiological findings too. A spirometry test was then repeated, confirming the suspect of COPD; specific therapy was started.
The patient gave an informed consent for the case report.
|
chronic obstructive pulmonary disease, copd, chronic obstructive pulmonary disease, high resolution computed tomography, hrct, human immunodeficiency virus, hiv, opportunistic infections, pulmonary nocardiosis, pn, pulmonary nocardiosis, tuberculosis, tuberculosis, tb, trimethoprim-sulfamethoxazole, tmp-smx
|
Chest HRCT scan showing multiple nodular lesions with cavitations in upper lung zone bilaterally.
|
|
PMC3291382_01
|
Male
| 52
|
A 52-year-old man with chronic eczema was admitted to the Prince of Wales Hospital, Hong Kong, with fever and chills. Before admission, he had been treated for infected eczematous lesions for several weeks with oral ampicillin, cloxacillin, and cefazolin. He had no history of hospitalization in the past 10 years, and none of his family members were healthcare workers. Examination showed an oral temperature of 40 C, blood pressure 95/55 mm Hg, and no audible murmur. Cellulitis in the left leg complicated his eczematous skin lesions. Chest radiograph showed right-middle-zone pneumonia. Neutrophilia (leukocytes 15.5 x 109/L, neutrophils 86%), thrombocytopenia (platelets 55 x 109/L), prolonged activated partial thromboplastin time (43.6 s), and elevated bilirubin level (31 mumol/L) were observed. Two initial blood cultures grew gram-positive cocci in clusters, identified as S. aureus by positive results for catalase and slide/tube coagulase and a negative result for ornithine decarboxylase. Intravenous cloxacillin (2 g every 6 h) was given on days 2-5. Antimicrobial drug susceptibility testing was performed by the disk-diffusion method (1 mug oxacillin/disk, Mueller-Hinton agar, 2% NaCl), followed by MIC determination with the agar dilution method in accordance with NCCLS (former National Committee for Clinical Laboratory Standards, now Clinical and Laboratory Standards Institute) recommendations. One blood isolate was identified as methicillin-resistant S. aureus (MRSA), with an oxacillin MIC 4 mug/mL. The other isolate was identified as methicillin-sensitive S. aureus (MSSA), with an oxacillin MIC of 0.5 mug/mL. In view of a possible CA-MRSA infection (which could have been beta-lactam-resistant), cloxacillin was substituted with intravenous vancomycin plus rifampin on day 5.
However, the patient's condition progressively deteriorated from day 2 to day 10 with persistent fever, chills, hypotension, and hemoptysis. A repeated chest radiograph showed small lung cavities with fluid, and a thoracic computed tomographic scan confirmed multiple lung abscesses. Results of an initial transthoracic echocardiograph were normal, but a subsequent transesophageal echocardiograph demonstrated tricuspid valve vegetation.
The MRSA isolate was susceptible to gentamicin, cotrimoxazole, erythromycin, ciprofloxacin, clindamycin, fusidic acid, tetracycline, chloramphenicol, vancomycin, and rifampin; a different pattern of multidrug-resistant MRSA isolates from that usually found in our facility (,). The isolate was also susceptible to ampicillin/sulbactam, with an equivalent breakpoint MIC <8/4 mug/mL by disk testing. Latex detection for PBP2a (Slidex MRSA-Detection, bioMerieux, Marcy l'Etoile, France) and polymerase chain reaction (PCR) detection for mecA were both negative, predicting nonresistance to oxacillin (-). A nitrocefin-disk test was positive for beta-lactamases, and a 4-fold reduction in MIC was demonstrated in the presence of sulbactam. Panton-Valentine leukocidin (PVL) gene locus was not detected. Community-acquired BORSA (borderline oxacillin-resistant S. aureus), infective endocarditis, and lung abscesses were diagnosed. Intravenous ampicillin/sulbactam (3 g every 6 h) was given on day 10 with rifampin; vancomycin treatment was stopped. Defervescence occurred 3 days later, subsequent blood cultures became sterile, and radiographic changes gradually resolved. Ampicillin/sulbactam was given for 6 weeks without complication.
As this case suggests, BORSA can sometimes be confused with CA-MRSA because of similar clinical signs and symptoms and overlapping oxacillin MICs (2-8 mug/mL and 4-64 mug/mL, respectively) (,,). Both pathogens can appear as community-acquired infections and may be related to previous antimicrobial drug usage (,). Although CA-MRSA has been associated with soft tissue infections and necrotizing pneumonia (,), MSSA or BORSA strains can also cause these diseases. Thus, in view of potentially different treatment options, when MRSA isolates (e.g., oxacillin MICs >4-8 mug/mL) are associated with community-acquired, serious infections (e.g., blood isolates) and are not multidrug resistant, one can consider mecA (or PBP2a) testing to delineate the resistance mechanism (Table). If mecA is present, further testing for PVL gene locus with or without staphylococcal chromosomal cassette mec (SCCmec) type IV can be performed to diagnose CA-MRSA; if mecA is not detected, further testing for BORSA may be indicated, and beta-lactam therapy should be evaluated individually. If these pathogens are not differentiated and all are treated as CA-MRSA, a non-beta-lactam antimicrobial drug, such as vancomycin, will be used (,,,). However, for serious and deep-seated S. aureus infections (e.g., bacteremia, endocarditis), vancomycin is inferior to beta-lactam antimicrobial drugs, even when in vitro testing indicates susceptibility. Treatment failures have been encountered. Linezolid is a good alternative but limited by availability and cost, and clindamycin therapy can be associated with inducible resistance. For BORSA-associated infections, beta-lactam antimicrobial drugs, including high-dose penicillinase-resistant penicillins (PRPs) (e.g., cloxacillin) or beta-lactam/beta-lactamase-inhibitor combinations (e.g., ampicillin/sulbactam) are regarded as treatments of choice (,,).
BORSA initially described nonheteroresistant strains of S. aureus with oxacillin MIC <2 mg/L, which produce ample beta-lactamases and are rendered fully susceptible to PRP by beta-lactamase-inhibitors (,). Subsequent BORSA strains described have had higher oxacillin MICs (4-8 mg/L). The proportion of BORSA among clinical isolates of S. aureus varies (1.4%-12.5%) but is usually 5% (,). A BORSA infection outbreak among dermatology patients with severe skin diseases has also been reported. Postulated resistance mechanisms include overproduction of conventional penicillinases, production of an inducible, plasmid-mediated, membrane-bound methicillinase, and in some cases, point mutations of penicillin-binding-proteins. The clinical importance of BORSA is unknown since early clinical/animal data suggest treatment efficacy of PRP (against strains with MIC <2 mg/L) (,,). Whether BORSA with higher oxacillin MICs (4-8 mg/L) will respond equally well to PRP is less clear. Further studies into the treatment of BORSA, including pharmacokinetic considerations, are needed. However, high-dose beta-lactam/beta-lactamase inhibitor combinations (e.g., ampicillin/sulbactam), as shown in animal models, are at least as effective as PRP. In conclusion, our report suggests that mecA (or PBP2a) detection may help manage serious, community-acquired, non-multidrug-resistant MRSA infections because of the potential confusion between BORSA and CA-MRSA.
| null | Not supported with pagination yet
| null |
PMC4268755_01
|
Male
| 35
|
We received a spontaneous vaginally delivered male outborn baby at 35 weeks period of gestation, weighing 1900 grams to a 24-years-old primigravida with antenatal risk factors of gestational diabetes mellitus and polyhydramnios. The baby was born limp without any cry and required immediate resuscitation with bag and mask ventilation. Baby continued to have respiratory distress with copious oral secretions. Baby was intubated and shifted to NICU for mechanical ventilation. The initial intubation was reportedly difficult, and baby required frequent ET suctioning for copious secretions.
Clinically, a strong suspicion of esophageal atresia (EA) and tracheo-esophageal fistula (TEF) was made as feeding tube did not pass into the stomach. The chest X-ray revealed lower end of feeding tube going very low almost to T 6/7 level, and serial X rays revealed the feeding tube going sometimes to left side and sometimes to the right side, in retrospect, we came to know that it was in the trachea and was negotiating to left or right main bronchus. Baby also had other associated anomalies at birth i.e. imperforate anus and absent right kidney.
The evaluation with ultrasound revealed dilated left renal pelvis with non-visualized right kidney. After initial stabilization, baby was taken up for emergency surgical exploration. The newborn was then placed in the left lateral decubitus position and a right posterolateral thoracotomy was performed. Tracheo-esophageal fistula was isolated just one cm above the carina and was transfixed and ligated. The upper blind pouch of esophagus could not be localized even after extensive search. The feeding tube could not be seen or felt in the operative field on maneuvering by anesthesiologist. A trial of passing a large bore tube i.e. mucus sucker was attempted, which went through but again could not be traced in the operating field as it was going into the trachea along with the ETT. A trial of suctioning attempt on the mucus sucker made the right lung deflate with desaturation noticed by the anesthesiologist, which gave us a clue as to the connection of the upper end of esophagus with the trachea. An attempt was made by the otolaryngologist intraoperatively to negotiate the fiber-optic scope through the blind upper pouch, which was unsuccessful. Neck exploration for creating a cervical esophagostomy was carried out; however, the procedure was abandoned due to non-visualization of the upper end of esophagus. Peroperatively, we found after neck exploration that there was only one continuous hollow tube containing the ET tube. A feeding gastrostomy and sigmoid colostomy was carried out, and baby was shifted back to neonatal intensive care after the completion of surgery.
Baby was continued on ventilation and other supportive management with antibiotics, IV fluids, and inotropes. However, the baby succumbed on day 4 of life despite aggressive therapeutic and ventilatory support. Direct laryngoscopy revealed only aryepiglottic folds behind the epiglottis, no vocal cords, and there was no visible opening for the trachea suggesting a common upper airway and digestive tract. Fiber-optic scope was passed through the common opening, which revealed normal hypopharynx with normal development of the epiglottis but no evidence of true vocal cords. There was a collapsing lumen opening into a non-collapsible structure i.e. esophagus (collapsible) with absence of proximal trachea continuing with distal trachea (non-collapsible) [Figure 1]. A complete autopsy revealed laryngeal atresia with absent vocal cords and a common aerodigestive tract continuing distally with trachea [Figure 2]. There was also a solitary left kidney with high anorectal anomaly fulfilling VACTERL association.
|
absent upper pouch, vacterl association and tracheo-esophageal fistula, laryngeal atresia
| Not supported with pagination yet
| null |
PMC5443919_01
|
Male
| 50
|
A 50 years old black male from Guinea-Bissau and living in Portugal for the past seven months presented to the emergency room complaining of persistent frontoparietal headaches for the past week. The headache was non pulsatile, associated with photo and phonophobia, nausea and anorexia, and it disturbed his sleep, leading the patient to wake up several times during the night. The headache didn't worsen with the decubitus position. He denied other symptoms, particularly in terms of the respiratory, gastrointestinal, urinary or cutaneous systems. According to his wife, he had periods of marked disorientation and apathy since 3 years ago, since the time he was diagnosed hypertension (HTA), treated with amlodipine. Other comorbidities included a probable dementia related to multi-infarctions suffered a year earlier, and had no known allergies.
At examination, the patient presented with apparent slowing of movements, associated with time and space disorientation. He was afebrile, calm, with a blood pressure of 105/78 mmHg, a heart rate of 87 beats per minute and a respiratory rate of 16 cycles per minute. The cardio-pulmonary auscultation, the abdominal inspection and palpation were all normal. He had severe xerodermia, but no other skin lesions. He had no edema and no palpable peripheral lymphadenopathy.
The neurological examination showed the patient was conscious, but uncooperative, without apparent dysarthria, preserved visual fields, isochoric and isoreactive pupils, eye movements with no limitation, without facial palsy or motor lateralization, and without ataxia or neck stiffness.
In the emergency department blood tests showed: leukocytes 5.3 x 109/L (Normal Range: 4-10 x 109/L), platelet count 213 x 109/L (NR 150-400 x 109/L), hemoglobin 14.7G/dL (NR 13-17G/dL), erythrocyte sedimentation rate of 36 mm/1st h (NR: <20 mm/1st h), creatinine 1,28 mg/dl (NR: 0.8-1.3 mg/dL), AST 40 U/L (NR: 5-30U/L), ALT 42 U/L (5-30U/L), gamma glutamyl transferase 346 U/L (NR: 6-50U/L), alkaline phosphatase 249 U/L (50-100U/L), lactate dehydrogenase 261U/L (50-150U/L). An abdominal and renal ultrasound were requested and revealed no hepatic or renal abnormalities. The CT scan of the brain initially performed revealed diffuse brain atrophy, showing multiple small nodular lesions, isodense and a ring enhancement after intravenous injection of iodinated contrast, involving the brain and the cerebellum (Fig. 1). Subsequently, a lumbar puncture was performed, and the cerebrospinal fluid (CSF) showed to be xanthochromic, with an opening pressure of 13cmH2O. CSF analysis included: glucose 52 mg/dl (NR: >60 mg/dL), protein 43 mg/dl (NR: <42 mg/dL), leukocytes <1/mm3, erythrocyte 2800/mm3; India ink test and Streptococcus pneumoniae antigen were negative.
Considered the possibility of an infectious etiology, the patient was transferred to the Infectious Diseases ward for further studies. These included: negative HIV and syphilis screening tests, immunity (IgG presence and absence of IgM) for toxoplasmosis, cytomegalovirus, Borrelia and hepatitis A virus; natural immunity to hepatitis B virus; absence of antibody for hepatitis C virus, amebiasis, hydatidosis or cysticercosis. The lymphocyte populations were normal, with lymphocyte T-CD4+ of 600 cells/mm3 (NR: >700cells/mm3) and a CD4/CD8 ratio of 2.48. Interferon gamma test (QuantiFERON TB Gold ) was strongly positive.
By the 7th day at the ward, a brain magnetic resonance was performed and it confirmed the presence of multiple nodular intra-axial lesions with intense enhancement, predominantly homogeneous, but in form of "ring" in the larger ones, and surrounding edema relatively large for their small dimensions, although with no significant mass effect. Given the characteristics of the lesions, the differential diagnosis of secondary brain (metastatic) lesions, or an infectious etiology with hematogenous spread, including tuberculosis, were considered. The lumbar puncture was repeated, with a normal cytochemical examination, as it was the adenosine deaminase (ADA). At that time, CSF was also collected for cultures and polymerase chain reaction in real time (PCR-rt) screening for mycobacteria.
By the 11th day in the ward, the liver enzymes remained elevated (AST 51U/L, ALT 54 U/L, GGT 375 U/L, alkaline phosphatase 328 U/L) and a liver biopsy was performed. Histological result of the sample revealed necrotizing granulomatous inflammation, setting the diagnostis of tuberculosis as the main one. Given these results, antituberculosis drugs (ethambutol, rifampicin, isoniazid and pyrazinamide) were initiated.
At that time, a bronchoscopy with bronchoalveolar lavage was performed, as well as CT scans of the thorax, abdomen and pelvis. These last exams revealed a mass in the posterior mediastinum with 2.6 x 2.3 cm in contact with the esophagus, multiple adenopathies above and below the diaphragm, micronodules in the lung, and an increased heterogeneous prostate. After these findings, a gastrointestinal ecoendoscopy and a prostate ultrasound were performed. The first test confirmed the presence of a retroesophageal mass and it was sent to histology and microbiological study. The direct examination (with Ziehl-Nielsen staining) was strongly positive (>10bacilis/field). On the other hand, transrectal prostatic ultrasonography showed a gland of increased dimensions (54cc) with symmetrical and regular borders, and several well-defined hypoechoic nodular formations (Fig. 2), some with internal calcifications. Given the clinical information, and after discussion with the Urology department, a tuberculous etiology was actually considered as the most likely.
Among the ongoing tests performed since the beginning of hospitalization, we highlight the negative results of mycobacterial research (cultures and PCR-rt) in CSF, blood, urine or broncho-alveolar liquid.
During his stay on the ward, the patient had a slight improvement in his cognitive functions, being able to communicate easier. Another CT scan of the brain was performed after one month of antituberculosis drugs, and it showed a change in the enhancement pattern, now more diffuse, an aspect that, combined with the absence of worsenning, favored the hypothesis of tuberculosis in opposition of neoplastic etiology.
The patient was discharged from the hospital at that time, clinically and hemodynamically stable. He remains in follow-up by the Infectious Diseases team as an outpatient, and shows improvement of his overall status, specially his cognitive functions.
|
tuberculosis, cerebral tuberculosis, liver tuberculosis, milliary tuberculosis, prostate tuberculosis, retroesophageal tuberculosis
| Not supported with pagination yet
| null |
PMC9487995_01
|
Female
| 29
|
We report and discuss a case of pulmonary tuberculosis (PTB) with failure after 9 years (February 2012, to July 2021) systematic treatment. The immunodeficiency workup on the patient, a 29-year-old female, revealed normal immunoglobulin levels and a negative result for the anti-HIV human T-lymphotropic virus. In addition, further relevant analysis showed no evidence of other comorbidities including hepatitis, diabetes, and other respiratory diseases. By analyzing her treatment history, drug susceptibility testing (DST), Computed tomography (CT) images, and isolated strains' whole genome sequencing (WGS), we hope to identify the causes of treatment failure and seek expert guidance to improve her survival time and quality of life (A detailed case report is provided in the Supplementary Appendix).
The case's whole treatment history can be divided into three stages: in the first stage (February 2012 to March 19, 2017), the patient was hospitalized in the local tuberculosis-designated hospital in February 2012 with her first pulmonary tuberculosis diagnosis and began to receive first-line antituberculosis regimen. After 1 year of treatment, she decided to stop treatment with improvement in March 2013. Only 2 months later, she was admitted to Beijing Chest Hospital due to the recurrence and deterioration of the previous clinical manifestations and then treated with para-aminosalicylate (PAS), rifabutin (RFB), pyrazinamide (PZA), amikacin (AMK), ethambutol (EMB), and levofloxacin (LFX) for 2 years but not cured. On March 19, 2017, CT images showed the lesions increased and progressed. Throughout this stage of therapy, DSTs results were absent. In the second stage (March 20, 2017, to June 12, 2019), the patient received standardized treatment for multidrug-resistant tuberculosis (MDR-TB) under DST results and was not cured. In this stage, based on the DST results, the patient was treated with TB sensitive drugs, including cycloserine (CS), linezolid (LZD), capreomycin (CM), moxifloxacin (MFX), clofazimine (CFZ), but the deterioration continued to progress. In addition, the CT images showed that two pre-existing independent thick-walled cavities 1 and 2 in the right upper lung had partial absorption. In the third stage (June 13, 2019, to July 23, 2021), the patient received Bedaquiline (BDQ) and Delamanid (DLM) combinated with the previous standardized treatment but was still not cured. In the absence of accessible therapeutic medications and after declining our surgical recommendations, the patient was gradually transitioned to palliative care. Interestingly, the DSTs results on June 13, 2019, and August 24, 2020, showed that LFX, protionamide (PTO) and isoniazid aminosalicylate (PA) changed from drug resistance to sensitivity. However, the subsequent five phenotypical DSTs results from October 30, 2020, to July 23, 2021, were similar to those in 2017. In addition, CT images presented those two cavities fused from connected locally to extensively. The fusion lesion infiltrated outward and deteriorated rapidly, resulting in the destruction of the entire right upper lobe (Figures 1A,B, Table 1).
Based on the continuously reversed results of phenotypical DSTs results and CT images in different stages, we speculate that the patient was infected with two different strains of Mycobacterium tuberculosis (Mtb). To verify our conjecture, the five existing sputum samples sampled in Apr 2017, Aug 2017, Jun 2019, Apr 2021, and May 2021 were sequenced by the whole genome (Details of WGS and Variant calling are provided in the Supplementary Appendix). All five strains belonged to lineage 2.3, differing by up to 196 SNPs, of which the strain in 2019 is a separate isolate (cluster 1), and the remaining four strains are from the same cluster (cluster 2). In view of the diversity of strains in vivo hardly exceeds 12 SNPs, we speculate that this patient has mixed infection. The two clusters shared four mutations in rpoB, fabG1_promoter, rpsL and gyrA, which were associated with rifampicin (RFP), isoniazid (INH), streptomycin (SM) and fluoroquinolones resistance, respectively. Additionally, five independent mutations in cluster 1 were associated with first-line drug resistance (INH, EMB, PZA), consistent with the patient's first-stage treatment history. Given that drug selection pressure is the primary driver of resistance mutations in strains, and no EMB and PZA related mutations were found in cluster 2, we speculate that cluster 1 was the first infected strain of this patient (Figures 1C,D). This was reflected in the emergence of mutations in rrl. Therefore, we speculated that the two strains coexisted in the lung for a long time, resulting in the failure of the patient's treatment.
Summarizing the above information, although most results of the phenotypical DSTs results were consistent with the genotype DST results, the occurrence of gene resistance to AMK, capreomycin (CAP), MFX was earlier than the phenotypical DST results, which indicates that the drug resistance mutations had gradually accumulated during the treatment, but the phenotypical DST results was not prompted in time. The treatment scheme was not changed in time, resulting in the gradual accumulation of resistance and treatment failure. In addition, with the advent of new drugs such as BDQ and DLM, the cure rate of multidrug-resistant TB can reach over 80%. However, this patient still failed to respond to treatment without resistance to BDQ and DLM, which may be related to severe cavities The necrotic tissue in the cavities is not only the rich medium of Mtb, but also the cavity walls composed of connective tissue may hinder the drugs' penetration, which is easier to induce Mtb to produce resilience, tolerance and resistance.
This study has some limitations. First, the treatment failure in the first stage can be explained from the genotype resistance results of the strain in April 2017, but we have no way of knowing the resistance situation and evolution process of the strains before. Second, the early strains were not preserved during the 9-year treatment process, so the sequencing of infection of the two clusters cannot be accurately obtained and the cumulative changes of drug resistance genes could not be dynamically observed.
In this study, we found that genotype DST findings, which are sooner than phenotypical DST results, can provide superior treatment guidance for tuberculosis. In addition, mixed infection of Mtb leads to the emergence of heterogeneous flora, making the treatment more complex. Therefore, we should be more cautious when prescribing medication to patients who haven't been healed for a long time and utilize WGS for drug resistance surveillance.
|
computed tomography-three dimensional, multidrug resistance, treatment failure, tuberculosis, whole genome sequencing
| Not supported with pagination yet
| null |
PMC7293903_01
|
Male
| 69
|
In April 2018, a 69-year-old man with a history of DM and chronic kidney disease and undergoing dialysis treatment was admitted to Okinawa Miyako Hospital after complaints of slight bloody sputum for the past seven days and weight loss of 5 kg in the past six months.
On admission, he was afebrile and did not present with respiratory distress; vital signs were normal. Auscultation revealed clear respiratory sounds, and other physical examinations also yielded unremarkable findings. However, blood tests revealed elevated white blood cell count (12,700 cells/microL) and C-reactive protein levels (14.76 mg/dL). A computed tomography (CT) scan of the chest showed multiple consolidations with cavities in both lung fields (Figure 1). The patient had routine chest X-rays every three months, due to ongoing dialysis treatment, to check his cardio-thoracic ratio and the chest radiographs from one, four, and six months ago also showed multiple interstitial infiltrations. The locations of these infiltrations were consistent with the lung abscesses revealed by the CT on admission; these results indicated that the abscesses had existed for at least six months (Figure 1). He was hospitalized based on the diagnosis of multiple lung abscesses. Additional examinations such as urine culture, cardiac ultrasonography, and CT scans of the abdomen and head were performed to determine whether this patient had other primary source or metastatic infections. Other sources of primary and metastatic infections, such as infective endocarditis, liver abscess, brain abscess, or ocular infection, were all absent.
To determine the causative pathogen, we performed a bronchoscopy. Gram-stain of bronchoalveolar lavage fluid (BAL) showed only capsulated gram-negative rods (Figure 2), and the microorganism was identified as K. pneumoniae by conventional methods. The involvement of other pathogens, such as anaerobes, was not evident from the BAL cultures. Two sets of blood cultures were also negative for other microorganisms. String test of the isolate was positive (the string reached a length of 12 mm), which is characteristic of the hypermucoviscous phenotype. The isolate was susceptible to all routinely tested antibiotics except ampicillin. We initially treated the patient with ampicillin/sulbactam for three weeks. Next, he received oral amoxicillin/clavulanic acid suppression therapy for four weeks, after which he recovered without recurrence.
We performed multiplex PCR (Figure 3) and multilocus sequence typing as described previously. We determined the capsular serotype of the isolate to be K2 and ST 375: K2-ST375; the isolate possessed rmpA (a positive regulator of capsular polysaccharide production), iutA, entB, and mrkD. The isolated strain produced a positive string test and a positive PCR amplification of the rmpA gene; therefore, we identified the isolated strain as hypervirulent.
|
japan, okinawa, hypervirulent klebsiella pneumoniae, primary lung abscess, sequence type 375, serotype k2
| Not supported with pagination yet
| null |
PMC8631821_01
|
Male
| 39
|
In September 2020, a 39 year-old man was admitted to Shanghai Ruijin Hospital complaining of a serious discharge from a small opening on his left flank. It had been occurring for 2 months without fever or pain. His medical history evidenced a laparoscopic left partial nephrectomy for clear cell renal cell carcinoma (ccRCC) in December 2015. Three weeks post-surgery, he had developed purulent discharge from the drainage tube orifice on his left flank. Seven months later, after three rounds of debridement and percutaneous drainage procedures, the wound was healed. There was no report of previous tuberculosis or pyelonephritis. Physical examination revealed that the fistula covered an area of 0.5 x 0.5 cm, and 6 cm deep on his left flank without tenderness or percussed pain (Figures 1A,B). Laboratory tests including renal function tests and urinalysis showed no unusual results. Urine culture and secretion culture were negative. To rule out tuberculosis, we conducted a fluorescent quantitative PCR test of the secretions from the fistula, which was also unremarkable.
Renal dynamic imaging revealed normal function of the left kidney. Preoperative abdominal CT showed the sinus tract, extending from the surface of left kidney near the lower upper pole to the skin without renal/ureteral stone or hydronephrosis (Figures 1C-E). The three-dimensional reconstruction clearly showed the relationship between the sinus tract and the kidney (Figures 1F-H). Next, we performed a fistulous tract endoscopy, which showed a mass of white flocculus stuck to the lining of the sinus tract (Figure 2A). Given the frequent recurrence and complexity of NCF, we planned to repair the fistula and prevent relapse using complete sinus tract resection combined with omental flap grafting.
Specific steps in the surgical operation were as follows:
Complete excision of the sinus tract under the guidance of methylene blue staining. The bottom of the sinus tract was stuck to the lower pole of the left kidney (Figure 2B).
We found a substantial amount of hard tissue similar to calcification, and a hemoclip at the lower pole of the left kidney. We removed all the abnormal tissue and hemostasis (Figures 2C,F).
We opened the posterior peritoneum and pulled part of the greater omentum tissue into the retroperitoneal space with oval forceps. Then, we tightly covered the left kidney with the omental flap and fixed it with sutures (Figures 2D,E).
We sutured the posterior peritoneum with the root of the omental flap. Then, we closed the posterior peritoneum incision. Finally, we closed the operation incision in layers in the usual manner.
The pathological investigation of the resected sinus tract showed chronic inflammation with granulation hyperblastosis and foam cells growth. The patient was discharged 6 days after the operation. Six months later, MR examination showed that the NCF had healed well with the omental flap tightly covering the kidney (Figures 3A,B). The patient was asymptomatic throughout 12 months of follow-up.
|
foreign body reaction, nephrectomy, nephrocutaneous fistula, omentum, sinus tract
| Not supported with pagination yet
| null |
PMC4023550_01
|
Female
| 6
|
The patient, BSB, was a 6-year-old girl, the product of the third pregnancy of a healthy, nonconsanguineous young couple with a previous history of two miscarriages. The pregnancy was uneventful. The mother reported that her younger sister also had three miscarriages, and her older sister gave birth to four normal children.
The patient was born by vaginal delivery at 36 weeks of gestation, presenting polyhydramnios, a birth weight of 2750 g (50th percentile) and birth length of 46 cm (50th percentile); her head circumference was 32 cm (50th percentile), and the Apgar scores were 6 and 8. She was kept in intensive care for 3 days due to respiratory distress. At the age of 2 months, she had difficulties in responding to sound stimuli, as a consequence of bilateral otitis. At 6 months, she underwent surgery for the correction of bilateral inguinal and umbilical hernia. Clinical evaluation at the age of six years showed failure to thrive, along with intellectual disability, hearing impairment, speech delay and dysphagia. Physical examination showed a high-set hairline, mild synophrys, ocular hypertelorism, upslanting palpebral fissures, a flat-bridged and broad-based nose, hypoplastic nostrils, prominent columella, long filtrum, thin upper lip, prominent chin and wide mouth with conical teeth. Her hands showed few palmar creases, clinodactyly of the 5th fingers, persistence of digital pads, and her toes had prominent interdigital folds. Some of these features can be seen in Figure 1.
X-rays of hands and feet showed no abnormalities, and a cranial CT scan and MRI were normal. The patient's early development was severely delayed, with a pronounced deficit in the acquisition of motor, language and social skills. Due to a sucking and swallowing disability, she showed difficulties in gaining weight. She displayed good behavior and a docile personality.
Upon initial analysis, the proposita's karyotype was determined as being 47,XX,+mar. C-banding and NOR-staining characterized the marker as a monocentric and monosatellited chromosome. A cytogenetic evaluation of the family revealed a 46,XX,t(15;16)(q13;p13.2) karyotype in the mother and one aunt, and a 46,XY,t(15;16)(q13;p13.2) karyotype in the maternal grandfather. The patient's karyotype was therefore redefined as 47,XX,+der(15)t(15;16)(q13;p13.2)mat.
Array-CGH analysis revealed a duplication of about 3.1 Mb of the proximal 15q segment (chr15:18,741,516-21,856,312), comprising TUBGCP5, CYFIP1, NIPA2 genes, and unexpectedly also showed a 1.3 Mb duplication of a distal 16p segment (chr16:2,056,890-3,346,212), comprising TBC1D24; PKD1; THOC6 genes, as well as a small duplication (0.6 Mb) of a proximal 16p segment (chr16:32,748,149-33,316,84). FISH analysis using a centromeric 15 probe and a WCP16 probe confirmed the involvement of chromosomes 15 and 16 in the rearrangement (Figure 2). The breakpoint and intervals of the duplications were entered into the UCSC Genome Browser to search for gene content and function, and were confirmed by the NCBI Map Viewer (http://www.ncbi.nlm.nih.gov/projects/mapview/). DECIPHER (https://decipher.sanger.ac.uk/) and DGV - Database of Genomic Variants: http://dgv.tcag.ca/dgv/app/home were also used to analyze the imbalanced regions.
|
15q duplication, 16p duplication, complex ssmc, familial inheritance
| Not supported with pagination yet
| null |
PMC3934610_01
|
Male
| 26
|
An asymptomatic 26-year-old previously healthy white male was referred to us following a routine optometrist visit. Visual acuity was 6/6 in both eyes, with no sign of anterior segment inflammation, but with the presence of a single, inactive, yellow choroidal lesion, 0.75 mm in diameter, located inferior to the left optic disc (fig. 1).
Time domain OCT of the lesion showed thinning and compression of the overlying choroidal vasculature with an apparently normal retina above it. Spectral domain OCT with enhanced depth imaging (fig. 2) helped locate the lesion on the choroid and sclera, under the attenuated choroidal vasculature, without evidence of disruption of the outer or inner retinal layers and the retinal pigment epithelium (RPE). FAF (fig. 3) revealed a hyperautofluorescent area corresponding with the mass.
Ultrasound B-scans failed to show any elevated lesion or thickening of the 'coats' since conventional B-scans cannot separate choroidal from scleral layers. Also, there was no evidence of the sub-Tenon fluid and the T-sign was negative.
The common systemic causes of a choroidal granuloma were tested for sarcoidosis (serum angiotensin-converting enzyme and calcium) and tuberculosis (QuantiFERON Gold), and both were negative. Other less common causes of SIC such as Toxocara, Brucella and Wegener's disease were excluded either by anamnesis and/or additional tests. Also, inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) were within normal limits and the chest X-ray showed no abnormalities. Taking the funduscopic appearance and the image characteristics on the OCT and FAF into account, the diagnosis of SIC was made.
|
fundus autofluorescence, optical coherence tomography, solitary idiopathic choroiditis
| Not supported with pagination yet
| null |
PMC5336629_01
|
Female
| 75
|
Female patient aged 75 years who had no history of chronic disease apart from depression presented at outpatient clinic with complaints of lassitude, loss of appetite, exertional dyspnea for 4 months, and dry coughing for 2 months. Her examination revealed marked decrease in respiratory sounds at basal segments of the right lung. Hematological values, sedimentation, glucose, urea, creatinine, sodium, potassium, total protein, and albumin levels were within normal limits. Levels of alanine aminotransferase (189 U/L), aspartate aminotransferase (113 U/L), cholesterol (251 mg/ dL), and lactate dehydrogenase (LDH) (193 U/L) were also measured. X-ray graphy revealed a dark shadow, consistent with pleural fluid masking the demarcation line between mediastinum and pleura at the inferior zone of the right lung (Figure 1). Thoracic computed tomography (CT) revealed multiple sites of lymphadenopathy, the largest containing dense calcification 22 mm in size, and marked pleural effusion on the right side (Figure 2). Milky fluid with high density sampled from pleural effusion did not precipitate after centrifugation, and contained TG (1072 mg/dL), cholesterol (173 mg/dL), LDH (148 U/L), and albumin (2.7 g/ dL) at indicated concentrations. Sudan staining disclosed presence of fat globules. Lymphocyte dominancy was also detected in pleural fluid. Sputum ARB -negativity was detected 3 times, and bronchoscopy of the patient did not reveal any endobronchial pathology. Pleural biopsy was performed. Bronchial lavage and bronchoscopic biopsy results indicated benign condition of chronic pleuritis. Positron emission tomography-CT was obtained to rule out mediastinal tumor. During lymphatic scanning performed to detect etiology of chylothorax, images taken at second, fourth, and sixth hours demonstrated passage of radiographic contrast substance through bilateral inguinal, parailiac, paracaval, and paraaortic lymph nodes, and accumulation of radioactivity in these lymph nodes at popliteal, femoral, and pelvic regions, respectively. On delayed thoracic images, no uptake of radioactivity apart from background activity was observed. Images suggested possible thoracic duct obstruction. Mediastinal lymph node biopsy performed at external center revealed presence of granulomas demonstrating caseous necrosis. Abdominal ultrasound was normal. Antituberculosis treatment was initiated. Symptoms regressed with treatment, and level of liver enzymes decreased. At sixth month, marked regression in pleural effusion was observed (Figure 3).
|
chylothorax, lymphadenitis, tuberculosis
| Not supported with pagination yet
| null |
PMC5758717_01
|
Female
| 44
|
A 44-year-old woman was referred to the gastroenterology practice for evaluation of long-standing heartburn refractory to proton pump inhibitor (PPI) therapy. Her 24-hour multichannel intraluminal impedance-pH study on dexlansoprazole 60 mg daily revealed increased esophageal acid exposure (5.7% total time spent with esophageal pH <4) and a total of 15 impedance reflux episodes (10 acidic, 5 weakly acidic). High-resolution esophageal manometry with impedance (HREMI) showed normal esophageal function with 100% bolus clearance. HREMI revealed a sliding hiatal hernia of approximately 2 cm, which was confirmed on endoscopy. Biopsies were negative for eosinophilic esophagitis. Due to her persistent symptoms and high daily dosage of antacids, she underwent robotic-assisted laparoscopic hiatal hernia repair and Nissen fundoplication, during which the anterior and posterior vagal nerves were identified and preserved. Her postprocedure recovery was unremarkable, and she was discharged on the second day after the procedure. She was readmitted on 7 days later due to dehydration with symptoms of nausea, vomiting, and coughing. During this admission, she was hydrated and her symptoms improved. However, she gradually developed postprandial weakness and was eventually admitted 5 months postsurgery with diaphoresis, dyspnea, and palpitations occurring 2 hours after meals. Upper endoscopy demonstrated an intact wrap (Figure 1). A barium swallow revealed a normal post-fundoplication appearance. Interestingly, gastric emptying scintigraphy revealed rapid gastric emptying (Figure 2) with only 7% solid meal left in the stomach after 1 hour, 3% after 2 hours, and 1% after 4 hours (<30% after 1 hour is considered abnormal). Small bowel transit was normal, with 60-70% accumulation of the radiotracer bolus in the ileocecal region at 6 hours. As a result of her symptoms and objective rapid gastric emptying, the patient was diagnosed with dumping syndrome. Dietary modification with a low-carbohydrate diet and separating solids from liquid ingestion was instituted. The patient reported improvement of symptoms with these dietary modifications during her next follow up clinic visit 2 months later.
| null | Not supported with pagination yet
| null |
PMC5758717_02
|
Male
| 70
|
A 70-year-old man presented with a 10-year history of heartburn, acid brash, and hoarseness. He was placed on oral pantoprazole 40 mg twice daily and oral ranitidine 150 mg at bedtime with some improvement of symptoms. In addition, he had been experiencing episodes of chest pain for 1 year that were attributed to acid reflux. His barium esophagram demonstrated esophageal dysmotility and a moderate hiatal hernia with evidence of gastroesophageal reflux. This was verified with a 24-hour multichannel intraluminal impedance-pH study performed while the patient was taking oral pantoprazole 40 mg twice daily and oral ranitidine 150 mg at bedtime, demonstrating increased esophageal acid and non-acid exposure (16 acidic episodes, 132 weakly acidic) and a Johnson-DeMeester score of 35.6 (normal <14.7). HREMI revealed a decreased lower esophageal sphincter pressure of 7.5 mm Hg (normal 13-43 mm Hg) with evidence of a hiatal hernia. Esophagogastroduodenoscopy showed a 4-cm hiatal hernia and esophagitis without evidence of Barrett's esophagus on distal esophageal biopsy. The patient underwent a robotic-assisted hiatal hernia repair with Toupet fundoplication without major complications. During his second postoperative visit, approximately 4 weeks after his surgery, he noted 3 episodes of sudden-onset lightheadedness, hunger, tachycardia, perspiration, and diarrhea shortly after eating. Due to his history of diabetes mellitus type 2, he was able to check his blood glucose and found himself to be hypoglycemic after these episodes. He was given recommendations to eat small, frequent meals with complex carbohydrates (e.g., oatmeal, brown rice) and to avoid sugar and caffeine. Stool studies were also negative. Despite keeping a strict log of his diet, he continued to experience his previously described episodes. He was prescribed oral dicyclomine 20 mg 3 times daily with mild improvement of his diarrhea; however, he continued to have similar postprandial episodes. After a few weeks, his diarrhea returned, and he discontinued the dicyclomine. He was started on oral acarbose 25 mg 3 times daily before meals with significant improvement in his symptoms. At 1 year postsurgery, his symptoms remain controlled.
| null | Not supported with pagination yet
| null |
PMC6532328_02
|
Male
| 14
|
After the patient admitted to having concealed a serious bone disease from his treating physicians, so as not to jeopardize the execution of his desired orthodontic-surgical treatment, several medical treatment documents could be requested, from which a complex, long-term illness could be traced. The patient had already been diagnosed with OI as a 14-year-old. Since the sixth month of life, he had experienced about 20 fractures until the establishment of the pediatric diagnosis. The medical documents showed that he had already been classified as OI type I due to his medical history, proof of blue sclera, thoracic kyphosis, and DI. The diagnosis was made simultaneously with his 18-year-old brother, who also showed DI but was less severely affected as far as the number of bone fractures is concerned. However, after the diagnosis, a BP medication was not immediately started. At 21 years of age, the patient received more than 1 year of intravenous (IV) BP therapy with Aredia (pamidronate, 30 mg) every 3 months for the prophylaxis of further fractures. Thereafter, the medication was changed to weekly Alendron (alendronate, 70 mg) for 1 year. From the age of 24 to 29 years, the patient received IV Bonviva (ibandronate, 150 mg, once a month). Indeed, the patient discontinued his current BP medication only for a short period of time after the completion of pre-surgical orthodontics. That means, this measure ("drug holiday") was effective only during the short period of consecutive orthognathic surgical procedures. After completion of surgery, the patient restarted his antiresorptive medication after consultation with the treating orthopedist. Since the age of 29 years, the patient has been treated with IV Prolia (denosumab, 60 mg) every 6 months.
The extractions of four premolars and consecutive wound healing proceeded inconspicuously according to the stage.
The very late knowledge about the patient's bone disease and his current medication led to the decision to control the operative procedure and, therefore, to proceed in individual steps. In our opinion, there was an increased risk of bleeding during and after the procedure. In addition, given the long-term use of BP medication, the capacity for bone healing was not reliable. For this reason, a two-stage procedure was performed in the osteotomies of the jaws. After coordination with his orthopedic doctor, he was told to interrupt the medication.
Surgical therapy took place about 1 year after the beginning of orthodontic therapy (patient age: 26 years). For the surgical correction of jaw positions, an osteotomy was performed at the Le Fort I level with a saw and chisel. After a downfracture of the detached maxilla was performed, the maxilla was mobilized and posteriorly impacted, and the occlusal splint was incorporated. Osteosynthesis of the segment was done with four miniplates. The displacement of the maxilla caused it to rotate posteriorly, which improved the overbite. By correcting the tilted occlusal plane, a unilateral open bite resulted on the left side. For postoperative stabilization, a composite buildup was installed on the first and second molar of the lower left side and was left in place until the second surgical step. The anterior crossbite remained and was corrected by the following mandibular procedure.
Vertical oblique osteotomy of the mandibular ramus combined with advancing genioplasty was performed 3 1/2 months after the maxillary procedure. This procedure resulted in setback of the mandible and shortening of the ramus on the left side. The right side only received rotation of the ramus with minimal setback. The following advancement of the chin was symmetrical, because the correction for panfacial asymmetry was already performed by jaw surgery. After the mandibular osteotomy, the stabilization resulted from MMF via the orthodontic appliances and integrated splint. The second splint had three perforations, each 1 cm2 in size, for easier breathing and allowing liquid nutrition. This second procedure also proceeded without major bleeding. In this case, the intraoperative procedure gave the impression that, without the knowledge of the underlying disease, the operative course of the procedure would not have differed from that of a patient not suffering from OI.
The surgical treatment documentation is shown in Figure 1 (Fig. 1), Figure 3 (Fig. 3), Figure 4 (Fig. 4), Figure 5 (Fig. 5), and Table 2 (Tab. 2).
Tissue samples of the ablated maxilla in the osteotomy line showed normal bone formation in the histological examination.
The last follow-up of the patient took place 3 years after the second surgical procedure. At this time, the patient had a stable class I occlusion, no signs of osteonecrosis, and radiologically unremarkable healed osteotomies of the jaw. The patient reported that he had suffered further fractures of the extremities after completion of orthognathic surgery and orthodontic procedures while continuing to receive denosumab medication. The skeletal function of the jaw had remained stable and uncomplicated since the orthodontic-surgical measures.
The literature review (Table 4 (Tab. 4)) included 27 reports of jaw osteotomies in patients with OI, including the present case (mean age: 22.59 years). Thirteen women and 12 men were treated (no information: two patients). The age of the women at the time of treatment was on average 23.38 years (range: 17.5 to 36 years) and that of men was on average 22.5 years (range: 12 to 40 years).
The diagnosis of OI was certain in all cases (Table 1 (Tab. 1)). Typing of OI was missing for seven patients. In another case, the typing could not unambiguously distinguish between two classes. OI type I was the predominant diagnosis (14 patients), followed by a few cases that had either OI type III (three patients) or type IV (two patients). None of the reports on orthognathic surgery in OI patients refer to an OI severity grading system when describing the individual phenotype of the respective patient(s). However, orofacial or maxillofacial findings are not an assessment factor for a recently proposed severity classification.
A Le Fort I osteotomy was chosen as access to the skull base solely in order to treat basilar impression in a report detailing treatment of four patients. The report mentioned some maxillary advancement combined with spine surgery in one of these cases. Transposition of the divided jaws was carried out in all other cases or at least intended.
In 13 cases, only one jaw was osteotomized (women: 4, men: 9). Both jaws were osteotomized in 14 cases, of which two were temporally separate procedures for the same respective jaw.
Osteotomies at Le Fort I level were made in 21 patients (women: 11, men 8, unspecified: 2). Lower jaw osteotomies (including genioplasty) were performed in 20 patients (women: 8, men: 10, unspecified: 2). Maxillary osteotomies were performed in all to achieve sagittal advancement of the maxilla.
In the lower jaw, the osteotomy aimed to relocate the corpus. Sagittal osteotomy of the mandibular ramus was the most frequently used technique (10 cases), followed by vertical ramus osteotomy (5 cases) and osteotomy within the mandibular corpus (3 cases). From the reports, it can be derived that the osteotomy of the lower jaw was used for setback of the corpus.
The osteosynthesis material corresponds to the developments of the procedures over the long period in which the recorded reports have been carried out. Wire osteosynthesis, which was previously used, has been replaced almost everywhere in later operations by miniplate osteosynthesis. However, a recent report shows that occasionally wire osteosynthesis is still used, combined with screw osteosynthesis. While some reports highlight the fragility and narrowness of bones, none of the reports on OI patients subjected to orthognathic procedures appear to have another fracture outside the selected osteotomy line that would have required surgical care.
BP medications are mentioned in three reports and are listed in detail for our own case. All other reports mention no such medication. For OI patients whose osteotomies were performed before the year 2000, it is assumed that BP medication is unlikely. In one case, BP medication was only mentioned and not specified. In another case, monotherapy with alendronate was performed; and in our own case, several medications of this type and another drug with similar activity were used before and after treatment. A short phase of interruption of medication for the operative treatment phase was known for two of the three cases. The fourth report was about successful orthognathic surgery on an OI patient. In this case, only a small amount of treatment information was found in the brief communication.
OI diagnosis had an impact on planning of the surgical strategy for a variety of reasons. The timing of the diagnosis had also been an important factor in therapy decisions. In one case, referring to the increased risk of complications during osteotomy, the surgical concept was adapted whereby the incomplete dentition allowed for prosthetic compensation of the unbalanced jaw relation. In another case, distraction of the segment was performed in the upper jaw after osteotomy instead of immediate transposition for the same reasons, as to avoid bleeding complications. In a third case, the bimaxillary operation was divided into two separate procedures, one jaw osteotomized each to minimize blood loss during and after surgery. In this case, for religious reasons, the patient had already refused transfusion of blood prior to surgical treatment. Despite these precautions with a long interval between interventions (7 months), perioperative hemorrhage occurred at the second procedure, necessitating omission of bone grafting. In our own case, the diagnosis of OI combined with the knowledge of pharmacotherapy led to the decision to osteotomize each jaw individually in one operation and to wait for a longer period of time between the procedures. These two patients are the only bimaxillary osteotomies in this review that were planned as two-time interventions. Another report indicates that previously unknown OI was noticed in two cases by the surgeon during osteotomy of the jaw due to significant brittleness of the bones. This finding has led to the adaptation of orthognathic intervention planning. In both cases, the osteotomy of the second jaw was omitted. Intraoperative suspicion of OI or very late knowledge of the disease is therefore available for at least three out of 27 patients.
Surgical complications, such as severe bleeding from the surgical situs, have been reported only for patients in whom no administration of BP medication was known or suspected. These reports described incidents more than 20 years ago. The brittleness of the bones and the difficult osteotomy are emphasized in individual reports.
|
bisphosphonate, oral surgery, orthodontics, orthognathic surgery, osteogenesis imperfecta
| Not supported with pagination yet
| null |
PMC3745100_01
|
Female
| 0
|
We present a case of domestic neonatal scald burns in an 11-day-old neonate, who sustained major scald burns in her lower limbs, trunk, and both upper extremities when the hot water bag, being used by her mother for shoulder pain accidentally burst while feeding the baby [Table 1]. The child was immediately rushed to a nearby hospital where primary fluid resuscitation was provided by a pediatrician and referred to higher center for definitive management. The child arrived at our institution within 3 h of sustaining burns.
The neonate was alert and excessively crying on arrival. Vital signs were within normal limit as determined by the neonatologist involved in management. Burn assessment for total body surface area (TBSA) was done using Lund and Browder Chart and was found to be 51%. At preliminary assessment, we found around half, that is, 50% of the involved area to be second degree superficial and rest second degree deep burns. A peripheral venous access was obtained and samples for routine blood investigation and swabs were taken. Fluid calculation was done according to Galveston's formula; dextrose containing solutions were added to the regimen as per the need. The target urine output of 1.5-2 mL/kg/h was strictly monitored and fluid adjustments made accordingly. For prevention of hypothermia, the neonate was dressed with occlusive dressings and covered by a blanket, and hourly monitoring of rectal temperature was done in a pediatric intensive care unit (ICU). Antibiotics and analgesics were started according to the body weight after consultation with the neonatologist. Strict input-output charting was maintained; vital parameters of heart rate, pulse, temperature, and pulse-oximetry were monitored hourly. The urine output too was monitored by weighing the diapers every hour. The serum electrolytes were monitored daily [Figure 1].
On the second day of admission, following resuscitation, the patient was taken onto operative room and under general anesthesia, the burn wound was cleaned with normal saline and polyurethane sheets were applied and dressed with sterile paraffin tulle gras.
The vitals of the patient remained stable for 3 days. During the course of treatment, the mother was constantly encouraged to breastfeed the baby. On the 4th day, the baby refused breastfeed, developed hypothermia, and urine output decreased. With suspicion of septicemia, the patient was immediately assessed by neonatologist and was placed on ventilator support for tachypnea and deterioration of vital parameters. A central venous catheter was placed and antibiotics were changed. Antifungals were added to the treatment, all routine investigations were sent including C-reactive protein, blood culture, and swab for culture and sensitivity from the wound. The patient was fed with expressed milk from the mother through a nasogastric tube. The patient improved after 2 days of intensive management and was weaned from the ventilator on the 7th day of admission.
During this period, there was no soakage or foul smell from the dressed wound site to indicate that the dressings need to be changed immediately. Antibiotics were changed after obtaining reports of culture sensitivity.
Dressings were changed on the 8th day and it was found that majority burns had healed except for about 10% over the abdomen and lower limbs. Polyurathane sheets were reapplied over rest of the areas and dressings were placed. Subsequently, dressings were done on every alternate day continuing the general care and nutrition of the baby [Figures 2 and 3].
The wound showed signs of healing, but around 10% of raw area remained over the lower limbs and anterior abdominal wall after 2 weeks that was considered for definitive coverage by operative procedure. The father was counselled for homograft donation for the child as the mother had herself sustained 10% scald burns over her back, gluteal region, and thighs from the accident, and the donor areas were unavailable for the neonate. On the 22nd day of admission, after normalization of all parameters of the baby and preparation of the wound bed by dressings, homograft was applied for the remaining raw areas. The graft-take was complete and the wounds healed completely. Subsequently, the neonate was discharged with advice for regular follow-up [Figure 4].
|
accidental, neonate, scald burns
| Not supported with pagination yet
| null |
PMC4900195_01
|
Male
| 22
|
The patient was a 22-year-old starting NCAA division I wide receiver. He was 6'5", weighed 225lb, and had a BMI of 26.7. After a 12-month history of intermittent low back pain, he presented to his team physician with a complaint of increasing generalized lumbar discomfort. He had been previously diagnosed with sacroiliac joint dysfunction, which had been managed with physical therapy until this recent exacerbation of symptoms. Physical examination revealed paraspinal muscle discomfort on forward flexion, tight hip flexors and hamstrings, and weak hip abductor muscles. Plain radiographs of the lumbosacral spine demonstrated six lumbarized vertebral bodies with lumbarization of S1 and mild degenerative disc changes at L4-5 (Fig. 1).
The patient was managed conservatively for almost 3 months, until he developed acute pain in his right buttock and upper posterior thigh, right-lower-leg numbness, and right-leg weakness. Physical examination revealed slightly diminished strength of knee flexion, ankle dorsiflexion, and plantar flexion in the right leg. A selective nerve-root block at L5 gave him sufficient pain relief to continue his football activities, but his leg weakness persisted over the next two months. A MRI scan demonstrated right paracentral herniation of the L4-5 disc, with a small annular tear associated with a cyst extending from the L4-5 level down to the L5-S1 level (Figure 2, Figure 3). The patient was referred for surgery.
After a right L4-5 laminotomy, lateral recess decompression, and wide foraminotomy, retraction of the L5 nerve root exposed a disc herniation with an associated large discal cyst that extended into the right neural foramen and inferiorly behind the L5 vertebral body. The cyst was decompressed; it contained very thick fluid with several fragments of disc material. After the cyst was resected, microdiscectomy revealed only small amounts of herniated disc material. The disc space was then flushed out with an antibiotic solution, and no further disc material was identified. Postoperatively, the patient's symptoms resolved, and he presented with intact motor strength at his 12-day followup visit.
| null | Not supported with pagination yet
| null |
PMC3108789_01
|
Female
| 34
|
A 34-year-old female with systemic lupus erythematosus was admitted due to progressive lower leg edema, massive ascites, and nausea after treatment of bed rest, salt and water restriction, diuretics, intravenous administration of albumin and methylprednisolone, and repeated paracentesis for 2 months. On physical examination, her blood pressure was 127/82 mm Hg, pulse rate 84 beats/min, respiratory rate 20 breaths/min, and body temperature 37 C. Her abdomen was markedly distended and her lower extremities were severely edematous. The results obtained in laboratory investigations were as follows: white blood cells (WBCs) 5.8 x 109/L (normal range 4.5-11 x 109/L), hemoglobin 89 g/L (normal range 120-160 g/L), platelets 220 x 109/L (normal range 150-350 x 109/L), serum blood urea nitrogen (BUN) 13.5 mmol/L (normal range 2.5-7 mmol/L), creatinine 170 mumol/L (normal range 50-110 mumol/L), albumin 19 g/L (normal range 37-53 g/L), alanine aminotransferase 0.24 mukat/L (normal range 0-0.66 mukat/L), aspartate aminotransferase 0.60 mukat/L (normal range 0.08-0.76 mukat/L), C-reactive protein 0.286 mg/dL (normal range 0-0.5 mg/dL), complement 3 (C3) 0.4 g/L (normal range 0.8-1.5 g/L), complement 4 (C4) 0.1 g/L (normal range 0.2-0.4 g/L), and double-strand DNA (dsDNA) 263 IU/mL (normal range <30 IU/mL). Urine protein excretion was 4.86 g/day. Chest X-ray showed massive bilateral pleural effusion. The WBC count of ascitic fluid was 10/mm3. The serum-ascites albumin gradient was 1.4 mg/dL. The results of bacteria culture, acid-fast stain, malignant cell, and tuberculosis- polymerase chain reaction of ascites were negative. The Doppler of the main portal vein, inferior vena cava, major portal branches, and hepatic vein were patent without obstruction. The abdominal computed tomography scan revealed massive ascites (Figure 2A). The echocardiogram showed no pericardial effusion and preserved left ventricle systolic function. Unfortunately, progressive massive ascite accumulation gave rise to breathing difficulty and decreased urine output, despite an increase in serum albumin from 19 g/L to 28 g/L after the aforementioned treatments for 1 month at the hospital. The serum BUN and creatinine levels were increased to 30.5 mmol/L and 720 mumol/L from 13.5 mmol/L and 170 mumol/L, respectively. Disseminated intravascular coagulation (DIC) was within a normal range. Her lupus activity did not respond to plasma exchange, methylprednisolone 1000 mg pulse therapy, oral prednisolone 60 mg daily, or cyclosporine 100 mg twice daily. Her renal failure, leg edema, and refractory ascites could not be controlled by HD due to frequent intradialytic hypotension, which could not be corrected by prophylactic fresh frozen plasma infusion, sequential ultrafiltration, and reduction in dialysate temperature during HD. Therefore, continuous flow control reinfusion of ascites into a dialyzer during HD was designed to alleviate the ascites. A paracentesis pigtail catheter was inserted into the abdominal cavity and connected to a sterile three-way stopcock. When ascite reinfusion was planned, a set of dialysis tubing was connected with the sterile three-way stopcock to draw out the ascites at the speed of 12-15 mL/min (720-900 mL/h) into the dialyzer to mix with the blood by the roller pump during HD. The mixed blood and ascites were then channeled into the systemic circulation during a 4-hour HD session (Figure 1A). The ultrafiltration rate of the dialyzer was maintained at around 1 L/h to allow fluid removal from the blood and infused ascites in each 4-hour HD session. After seven sessions of continuous reinfusion of ascites into the dialyzer during HD, ~20 L of ascites were drawn out. The body weight and abdominal girth decreased from 50.2 kg to 37 kg and from 92 cm to 72 cm, respectively (Figure 3). The serum albumin level increased from 30 to 35 g/L (Figure 3). There was no intradialytic hypotension, fever, gastrointestinal bleeding, or DIC. Her respiratory distress and general condition also improved after the procedures. The levels of complements C3 and C4 also increased from 0.4 g/dL to 0.7 g/dL and from 0.1 g/dL to 0.13 g/dL, respectively. After the ascites subsided, the paracentesis pigtail catheter was removed. A piece of omentum was obtained by peritoneoscopy for the evaluation of the cause of refractory ascites. The pathologic finding of the omentum was chronic inflammation with cell infiltration, congestion, and fibrosis (Figure 4).
In the following 60 months, the serum creatinine level was around 120 mumol/L on immunosuppressive agents. There were no further episodes of refractory ascites. The patient also had an increase in muscle mass.
|
ascites, lupus, reinfusion, renal failure
| Not supported with pagination yet
| null |
PMC9701777_01
|
Female
| 28
|
A 28-year-old woman presented with "rosacea" of 2 years' duration for which her previous physician had prescribed daily use of triamcinolone 0.1% cream. She complained of swelling and a worsening situation of her rosacea each time she discontinued it. She went to visit another doctor who prescribed tacrolimus and crisaborole ointment to treat her. She insisted the prescription was making the symptoms worse. Increasing involvement of the face and suffering feelings were causing her significant pain. When she first came to our visit, she reported intermittent itching (Figure 1A) (peak pruritus was 6 of 10 on a numeric rating scale). She was given the hydroxychloroquine (0.2g twice daily), minocycline (50mg/d) and topical metronidazole gel for 2 weeks. However, the patient reported severe itching (peak pruritus was 9 of 10 on a numeric rating scale). Her face was more swollen and irritable (Figure 1B). Given the progressive disease, the ineffective routine plan and the patient's associated concern, we decided to pursue a more aggressive strategy. We were looking for a new anti-inflammatory drug. An article published on JAAD which referred to JAK/STAT signaling pathway contributing to multiple inflammatory dermatoses inspired us to treat her with JAK1/3 inhibitors tofacitinib after she had undertook blood routine examination, biochemistry analysis, coagulation function and chest CT to exclude severe infection, coagulation disorder, hepatic failure, renal disfunction and tuberculosis. Treatment with tofacitinib was initiated at a dosage of 5 mg twice daily. After 2 weeks symptoms relieved a lot, swelling on her face reduced (Figure 1C). The dosage was decreased to 5 mg/d for the next 2 weeks. As we can see, only perioral erythema remained and the patient reported middle itching (Figure 1D) (peak pruritus was 4 of 10 on a numeric rating scale. Then, the dosage was decreased to 5mg every other day for 2 weeks. She kept on using the topical metronidazole gel during the whole process. Finally, the skin lesions almost disappeared completely (Figure 1E) with occasionally itching (peak pruritus was 1 of 10 on a numeric rating scale and her mental health improved. Course of drug treatment suspended. She discontinued tofacitinib for 12 weeks without relapse of the eruption (Figure 1F). No adverse effects were detected.
|
jak inhibitors, steroid-induced rosacea, tofacitinib
| Not supported with pagination yet
| null |
PMC3296115_01
|
Male
| 2
|
A 2 year old boy was presented with a swelling of right middle metacarpal for last 4 months. The swelling was initially small gradually increasing in size. It was associated with dull aching pain for the last 3 months. There was no history of any trauma. However, h/o exposure to pulmonary tuberculosis was present.
On examination, a semi tubular swelling 5cm *2cm was noted over right 3rd metacarpal. The swelling was bony hard, fixed to underlying bone, tender and local temperature slightly raised.
A radiograph of the right hand showed a cystic expansile lesion of right 3rd metacarpal. The margins were well defined with internal septations and associated cortical sclerosis. There was cortical destruction but no periosteal reaction.
A provisional diagnosis of enchondromata was made, while tuberculous dactylitis was kept in mind. Differential diagnoses were osteoblastoma, syphilis. The boy was recommended for surgery and excision of 3rd metacarpal was done followed by strut graft from ipsilateral fibula. The specimen sent for HPE showed epitheloid granuloma suggestive of tuberculosis. Mantoux test was positive and sputum for AFB also revealed positive result (Figs. 1, 2).
|
tuberculous dactylitis, spina ventosa, spindle shaped expansion, tuberculous granuloma
| Not supported with pagination yet
| null |
PMC7181516_01
|
Male
| 14
|
A 14-year-old African-American male with hyperthyroidism and intermittent asthma from Long Island, New York presented with acute onset of bilateral leg weakness. The patient reported experiencing bilateral leg soreness after swimming 2 days prior to presentation but denied particularly intense activity. The soreness progressed to generalized weakness and pain. Upon wakening to go to the bathroom in the early morning, the patient found himself unable to bear his own weight. The patient otherwise denied recent fevers, shortness of breath, cough, congestion, nausea, vomiting, diarrhea, rash, headaches, or visual disturbances. Recent diet was not ascertained, however the patient reported eating his usual dinner the night prior to the onset of symptoms.
With regards to his past medical history, he was diagnosed with reactive airway disease at 8 months of age. He was well controlled on inhaler therapy with budesonide and albuterol and he had recently required rescue albuterol after experiencing chest tightness while playing football. Three months prior to this presentation he was evaluated for the complaints of fatigue, tremors, palpitations, heat intolerance, difficulty focusing in school, and weight loss without any history of muscle weakness. His physical examination was remarkable for a nontender goiter and proptosis. Subsequent bloodwork revealed a thyroid-stimulating hormone (TSH) level less than 0.01 mIU/L (normal 0.52-5.05 mIU/L) and an elevated T4 level of 30.4 mcg/dL (normal 4.84-10.13 mcg/dL), consistent with the diagnosis of Graves' disease. After a pediatric endocrinology consultation, he was prescribed methimazole (5 mg every morning, 10 mg every evening) and 25 mg atenolol daily. He was also instructed to avoid physical activity until his thyroid hormones had declined. He reported good medication compliance until approximately 2 weeks prior to current presentation at which point he ran out of medication at home and failed to refill the prescriptions. Family history was only significant for a maternal grandmother with hyperthyroidism.
On presentation to the emergency department the patient's vital signs were notable for tachycardia (109 bpm) and an elevated blood pressure (154/87 mmHg). Physical examination was remarkable for bilateral lower and upper extremity weakness with lower extremity areflexia and upper extremity hyporeflexia. Initial laboratory tests revealed a potassium level of 2.0 mmol/L (normal 3.4-4.7 mmol/L) and a TSH level less than 0.005 mIU/L. Electrocardiogram demonstrated normal sinus rhythm, no ST elevation, and positive U waves. Initial concern for intracranial hemorrhage or other central nervous system abnormalities were ruled out following normal imaging of the brain, including computed tomography scan and magnetic resonance imaging. The patient was given 40 meq potassium oral replacement therapy, 1 L of normal saline mixed with 20 meq of potassium, and 1 g of magnesium before being transferred to our hospital.
On arrival to the pediatric intensive care unit, the patient appeared comfortable and was answering questions appropriately. His muscle weakness had dramatically improved, however he continued to remain tachycardic (117 bpm) and hypertensive (128/70 mmHg). Muscle power in the proximal right lower extremity was appreciated to be 4/5, while full muscle power was appreciated in all the other extremities. Deep tendon reflexes in bilateral lower and upper extremities were 1+ and 2+ respectively. Cranial nerves II-XII were grossly intact and the rest of the neurologic examination was otherwise unremarkable. A thyroid function panel was obtained and demonstrated a TSH level of less than 0.005 mIU/L, T3 level greater than 651 ng/dL (normal 110.02-184.88 ng/dL), T4 level of 16.8 mcg/dL, and a free T4 level greater than 7 ng/dL (normal 1.03-1.77 ng/dL). Repeat basic metabolic panel revealed an improved potassium level of 4.7 mmol/L and magnesium level of 1.8 mg/dL. The pediatric endocrinology team advised re-initiation of 20 mg methimazole two-times daily and 20 mg propranolol three-times daily. His clinical status rapidly improved and he was discharged home on the second day of admission.
Upon follow-up the patient reported good medication compliance with no further episodes of weakness or worsening of pulmonary symptoms. Because he was asymptomatic, the propranolol was discontinued after 1 month and methimazole dosing was decreased to 10 mg daily after 3 months. Six months after discharge the patient remained clinically euthyroid. His thyroid hormonal profile revealed normalization of T4 (7.6 mcg/dL) however, he continued to have a low TSH (0.02 mIU/L) and an elevated thyroid stimulating immunoglobulin (400%; normal < 140%). He was instructed to continue methimazole 10 mg daily and to obtain repeat thyroid function tests prior to the next appointment in 3 months.
|
adolescent, hyperthyroidism, hypokalemia, thyrotoxic periodic paralysis, thyrotoxicosis
| Not supported with pagination yet
| null |
PMC9691660_02
|
Male
| 7
|
A 7-year-old boy was admitted to our emergency department after losing consciousness for an hour. The boy suffered a sudden cardiac and respiratory arrest 1 h ago while exercising at school. He was immediately given external chest compression and defibrillation used AED equipped in the school. As a result, his heartbeat and respiration recovered. When the boy was sent to our hospital, he was conscious and agitated which was alleviated by sedation and intracranial pressure reduction. His vital signs were measured: heart rate 124/min; respiratory rate 25/min; blood pressure 102/55 mmHg. On his physical examination, the liver and spleen were not touched under the ribs and there was no edema in the lower limbs. The boy and his parents were in generally good health and other family members denied a history of syncope, sudden death or cardiomyopathy.
There were no obvious abnormalities in the brain MRI and video electroencephalogram (VEEG). The electrocardiogram (ECG) revealed Ptf-V1 < -0.04 mm s and biphasic P waves, indicating left atrial enlargement and the chest X-ray revealed an enlarged heart shadow. The echocardiogram showed biatrial enlargement, especially in the left atrium (LA: 32 mm, RA: 30 mm), decreased diastolic function (E/A = 2.6; E Peak deceleration time 130 ms; Constant volume diastolic time 49 ms), moderate mitral valve regurgitation and left ventricular ejection fraction of 51.8%. Except for bilateral enlargement, cardiac magnetic resonance (CMR) indicated no abnormalities (see Figure 1). The ECG at the time of the AED discharge indicated ventricular fibrillation (see Figure 2). Troponin T and brain natriuretic peptide (BNP) levels were normal.
Genetic analysis revealed the boy had a heterozygous missense mutation [c.611(exon8)G>A,p.R204H] of TNNI3 which was not detected in his biological parents and de novo (see Figure 3). This mutation located in exon 19 of TNNI3 which was conserved. We analyzed the mutation using bioinformatics protein function prediction software PolyPhen_2 and Mutation Taster and found the variant is possibly detrimental. Common genetic mutations in arrhythmias such as SCN5A, KCNH2, KCNQ1, CACNA1C, RYR2, and LMNA were not found in the family members.
According to the results above, we concluded that the boy was a patient of primary RCM. At present, the vital signs of the boy are stable. We communicated with the parents and suggested installing an implantable cardioverter defibrillator (ICD) for the boy, but the parents refused. We have informed his parents that a heart transplant may be required if the disease progresses further. At present, the boy is being treated with oral diuretics and metoprolol tartrate tablets and he is in outpatient follow-up.
|
aed, tnni3, constrictive pericarditis, restrictive cardiomyopathy, ventricular fibrillation
| Not supported with pagination yet
| null |
PMC6826313_01
|
Male
| 28
|
A 28-year-old male presented with a chronic dry cough and weight loss of 1-year duration, and 2 months of worsening mid-thoracic back pain accompanied by bilateral lower extremity weakness. Notably, there were no known TB exposure, recent travel, smoking, or history of intravenous drug use. On the initial physical examination, he had bilateral rales and left lower extremity weakness (4/5).
Magnetic resonance imaging revealed an epidural collection causing compression of the spinal cord from T5 to T6; this was accompanied by a moderate-sized, left-sided pleural effusion, a left lingular/lower lobe consolidation, and multiple cavitary lesions in the right lung [Figures 1-4]. The patient was initially diagnosed with pulmonary TB/Pott's disease and myelopathy, resulting in the standard TB regimen of rifampin, isoniazid, pyrazinamide, and ethambutol.
Surgery included a T5-T6 decompression (e.g., costotransversectomy); the largely ventral mass (e.g., anterior to the cord) was removed. This was followed by open reduction and a posterior T4-T8 fusion.
A 1.2 cm x 0.9 cm x 0.6 cm bone tissue fragment from the T6 lamina and 3.0 cm x 2.0 cm x 1.0 cm aggregate from the T6 epidural tissue demonstrated metastatic adenocarcinoma, likely primary from the lung.
The patient's postoperative course was complicated by an inability to be weaned off the ventilator; the high oxygen requirements were attributed to the left lung disease. A chest radiograph performed the day of surgery demonstrated an enlarging left pleural effusion and a left pneumothorax. There were also patchy right perihilar airspace opacities/scattered nodules, considered infectious/inflammatory [Figure 5]. The patient subsequently experienced acute hypoxemic respiratory failure, acute respiratory distress syndrome, shock, and cardiac arrest. Of interest, the TB polymerase chain reaction came back negative.
|
lung adenocarcinoma, mycobacterium tuberculosis, pott’s disease, spine, thoracic, vertebra
| Not supported with pagination yet
| null |
PMC4491452_01
|
Male
| 38
|
We report a case of 38-year-old businessman who presented to the medical outpatient clinic of the Niger Delta Teaching hospital here in Nigeria, with a 2-week history of bloody ejaculate. He had 3 episodes with bright red blood noticed in the ejaculate. There was no associated history of urgency, urge incontinence or of dysuria. He was diagnosed hypertensive 5 months prior to onset of symptom but had not been regular with oral anti-hypertensives and follow-up clinic visits. There was no history of trauma to the genitals or prostate. No history of scrotal pain, no bleeding from any other part of his body, no weight loss, no chronic cough, no noticeable fever or drenching night sweats. He was not a known diabetic or sickle cell disease patient. He had been taking oral sildenafil intermittently for 2 years to enhance sexual performance. The patient had a positive history of unprotected sexual intercourse with current and previous girlfriends. His father and elder sister were known hypertensives. He drank alcoholic beverages occasionally but was a lifelong non-smoker. On examination, he was not in any obvious respiratory or painful distress, not pale, anicteric, afebrile to touch, acyanosed, no digital clubbing, no significant peripheral lymph node enlargement, and no dependent oedema. There were no signs of Cushing's syndrome, acromegaly, or systemic sclerosis. He was conscious and alert, well oriented in time, place and person, scoring 10/10 on the abbreviated mental test score. He had no obvious focal neurologic deficits. His arterial walls were thickened and locomotor brachialis was present. Radiofemoral delay and renal bruits were absent. His blood pressure was 200/120 mmHg. The apex beat was at the 6th left intercostal space, lateral to the mid-clavicular line, heaving. The first, second and fourth heart sounds were present, all of normal intensity with no murmurs heard. The respiratory rate was 20 cycles per minute. Respiratory system examination revealed no abnormalities. Abdominal examination was normal. Genito-urinary system examination including prostate examination was also normal. A diagnosis of severe hypertension and haematospermia was made.
Investigation results included a full blood count that revealed a haematocrit of 34% while other parameters were within the normal range. Serum chemistry showed increased urea levels (urea 11.5 mmol/l), and elevated creatinine (300/mol/l). His lipid profile revealed mildly elevated LDL cholesterol (3.4 mmol/l) whiles other lipid parameters were normal. His fasting blood sugar was normal. Immunological testing showed no evidenced of HIV or tuberculosis. Radiography of the chest showed cardiomegaly with left ventricular preponderance but no evidence of aortic coarctation. Electrocardiography showed sinus rhythm with increased P-terminal force (left atrial enlargement), T-wave inversion in leads II, III, aVF, V5 and V6, left ventricular hypertrophy (Sokolow Lyon voltage criteria SV1 + RV5 = 41 mm). Dipstick urinalysis showed trace glycosuria but no protein or blood. Urine microscopy revealed 0 - 3 pus cells per high power field but showed no casts. There was no significant bacterium growth on urine culture. Seminal fluid analysis revealed a brownish gelatinous fluid with a volume of 2.5 mL, pH 8.5 (7.2 - 7.8), numerous red blood cells, 0 - 5 pus cells per high power field, the spermatozoa count 10.2 x 106 ml with 30% being actively motile, sluggishly motile 10%, non-motile 60%, normal morphology 60%, abnormal morphology 40%. Seminal fluid culture did not have any significant yields. Fundoscopy was not done. Renal ultrasound scan, echocardiogram, prostate scan and prostate specific antigen were also ordered for but were not done on account of financial constraints. The patient was admitted and counseled on his clinical condition, lifestyle modification and DASH (dietary approach to stop hypertension) diet. His blood pressure was lowered gradually, over several days, with oral nifedipine control release tabs, tabs moduretic, and lisinopril and he discharged home after 2 weeks at which time his blood pressure was 150/100 mm Hg. An appointment for follow-up visit was made (cardiology and urology) for the following week. In follow-up visits, he complained that when his blood pressure was measured outside the hospital, it was significantly less than the values obtained at the hospital. He also had the problem of his sexual partners running scared and avoiding him once they discover the haematospermia. Some weeks after being on regular anti-hypertensives, the haematospermia resolved completely. He was however lost to follow-up, as his family members convinced him that his problems had been a "spiritual attack" and he resolved to seek further treatment at a church. About three months after last visit, he is said to have suffered a stroke while still at the church (this information was gotten from one of his relations as we made attempts to contact him). He is yet to be seen or heard from since.
|
haematopermia, severe uncontrolled hypertension, worried man
| Not supported with pagination yet
| null |
PMC6614786_01
|
Female
| 63
|
A 63-year-old woman with partial Situs Viscerum Inversus (without dextrocardia) was diagnosed with giant sliding hiatus hernia and cholelithiasis. The symptoms were typical: heartburn, chest pain, burping and atypical respiratory symptoms as coughing and asthma. The preoperative evaluations was: gastrointestinal endoscopy (EGD), a barium swallow radiography, an intraluminal 24-H pH measurement and oesophageal manometry, abdominal ultrasound, chest and abdominal CT scan with contrast. The diagnosis of Situs Viscerum Inversus was on CT scan exam, and previously unknown. Anatomical variation has to be identified in the preoperative study to avoid intraoperative risks of iatrogenic injures during dissection and exposure of the anatomical structures.
A robot-assisted reduction of hiatal hernia in abdomen and Toupet fundoplication was performed, a Bio A mesh was placed as hiatoplasty reinforcement and gastropexy (to reduce the post-operative recurrence risk); the cholecystectomy was performed as well as the patient was affected by cholelithiasis. The patient was discharged on third postoperative day after X-ray check and he tolerated a solid food. The operation technique in SVI is quite specular to the conventional technique for robot-assisted fundoplication. The patient was placed in French position, with the surgeon standing between the patient's legs and robotic cart from the head/right shoulder. We used a 4-arms cart with the 4th arm from the left side (Fig. 1).
Carbon dioxide pneumoperitoneum was established using Veress' needle technique; a 12-mm incision in the right abdominal quadrant area over-umbilical line was made to insert the trocar for the camera; the ports were placed in a configuration that was roughly the mirror image of our usual fundoplication procedure. The first 12 mm port for the camera was inserted into the abdominal cavity in right ipocondrium, about 2 cm above umbilical line and four more trocars were inserted. After the internal organs of abdominal cavity examination we proceeded with the insertion of three more trocars under direct view. Trocars were placed in epigastric region, left hypocondrium and left lumbar region. The other robotic trocar and auxiliary AirSeal trocar placed.
Cholecystectomy was first performed. The right hepatic lobe lifted to expose the hiatus. The procedure was carried out in the standard fashion with trans-hiatal dissection of the oesophagus for about 6 cm distally. Reduction of the stomach into the abdomen and the right and left crura exposed, a para-hernia lipoma removed and oesophagus encircled using the articulated tips of robotic instruments and distal oesophagus prepared for about 6-8 cm into the mediastinum, removing the hernia sac. The right side of stomach dissected, no typical short gastric vessels were found, as the spleen was represented by two fetal presentation with a vascular supply from the kidney vessels (Fig. 2).
The left pillar dissected from the cava, the hiatoplasty performed using three stitches with pledgets and a Bio A mesh placed and sutured above the crura plane. Closure of the crura and a posterior partial fundic wrap (Toupet technique) was done with 3/0 Prolene (Prolene, Ethicon Inc., Johnson and Johnson) stitches tied intra-corporeally with hand made pledgets, using pieces of BioA prothesis.
Finally two trans parietal 0-Vycril stitches for the gastropexy between antrum and abdominal wall. The gallbladder was removed with an Endo-bag through the 10-mm operating port; and a drain placed near the hiatal area. The total operative time was 190 min. The patient was discharged on third postoperative day after a contrast upper GI X-ray exam and an oral semi-solid diet.
The operation time (cholecystectomy and fundoplication) was 190 min with no intraoperative complications. The postoperative course was uneventful, and the patient was discharged on postoperative day 3.
|
cholecystectomy-case report, gastro oesophageal reflux disease, giant hiatal hernia, mesh placement, robotic surgery, situs viscerum inversus, toupet fundoplication
| Not supported with pagination yet
| null |
PMC9637682_01
|
Male
| 42
|
A 42-year-old male with a reported history of resolved hepatitis C was admitted to hospital due to fever and sore throat for 9 days, combined with a generalized rash over the entire body for 5 days.
The patient had not taken new medications in the recent 2 months until 8 days prior to this admission, when the patient visited a local clinic for flu-like symptoms of fever, sore throat, and general malaise combined with muscle weakness and was prescribed non-steroidal anti-inflammatory drugs (NSAIDs):mefenamic acid and diclofenac:for 3 days with acetaminophen and famotidine. However, the fever persisted despite the treatment, with subsequent development of itchy erythematous macules and papules spreading from the face and trunk to the extremities 5 days prior to this admission. Associated symptoms of ophthalmalgia, conjunctival injection, and lip swelling were also noted. After revisiting the local clinic and obtaining an additional prescription for ibuprofen, he visited the emergency department of another hospital 4 days prior to this admission, where numerous confluent erythematous to purpuric macules and patches on the face, trunk, and extremities, with variously sized erosions arising from the erythematous base were seen. The diagnosis of SJS was thus confirmed. The patient was then transferred to our hospital for further treatment.
On admission to our hospital, the patient was clear and oriented, with stable vital signs. Physical examinations showed that generalized confluent erythematous to purpuric macules and patches were distributed on the face, trunk, and limbs, with some flat erythematous spots present on the distal extremities. A sum of erythema of 35% total body surface area (TBSA) with blistering of 8% TBSA was calculated after the RegiSCAR review. Mucosal involvement with the presence of conjunctival and oral ulcers were also noted (Figure 1). Viral panel, mycoplasma pneumoniae infection and autoimmune survey yielded negative results (Supplementary Tables S1, S2).The skin lesions gradually improved to brownish and scaly characteristics, with near resolution noted at around day 14 under treatment with systemic corticosteroids (intravenous methylprednisolone), equivalent to 1.2-2.6 mg/kg of prednisone per day, combined with topical antibiotic ointments. Further laboratory examination revealed progressively elevating liver function enzymes and jaundice on day 6 of hospitalization (AST/ALT: 581/1488 U/L; ALP/GGT: 667/1990 U/L; T-Bil/D-Bil: 19.81/15.35 mg/dl) (Figure 2). Elevated IgG, C3, and C4 levels were also noted, without evidence of viral hepatitis. A liver biopsy was thus performed on day 6, and showed cholestatic hepatitis with marked perivenular cholestasis, ballooning of hepatocytes, and Councilman bodies with microgranulomas. Aggregation of histiocytes, neutrophils, and eosinophils were seen in the hepatic lobules. In a total of 10 portal tracts, only three bile ducts survived, which showed degeneration and cell senescence changes (i.e., increased nuclear to cytoplasm ratio, uneven nuclear spacing, drop-out, and syncytia formation; Figures 3A,B). VBDS was thus diagnosed. Due to progression of cholestasis and hepatitis on day 12 (AST/ALT: 973/2455 U/L; ALP/GGT: 815/3165 U/L; T-Bil/D-Bil: 34.91/22.81 mg/dl), systemic corticosteroids were up-titrated to the equivalent of 2.6 mg/kg of prednisone per day, with the capsule form of mycophenolate mofetil initiated at 250 mg twice a day and later up-titrated to 750 mg twice a day. Evaluation for possible liver transplantation was arranged after discussion with the patient and family.
The patient completed pre-transplantation evaluation uneventfully. A significant decrease in liver enzymes and bilirubin levels was observed on day 28 (AST/ALT: 184/647 U/L; GGT: 1,131 U/L; T-Bil/D-Bil: 25.58/15.18 mg/dl). The patient showed resolution of SJS with scars remaining on his extremities and denied discomfort. However, the patient showed a gradual decrease in the hemoglobin level to 8.7 g/dl on day 34, with subsequent discovery of cytomegalovirus (CMV) infection via colonoscopy biopsy and blood tests (a CMV viral load of 1,040,000 cp/ml was detected on day 42). Ganciclovir was thus initiated for treatment. Due to the presence of nucleated red blood cells in peripheral blood and the pathological finding of sinus histiocytosis with hemophagocytosis in specimens of the small intestine, a bone marrow study was conducted on day 58. The result showed hemophagocytosis in bone marrow, which met the HLH diagnostic criteria. Intravenous immunoglobulin was thus given from day 61 to day 64.
A repeat liver biopsy was performed on day 127, which revealed intrahepatic cholestasis with marked perivenular bile stasis. Eight portal tracts were observed with seven bile ducts, with six showing degeneration and cell senescence changes. An increase in the bile duct ratio was seen compared to that of the previous study (Figure 3C). Despite the resolution of SJS and VBDS, the patient still developed hypovolemic shock combined with septic shock episodes and died on day 236.
|
stevens-johnson syndrome, allergy, drug-induced liver injury, hemophagocytic lymphohistiocytosis, non-steroidal anti-inflammatory drugs, vanishing bile duct syndrome
| Not supported with pagination yet
| null |
PMC6525833_01
|
Male
| 63
|
A 63-year-old male with a history of hypertension, 20 pack-years of smoking, thyroid cancer in remission with partial thyroidectomy, and chronic obstructive lung disease (COPD) presented to the emergency department (ED) with acute abdominal pain. Abdominal ultrasound showed gallstones with no evidence of cholecystitis or biliary obstruction. The patient was diagnosed with biliary colic and discharged home with a plan to perform an elective cholecystectomy. Computed tomography (CT) scan of the abdomen was performed as an outpatient as part of the preoperative workup which showed evidence of gallstones, small bilateral pleural effusions, and moderate pericardial effusion with high density suggestive of hemorrhage (Figure 1). Consequently, the patient was referred to the emergency department for further workup. Upon arrival, the patient was asymptomatic, his vital signs were normal, and his physical examination was unremarkable. An electrocardiogram (EKG) revealed a nonspecific interventricular conduction delay. Chest x-ray showed a mildly enlarged cardiomediastinal silhouette with prominent perihilar opacities suggestive of a prominent vasculature (Figure 2). Complete blood count and basic metabolic profile were within normal limits. Further laboratory workup showed normal erythrocyte sedimentation rate, rheumatoid factor, complement level, and procalcitonin with negative serology for antinuclear antibodies and double-stranded DNA antibodies. A transthoracic echocardiogram (TTE) showed a large pericardial effusion without signs of tamponade, moderate aortic regurgitation, and normal left ventricular ejection fraction and size. Given the large volume of pericardial effusion, a pericardial window was attempted. A total of one liter of hemorrhagic fluid was drained. Pericardial tissue biopsy showed acute and chronic inflammatory cells with thickened pericardium, and no malignant cells were detected (Figure 3). Tuberculosis quantiferon assay, acid-fast bacilli staining, and fungal and bacterial cultures of the pericardial tissue were negative. The source of pericardial effusion remained elusive. Given the hemorrhagic nature of the pericardial effusion in the absence of recent use of anticoagulation and the negative workup for infectious or autoimmune etiology, a CT scan of the chest with contrast was obtained to evaluate for possible malignancy. CT showed an ascending aortic aneurysm measuring up to 7.5 cm with chronic dissection with no involvement of the coronaries or the great vessels (Figure 4). The patient underwent successful open surgical repair of his chronic Stanford type A aortic dissection with an uneventful postoperative course.
| null | Not supported with pagination yet
| null |
PMC4354942_01
|
Female
| 6
|
The patient was a 6-year-old, typically-developing bilingual Hispanic female who underwent total surgical resection of a suprasellar cystic and solid, third ventricular craniopharyngioma at a children's hospital when aged 5 years. Eleven months after surgery, she was referred to the authors for assessment and treatment of physical aggression (hitting, pinching, scratching, hair pulling, and throwing objects at others). Additional problem behaviors included property destruction, fecal smearing, verbal threats, and cursing. Parental interview indicated that these behavioral problems emerged approximately 4 months post-surgery; the severity and intensity of the behaviors continued to increase over time to the point where her parents felt that they could no longer manage her behavior in the home setting. Therefore, 7 months following surgery, she was readmitted to the children's hospital as an in-patient for assessment and treatment. There was no evidence that her display of problem behavior was cyclical in nature. Because of the severity of these behaviors, the patient was assigned constant one-to-one supervision in an isolated hospital room. Although previously toilet trained, she was required to wear a diaper, and often was restricted to her hospital bed (i.e. to deter her display of behavior problems on the unit). She had also developed hyperphagia. Approximately 2 months following surgery, her body mass index (BMI) was 19.3 (normal range); within 7 months, it had increased to 27.3 (overweight range). At the time of her assessment and treatment, her BMI was 32.3 (obese range). Medications included perphenazine (an antipsychotic) and valproic acid (an antiepileptic). In an effort to reduce the severity of her behavioral outbursts, diphenhydramine (an antihistamine) also was prescribed as needed.
Assessment and treatment sessions were conducted in a therapy room at the children's hospital. The room was approximately 8 feet by 15 feet in dimension and contained two small side tables, a couch, two cushioned chairs, a television on a stand, and a one-way mirror. A wheelchair, which was used to transport the patient to the session room, also was present. Sessions were 5 minutes in duration, and 6 to 12 sessions were conducted 2 to 3 times per week over the course of 3 weeks with the primary therapist (JH). All sessions were videotaped for data collection purposes by the second therapist (SH) who was also present in the room. Before the initiation of assessment and treatment, parental consent was obtained. The therapists met with both parents and a Spanish interpreter (who was employed by the hospital) during which time functional analysis procedures were thoroughly reviewed, general treatment strategies were discussed, and permission to videotape the sessions was obtained. Prior to submitting this report for publication, parental consent also was obtained.
In order to isolate the conditions under which the patient's aggression occurred, a functional analysis was conducted. As stated above, the purpose of this assessment was to systematically identify the cause-and-effect relations between the patient's aggression and environmental variables. The results were used to inform the development of an effective, function-based treatment using principles of reinforcement.
The functional analysis was conducted using procedures similar to those described by Iwata et al. Five conditions, each lasting 5 minutes, were presented 3 to 4 times in a multi-element design. The conditions were: 'demand (escape)' (the patient received a brief break from task demands when aggression occurred), 'ignore' (the therapist did not respond to any of the patient's behaviors, including aggression), 'attention' (contingent on aggression, the therapist delivered a reprimand and brief physical contact), 'tangible' (a small piece of food was provided to the patient following aggression), and 'play' (the patient received non-contingent attention and food while she played with toysa).
Three behaviors were targeted: aggression (hitting, pinching, scratching, hair pulling, and throwing objects at others), compliance (completion of a task following the first verbal or second model prompt), and mands (vocal requests for a food item while saying 'please'). The primary observer (see Acknowledgements) recorded the frequency of each dependent variable using ObsWin for Windows software (Antam, London, UK). Interobserver agreement was assessed by having a second observer (JH) simultaneously but independently collect data (via observing and scoring the videotapes) for at least 25% of the sessions within each condition. Agreement was calculated for each behavior by comparing the two observers' records on a second-by-second basis with a 2 seconds tolerance window. Percentage occurrence agreement was 95% for aggression, 83% for compliance, and 97% for mands.
Treatment was evaluated using a reversal (ABAB) design and consisted of differential reinforcement of alternative behavior (DRA) plus extinction (EXT). Treatment was matched to the maintaining variables identified in the functional analysis (i.e. escape and tangible reinforcement). In sessions designed to treat the escape function (DRA + escape EXT), aggressive behavior no longer resulted in a brief break; rather, the patient was instructed to complete a task on her own (e.g. to pick up items she had thrown). If she refused following a second model prompt, she then was physically assisted to complete the task. During initial treatment sessions, the assistance of the second therapist often was necessary to implement escape EXT (i.e. if the patient was sitting on the floor, she needed to be lifted onto her feet in order to complete the task). As treatment progressed, however, two therapists were not required to ensure task completion because the patient would immediately comply with the directive. If she completed the task on her own, she received a small piece of food (DRA), which was paired with social reinforcers, such as praise, high-fives, and brief applause. Although it may seem counter-therapeutic to deliver food contingent on appropriate behavior, we chose this strategy for two reasons. First, the patient was heavily food-motivated; thus, during initial treatment sessions, it was important to deliver a highly-potent reinforcer that could effectively compete with her problem behavior. Second, although we could have reinforced compliance with the functional reinforcer - escape - this would have amounted to periods of non-instructional time. Therefore, we chose to reinforce compliance differentially with a small edible item so that reinforcer access (i.e. a break) would not compete with on-task behavior. Previous research has shown that providing an edible item contingent on compliance may be more effective than providing escape for problem behavior maintained by negative reinforcement. As the patient showed sustained improvements in compliance and low rates of aggression during the demand (escape) condition, task requirements were systematically extended, requiring that she complete a greater number of tasks before earning the small food item (Fig. 2). Specifically, in the second treatment phase, the schedule of reinforcement was increased by one response requirement (e.g. from a fixed-ratio [FR] 1 schedule, to an FR 2, then an FR 3, etc.) contingent on aggression being at or below 0.9 responses per minute (rpm; i.e. less than 2 responses per 5min session). Task variety was also increased, such that instructions included appropriate academic tasks (e.g. spelling and counting).
In sessions designed to treat the tangible function (DRA + tangible EXT), aggressive behavior no longer produced access to food. Rather, if the patient manded appropriately, she received a small edible item (e.g. piece of rice cake). As before, tangible reinforcement was systematically thinned over time as she showed sustained improvements in mands and concomitant reductions in problem behavior (however, verbal praise continued to be delivered on an FR 1 schedule). Specifically, in the second treatment phase, the schedule was systematically thinned by adding 5 to 15 seconds to the amount of time (the fixed interval [FI]) that she had to wait contingent on aggression being at or below 0.5rpm (e.g. 1 response or less per 5min session). In an effort to attenuate excessively high mand rates, a signaling procedure was also implemented to facilitate discrimination of times when requests for food would and would not be granted. A green card was displayed during times in which mands would be reinforced, whereas a red card was displayed during EXT periods (see Fig. 2 for the reinforcement thinning/ signal schedule.) If aggression occurred, the interval was reset according to the FI schedule in effect for that session.
| null | Not supported with pagination yet
| null |
PMC9880383_01
|
Female
| 32
|
A 32-year-old Tunisian woman, presented to our department with a history of painful and dragging swelling of the back of her left hand evolving for 4 months. Originally, she responded well to non-steroidal anti-inflammatory drugs until 2 months before her consultation. She complained also of a long-lasting skin lesion gradually enlarging and suppurating on her fifth right toe. Her physical examination was notable for diffuse erythema and swelling in the third, fourth, and fifth fingers, extending into the palm, as well as her dorsal hand. She had fusiform digits and pain over flexor sheaths. Her 3 digits were held in slight flexion at rest. Laboratory studies showed a white blood count of 6520 cells/mm3, lymphocytes count of 1390 cells/mm3, and the C reactive protein level was 28 mg/L.
T1 weighted MRI images showed thickening of flexor sheaths (Figure 1A), intensely enhanced after injection of gadolinium (Figure 1B), conforming with flexor tenosynovitis of the left hand.
Skin biopsy of the lesion of her fifth toe revealed tuberculoid granuloma without caseous necrosis. Tuberculosis (TB) skin test was positive. After ruling out others causes of granulomatosis particularly sarcoidosis and malignant disorders, cutaneous and tenosynovial tuberculosis was retained. The patient was put on 15 months of isoniazid and rifampicin, supplemented in the first 2 months by both pyrazinamide and ethambutol. Our patient achieved remarkable response with healing of skin lesion without scarring and full regression of tenosynovitis on MRI (Figure 2A,B).
Tuberculous tenosynovitis of the hand is not only a scarce condition, but also a great mimicker thus explaining the usual delay in diagnosis. In developing countries, like ours, tuberculous should be considered among causes of chronic tenosynovitis.
The diagnosis of tuberculous tenosynovitis of the hand could be suspected by clinical and radiological findings and confirmed by bacterial and histological findings. An appropriate and fast diagnosis of tuberculous tenosynovitis is crucial to avoid inappropriate treatment.
A particularity of our case is the success of anti-TB drugs alone without surgical treatment.
|
mri, tenosynovitis, treatment, tuberculosis
| Not supported with pagination yet
| null |
PMC4609809_02
|
Male
| 56
|
OLP, a 56-year-old male fashion designer, had a very unhappy childhood, with few friends and many arguments at home. At university, he underwent a substantial change, becoming extroverted, making a lot of friends, and travelling extensively. As a professional, he was very creative, had many friends, and enjoyed going out. His father, an alcoholic who was extremely verbally abusive, committed suicide at 60 years of age. A nephew was diagnosed with depression. About 13 years ago, OLP began to isolate himself from his friends and to become very nervous whenever he was with a lot of people. He had difficulty concentrating, lost his enthusiasm for work and his creativity, and seemed to have lost his love for life. During that same period, he felt irritated and was bothered by noise, often getting into arguments with his neighbors for that reason. At work, he would also argue with colleagues, becoming angry easily and later regretting his outbursts. Since that time he has been undergoing psychotherapy and for the past eight years has undergone psychiatric treatment. He has used venlafaxine, mirtazapine, these two drugs together, and bupropion; however, there has been no remission of the depressive symptoms. After two months on bupropion (300 mg/day) and lamotrigine (100 mg/day) without any satisfactory improvement, aripiprazole (10 mg/day) was added. After five days of this combination, he developed major psychomotor agitation, accompanied by increased anxiety and restlessness, and akathisia that did not allow him to sit still for even three minutes at a time. He was sleepless at night even when taking 120 mg of flurazepam and complained of marked tiredness and muscle pain. In addition, his suicidal thoughts intensified and he experienced extreme anguish. He was given 50 mg of clozapine and all the other drugs were discontinued. After 10 days there was a significant improvement in his sleeping, allowing him to return to work. In addition, his psychomotor agitation decreased and there was a partial improvement in his feelings of anguish. Nevertheless, depressive symptoms remained: negative thoughts, extreme apathy, indisposition, and poor concentration. He was prescribed imipramine 150 mg and lithium 450 mg. He remained stable, with mild depressive symptoms after nine months. In the case of this patient, there is no clear evidence of hypomanic episodes; however, there is a report of unusual moods during his time at university. Since there are no relatives available to provide further details, the suspected diagnosis is dysthymia and double depression or type II bipolar disorder.
| null | Not supported with pagination yet
| null |
PMC9224403_01
|
Male
| 0
|
A boy aged 5 years and 2 months presented with recurrent high fever for 18 days, accompanied by a mild cough, abdominal pain, diarrhea and hematochezia. Although he was actively treated with intravenous antibiotics and immunoglobin, his fever was persistent. He was the first child in his family. A single episode of mild pneumonia had occurred when he was 4 months old and he received intravenous antibiotics for several days. There was no history of recurrent respiratory infection or eczema. No history of prolonged umbilical stump as well. He did not have chickenpox and was not vaccinated against this. On physical examination, he had moderate malnutrition, multiple ulcers in the oral cavity and on the surface of the tongue, and no lymphadenopathy or hepatosplenomegaly. Laboratory examinations revealed obvious elevation of inflammatory markers; the white blood cell count was 19.44 x 109/L, of which neutrophils accounted for 66.4%; the C-reactive protein (CRP) level was 17.34 mg/L; the erythrocyte sedimentation rate (ESR) was 73 mm/h; and the serum procalcitonin level was 0.412 ng/ml. In addition, the patient had moderate anemia, with a hemoglobin level of 84 g/L, and his serum albumin level was decreased, at 27.4 g/L. Serum antibodies against cytomegalovirus (CMV) and Epstein-Barr virus (EBV) were negative, and a T-SPOT TB test was negative. Number of CD19+ B lymphocytes was reduced, accounting for 16.45% (reference range (r.r): 18.5-28.0%) of lymphocytes; CD3+ T lymphocytes accounted for 54.1% (r.r: 56.0-68.0%) of lymphocytes, CD4+ T lymphocytes accounted for 31.45% (r.r: 29.0-40.0%), and CD8+ T lymphocytes accounted for 18.35% (r.r: 19.0-25.0%). The ratio of CD4+ to CD8+ T cells was normal, 1.71 (r.r: 1.1-2.0/1). Abdominal computed tomography scan showed irregular wall thickness, enhancement of the ascending colon and terminal ileum wall, and enlargement of the abdominal lymph nodes (Figure 1A). Intestinal ultrasound showed that the walls of the terminal ileum and colon were thickened. The inflammation affected the vermiform appendix. Colonoscopy was performed, and multiple ulcers in the terminal ileum and colon were detected. The area of the largest ulcer was approximately 3 cm x 4 cm. The ulcers had sharp margins, and most had no exudate or edema; these lesions were mainly located in the ascending colon and cecum. There were also scattered ulcers located in the rectum ( Figure 1B). Histological study of a mucosal biopsy indicated segmental chronic active inflammation of the colon and terminal ileum, cryptitis and crypt abscess were observed at the colon; additionally, several noncaseating granulomas was observed in the ileum and descending colon (Figure 1C). Microbial metagenomic next-generation sequencing (mNGS) of the intestinal biopsy did not reveal any opportunistic pathogens. The patient was diagnosed with VEOIBD and was treated with 2 mg/kg intravenous methylprednisolone twice a day. His fever subsided on the second day of the intravenous methylprednisolone regimen. Then, he was switched to oral prednisone, which was gradually tapered down within 3 months. Repeated colonoscopy showed that the intestinal inflammation was remarkably improved. Only small ulcers in the terminal ileum were observed, and the ulcers located in the colon had healed, leaving a scattering of small inflammatory polyps (Figure 1D). Capsule endoscopy confirmed terminal ileal ulcers. The patient took oral mercaptopurine for 4 months. Although he had a wild-type genotype for thiopurine methyltransferase (TPMT) and NUDT15, he experienced recurrent neutropenia. He was switched to methotrexate (MTX) due to neutropenia, and he had severe sepsis when he was on MTX. He presented with high fever, chill, delirium and shock, accompanied with remarkably elevated CRP and procalcitonin levels. He was treated with vasoactive drug and intravenous antibiotics in the intensive care unit for 1 week. Because of nonspecific intestinal colitis and early onset of disease, he was suspected to have monogenic IBD. The repeated immune work of the peripheral blood was showed in Supplementary Table S1. The number and percentage of memory B cells, central CD8+ T cells, Th1 cells and NK cells were obviously reduced.
|
colitis, intestinal ulcer, moesin gene, primary immunodeficiency disease, very early-onset inflammatory bowel disease
| Not supported with pagination yet
| null |
PMC7569537_01
|
Female
| 65
|
A 65-year-old lady presented with cough, night sweats and exertional breathlessness of two months' duration. There was no history of weight loss or fever. Her past history was significant for hypertension and non-productive cough two years before that was evaluated at another center. CT had shown right paratracheal and bilateral hilar lymphadenopathy along with upper lobe-predominant micronodules. Tuberculin skin testing was negative and endobronchial ultrasound-guided needle aspiration (EBUS-FNA) and transbronchial lung biopsy (TBLB) were performed. EBUS-FNA showed few non-caseating granulomas and TBLB was non-contributory. A presumptive diagnosis of tuberculosis was made and four-drug anti-tuberculosis treatment (ATT) was administered for six months with improvement in cough. Examination was unremarkable during current evaluation. There was no exposure to pets, drugs or occupational dusts. Chest radiography was normal. Spirometry was normal and diffusion capacity showed mild reduction. HRCT showed profuse random micronodules without lymphadenopathy and an upper-lobe and peripheral predominance that was visualized better with maximum intensity projection (MIP, Figure 1). Renal function tests, serum calcium and angiotensin-converting enzyme (ACE) levels were normal. Fibreoptic bronchoscopy (FOB) was done with bronchoalveolar lavage (BAL) and TBLB; BAL Fluid was negative for acid-fast bacilli and Xpert MTB/RIF and TBLB showed interstitial inflammation only. Surgical lung biopsy was performed and showed extensive perivascular non-caseating granulomas (Figure 2). BAL mycobacterial cultures were subsequently reported as negative. She was started on steroids with resolution in cough and breathlessness by second month of treatment. She remains asymptomatic and off steroids after one year of treatment.
|
micronodules, miliary, miliary tuberculosis, sarcoidosis
| null |
|
PMC7569537_02
|
Male
| 40
|
A 40-year old gentleman presented with polyuria, fatigue and nausea of 20 days duration. There was also history of unquantified weight loss and exertional dyspnea. He did not smoke and did not raise pets, abuse drugs or have exposure to occupational dusts. Physical examination showed raised papulonodular skin lesions over hands and legs. Renal function tests showed serum creatinine of 2.65 mg/dL (normal <0.8 mg/dL); Urine microscopy showed 2+ albumin with bland sediments. Hemoglobin was 10.9 g/dL with normal leukocyte and platelet counts. Ultrasound showed normal kidney size with preserved corticomedullary distinction. Chest radiographs showed miliary nodules. Serum ionized calcium was 1.8 mg/dL (normal 1.1-1.3 mg/dL) with suppressed parathyroid hormone (1.9 pg/mL, normal 15-65) and normal vitamin D levels (23.6 ng/mL). ACE levels were elevated (98 U/mL, normal 8-53). Tuberculin skin testing was negative. CT-Chest showed random micronodules without zonal preponderance and bilateral hilar lymphadenopathy (Figure 3, left). FOB was done with BAL and TBLB; BAL was negative for acid-fast bacilli and Xpert MTB/RIF. TBLB showed perivascular non-caseating granulomas with Schaumann and asteroid bodies (Figure 4). Punch biopsy of the skin also showed non-caseating granulomas in the dermis. He was started on steroids with resolution of hypercalcemia and normalization of renal function. His subsequent course was complicated by steroid-aggravated diabetes presenting with hyperosmolar coma; methotrexate was added and steroids were tapered. He remained asymptomatic with near-complete radiologic clearing (Figure 3, right) after one year of stopping methotrexate but relapsed by 18 months and remains on methotrexate.
|
micronodules, miliary, miliary tuberculosis, sarcoidosis
| null |
|
PMC7569537_03
|
Male
| 55
|
A 55-year old gentleman with moderately controlled diabetes mellitus presented with exertional breathlessness, cough, right-sided shoulder pain and weight loss of one month's duration. There was no anorexia or expectoration. He was a farmer by occupation and did not have exposure to poultry or occupational dusts and chemicals. Physical examination showed fine bilateral crepitations without clubbing. Renal function tests, serum calcium, urine microscopy, ACE levels and hemogram were normal. Chest radiographs showed miliary nodules (Figure 5, left). Human Immunodeficiency virus was negative by enzyme-linked sorbent assay. Tuberculin skin testing was strongly positive (18 mm, 1 TU). CT-Chest showed random micronodules without lymphadenopathy (Figure 6, left). FOB was done with BAL and TBLB; BAL was negative for acid-fast bacilli and Xpert MTB/RIF. TBLB showed well-formed perivascular granulomas with Langhans' giant cells, occasional fibrinoid necrosis and lymphocytes rimming (Figure 6, right). A diagnosis of miliary tuberculosis was made and four-drug weight-based ATT was started. However, his symptoms continued to worsen with worsening nodules and development of hypoxemia needing oxygen at four weeks (Figure 5, right). BAL mycobacterial cultures were negative in the meantime. Steroids were added with a presumptive diagnosis of immune reconstitution syndrome, with resolution of hypoxemia and symptoms. Symptoms recurred after stopping steroids at four weeks; right-sided vision loss due to uveitis occurred. After a clinico-pathological review, a possibility of concurrent miliary tuberculosis with sarcoidosis was considered. ATT was continued and low-dose tapering steroids and methotrexate (in view of poorly controlled diabetes) were added, with complete resolution of symptoms by eight weeks. He remains asymptomatic after two years of cessation of ATT and methotrexate.
|
micronodules, miliary, miliary tuberculosis, sarcoidosis
| null |
|
PMC7569537_04
|
Male
| 48
|
A 48-year-old gentleman presented with fatigue for two weeks. There was no history of night sweats, weight loss or fever. He did not smoke, did not raise pets, abuse alcohol or drugs and did not report exposure to occupational dusts. Physical examination was normal. Renal function tests, urine microscopy and hemogram were normal. Serum calcium was elevated (1.43 mg/dL, normal 1.1-1.3). Chest radiographs showed miliary nodules (Figure 7, left). ACE levels were elevated (76 U/mL, normal 8-53). Tuberculin skin testing was negative. CT-Chest showed upper lobe predominant random micronodules and lower-lobe interlobular septal thickening (Figure 8, left & right). FOB was done with BAL and TBLB; BAL was negative for mycobacterial cultures and Xpert MTB/RIF. TBLB showed prominent perivascular non-necrotizing epitheloid granulomas with asteroid bodies (Figure 7, right). A diagnosis of miliary sarcoidosis was made and steroids were initiated. His symptoms and hypercalcemia resolved by four weeks and steroids were tapered over the next nine months. He remains asymptomatic on follow-up.
|
micronodules, miliary, miliary tuberculosis, sarcoidosis
|
Composite image of the HRCT image of Patient 4 showing. upper lobes) random nodules with perilymphatic predominance, beading of the right oblique fissure, and ,perivascular nodules, and.
|
|
PMC4260285_01
|
Male
| 27
|
A 27-year-old male patient presented as multiple fistulas along with pain and swelling of the left hand since 2 years. He was a daily laborer and remembered a history of trauma to his hand around 2 years ago. He received different antibiotics but was left without cure. There was no history of any immunodeficiency or underlying disease. Physical examination was normal except a swelling in the left hand that extended from wrist to metacarpophalangeal joint of all the fingers, along with multiple discharging sinuses at the dorsal and palmar aspects of the hand [Figures 1 and 2]. The discharge was scanty and purulent with an offensive odor. The swelling was immobile in all directions and was fixed to the underlying structures. The movement at the metacarpophalangeal joint was restricted.
An initial clinical diagnosis of tuberculosis was made due to endemicity of the disease in Odisha, India. The routine biochemical and hematological tests including erythrocyte sedimentation rate were within the normal range. The X-ray of the upper extremity revealed no significant abnormalities. Swabs collected from the discharging sinuses were subjected to microbiological studies. Few pus cells and no bacteria were detected in the microscopy. Microbiological cultures for aerobic and anaerobic organisms showed growth of only Staphylococcus aureus which was sensitive to linezolid. Based on acid fast staining and culture, tuberculosis was ruled out. Hence, patient was advised with oral 600 mg of linezolid twice daily for 10 days, which yielded only a partial, but not complete relief. Finally, curettage and biopsy of the draining sinuses was made, which revealed many neutrophils with some colonies consisting of radiating filaments, surrounded by eosinophilic hyaline material creating a sun-ray pattern, similar to that of actinomycosis [Figure 3]. There was no evidence of lung disease, tooth problems or gingivitis in patient. Hence, a diagnosis of primary actinomycosis of hand was made for which he was treated with surgical debridement [Figure 4] and intravenous and oral penicillin therapy for 6 months to which patient showed excellent response.
|
actinomycosis, anaerobicbacteria, hand
| Not supported with pagination yet
| null |
PMC7607950_01
|
Male
| 12
|
A 12-year-old male patient was admitted to the clinic due to mild pain on anterior aspect of his right shoulder with mild limitation in active motion. Medical history of the patient was uneventful except falling to right side while playing football three days before admission. He recalled hitting the lateral aspect of his right shoulder to the ground and crepitation which occurred for once while self- examining shoulder motion in the direction of forward flexion. The patient had been admitted to the emergency department of a hospital on the same day of injury and informed to have soft tissue injury. Analgesic medication without any external support was given. A written informed consent was obtained from the legal guardian of the patient.
No unusual finding could be identified on inspection. Mild-to-moderate pain could be elicited with palpation on the coracoid process. Crepitus was not identified upon palpation as well as during active and passive shoulder motion. The range of forward flexion, extension, abduction, adduction, external rotation and internal rotation were 0-170, 0-20, 0-160, 0-40, 0-20 and 0-90 degrees on the right shoulder, respectively, while the range of forward flexion, extension, abduction, adduction, external and internal rotations were 0-180, 0-50, 0-170, 0-60, 0-40 and 0-90 degrees on the left shoulder, respectively. The patient mentioned mild aggravation of pain during active motion of the right shoulder. Active elbow flexion- extension was within the range of 0-150 degrees for both sides. Neurological evaluation was normal. Bilateral shoulder radiographs did not demonstrate any apparent finding of pathology. With respect to history of previous trauma, computed tomography (CT) images were obtained and revealed isolated coracoid mid-process fracture (Eyres type 2) with considerable anteroinferior displacement of the distal fragment (Figure 1). Conservative management including Velpeau sling use and analgesic medication were opted for the patient.
Physical and radiologic examinations were performed on each outpatient follow-up visit. The patient reported significant relief with Velpeau sling use two weeks after admission. At the end of fourth week, he was almost pain-free during rest as well as active and passive shoulder motion. The follow-up CT examination, obtained at the end of fourth week, confirmed significant callus formation (Figure 2). With respect to callus formation and vanished symptoms, Velpeau sling was discontinued and active shoulder motions were allowed with restrictions from weightlifting for four weeks. Physical examinations and plain radiographs could not identify any remarkable finding during follow-up. Until he fell to his right side from a couch at 11th month of follow-up, the patient was symptom-free in daily activities including weightlifting and sport activities. Upon this incident, he reported pain and limitation in the right shoulder motion. No additional finding could be detected on examination. Radiologic examination including CT, which proved complete osseous union with disappearance of coracoid fracture line, did not reveal any pathologic finding (Figure 3). The patient was completely symptom-free on the last visit which was 14 months after the diagnosis of fracture and the Disabilities of the Arm, Shoulder and Hand score was 0.
| null | Not supported with pagination yet
| null |
PMC7733463_01
|
Male
| 80
|
An 80-year-old man was referred to our pain clinic for radicular pain of the left lower limb. Just over 6 months previously, he had presented with severe radicular pain in his left leg. Magnetic resonance (MR) images revealed lumbar intervertebral disc protrusion (migrated to the left, L4-5, L5-S1) (Figure 1A and B), lumbar spinal canal stenosis, and degenerative lumbar scoliosis. At that time, he received posterior lumbar decompression interbody fusion plus spinal stabilization (L1-5), and his symptoms disappeared after surgery.
Four months later, the radiating pain in the lateral aspect of the left lower limb and the dorsum of the foot recurred, at the same sites as pre-surgery. The worst visual analogue scale (VAS) was 9/10. He could hardly stand up and walk. Weakness of dorsiflexion and plantarflexion was found in the foot. The lying straight leg raise test was positive, and the Achilles reflex was absent.
The previous medication included oral nonsteroidal anti-inflammatory drugs and physiotherapy, but neither treatment was effective. All the symptoms and physical examinations suggested problems of the L4-5, L5-S1 intervertebral discs, likely due to insufficient surgical decompression, recurrent disc herniation, or foraminal stenosis.
Ultrasound-guided experimental L4 and L5 PVB was then planned for this patient. The difficulty in this case was that the anatomical structure could not be located by body-surface land markers, due to the removal of spinous processes during the prior surgery. In addition, the anatomical structure could not be seen clearly through ultrasound, because of echogenic artifacts due to the surgical instruments, and the disordered fascia and muscle structure due to the surgical incision. Fortunately, on the X-ray film we found that the last-but-one pedicle screw and the terminal of the left rod were just next to the needle pathway we designed for the block, and might be used as markers for the puncture (Figure 1C and D).
The patient was placed lateral with the affected side upward. A convex type transducer (Wisonic Medical Technology, Shenzhen, China) was placed in a longitudinal orientation at the level of L5-S1, 3 cm lateral to the midline. With the probe sliding upward, the pedicle screws and the rods could be identified (Figure 2). Surface landmarks, such as iliac crest and locations for the pedicle screws and the rods were made on the skin. At the caudal side of the last-but-one pedicle screw and terminal of the rod, two lines were drawn perpendicular to the spine.
A paramedian transverse oblique scanning approach with needle in-plane was chosen for the L4 PVB. After proper sterile technique, the gel was applied to the ultrasound transducer. The transducer and cable were covered with a sterile sleeve, and placed along the vertical line with the caudal side of the target pedicle screw. The bone structure and fascia between muscles could not be seen clearly, because of the previous lumbar surgery. The pedicle screw was the most easily recognizable structure for hyperechoic. After local anesthetic (2 mL lidocaine 1%) infiltration to the skin and subcutaneous tissue, a 22G 90-mm needle (TuoRen Medical Instrument Finty, Zhengzhou, China) was inserted in a medial to lateral direction, overstride the pedicle screw, and reached the outboard of the foramen intervertebral (Figure 3). After confirmation of the needle tip with 2 mL normal saline, 5 mL of 0.1% lidocaine mixed with 3 mg betamethasone was injected.
Because of the obstruction of the iliac bone, a paramedian transverse oblique scanning with needle in-plane approach could not be used for L5 PVB. Instead, we chose a paramedian transverse scanning with needle out-plane approach. The transducer was placed along the vertical line with the terminal of the rod, beneath the space between the iliac bone and the nail rod. A hyperechoic area was observed, which was assumed to be L5 paravertebral space. After the puncture point was anesthetized with 2 mL of 1% lidocaine, the needle was inserted towards the hyperechoic area, out the plane of the ultrasound beam in a cephalad-to-caudal direction (Figure 4). Considering that the entire needle with an out-plane approach could not be confirmed by ultrasonography, the depth of the needle tip was referenced according to the previous measurement on the X-ray film. Another 5 mL solution was injected.
After 20 minutes of observation, the patient felt well enough to return home. At the third day after the block, the patient reported that his daily baseline level of pain had diminished to 50% of its original severity. We repeated this procedure every 14 days, three times in total. Three months after the last procedure, his visual analogue scale (VAS) was 2/10, and he could go for walks, and he was satisfied with the extent of pain control.
|
failed back surgery syndrome, lumbar paravertebral block, pre-designed route, ultrasound-guided
| Not supported with pagination yet
| null |
PMC7425252_01
|
Male
| 45
|
The case is of a 45-year-old African American male, nonsmoker, with a history of arterial hypertension, iron deficiency anemia, and auricular fibrillation. For the past 10 years, he had multiple episodes of community-acquired pneumonia, requiring admission to the intensive care unit on several occasions. Immunodeficiency virus (HIV), cystic fibrosis, tuberculosis, and several primary immunodeficiencies had been discarded and negative family history for similar diseases. He had chronic dry cough and dyspnea with modified Medical Research Council (mMRC) scale 3/4, requiring use of domiciliary oxygen. He consulted to our institution when his respiratory symptoms increased, presenting with productive cough with purulent secretion, functional class deterioration, and dyspnea with mMRC scale 4/4. At admission, physical examination reported the following vital signs: arterial tension 130/85 mm Hg, heart rate 100 beats per minute, respiratory rates 18 breaths per minute, SO2 93%, and temperature 38 C. He showed dyspnea at rest with use of accessory muscles; wet mucous membranes, without jugular ingurgitation or neck masses; regular and rhythmic tachycardic heart sounds, without murmurs; diminished respiratory sounds in both pulmonary fields; tubal murmur in bases; unremarkable abdomen; absence of edema in extremities; and presence of digital hippocratism.
Blood work with white blood cell count 7.4 x 103/microL, neutrophils 3.17 x 103/microL, lymphocytes 2.77 x 103/microL, hemoglobin 9 g/dL, hematocrit 34%, positive C-reactive protein 16.2 mg/dL, lactate 0.7, arterial blood gases with a pH 7.38, partial pressure of carbon dioxide (PaCO2) of 76.6 mm Hg, PaO2 46 mm Hg, oxygen saturation (SaO2) 80%, and HCO3 39 mg/dL.
Chest X-ray showed a central trachea and multiple thin-walled cystic images, occupying the lower two thirds of both hemithorax, no masses, or pneumothorax (Figure 1). Thoracic CT scan showed an increased tracheal diameter, measuring 28 x 33 mm (anteroposterior x transversal) 2 cm above the aortic arch, with atonic walls and presence of cylindrical and cystic bronchiectasis (Figure 2A). Enlarged mainstem bronchus with a right mainstem bronchus (RMB) and left mainstem bronchus (LMB) diameter of 22 and 17 mm, respectively (Figure 2B-F). On 3-dimensional coronal volumetric reconstruction and multiplanar reconstruction with digital subtraction, a gradual enlargement of the trachea and diffuse dilatation of the bronchi is observed, along with cystic bronchiectasis that give a cobblestone appearance (Figure 3).
A fibrobronchoscopy was then performed revealing tracheal dilatation, with tracheal diverticula and moderate purulent secretions. Posterior to the right main bronchus giving the branches for the superior and intermediate lobe, the anatomy is altered giving rise to multiple cavitations, some of them occupied by abundant pus. Similarly, on the left main bronchus, a loss of normal structure is seen, with multiple purulent filled cavitations (Figure 4). Bronchoalveolar lavage was performed, being positive for Achromobacter xylosoxidans spp (resistant to cefepime and ceftriaxone) and Pseudomonas aeruginosa. Taking into consideration the patient's clinical, imagenological, and endoscopical findings, MKS is diagnosed.
The patient was successfully treated for this infectious episode, for which treatment with piperacillin/tazobactam was administered for 14 days. Respiratory therapy was initiated, consisting of inhalation therapy and mucus drainage using the Acapella airway clearance device. After 10 days, the patient was discharged home.
Typical out-of-hospital management includes domiciliary therapy using Venturi 50% per day, salbutamol and ipratropium bromide inhaler as needed, respiratory therapy, and inhalation of a hypertonic solution to mobilize mucous secretions. The patient has been hospitalized in approximately 17 occasions from 2015 until 2019 for pulmonary infections that have been managed efficaciously without further complications. On his last appointment with the pulmonologist, his baseline disease was stable with no further deterioration in functional class. A control chest X-ray showed an improvement in pulmonary radiolucency with decrease in air fluid levels (Figure 5).
|
mounier-kuhn syndrome, bronchoscopy, case report, computed tomography, tracheobronchomegaly
| Not supported with pagination yet
| null |
PMC3004171_01
|
Male
| 49
|
A 49 years old Chinese male patient had a history of paroxysmal palpitation for 25 years, and recurred more frequently in the month before admission. Tachycardia usually burst on and off suddenly. The duration of the development was from a few minutes to 10 hours. A history of hypertension was for 4 years. Physical examinations show no abnormal physical signs. Ultrasound examination met with high blood pressure heart changes: mild expansion of the ascending aorta, normal left ventricular systolic function and diastolic dysfunction. Electrocardiogram (ECG) showed no abnormity under sinus rhythm; on paroxysmal tachycardia outburst [Figure 1], narrow QRS complex presenting together with heart rate (HR) 178/ min, interval from QRS wave to P wave (RP interval) was 120ms and RP interval < PR interval (interval from P wave to QRS wave). It's difficult to distinguish it from atrioventricular reentrant tachycardia.
Intracardiac electrophysiological examination: right atrial programmed basic stimulus coupled with cycle length decreasing extrastimulus (S1S2) at 500/280ms induced a narrow QRS complex tachycardia, but it was not accompanied by atrioventricular conduction jump phenomena [Figure 2]. Ventricular wave (V) and atrial wave (A) did not interfuse. VA interval = 120ms. It seems to be a side pathway, but right ventricular grading frequency increment stimulation showed ventricle to atrium decline conduction as well as Wenckebach phenomenon. Tachycardia could not be terminated by ventricular premature beat [Figure 3]. This suggested that there was no atrioventricular side pathway and atrioventricular nodal reentry was very likely. While we tried to improve atrioventricular node by ablating the slow pathway in lower region, atrioventricular junctional rhythm occurred, and the junctional beats decreased with the process of ablation. Subsequent repeated electrophysiological examination showed that under almost the same condition, right atrial programmed S1S2 at 550/270 ms induced atrioventricular conduction jump phenomenon, and the same paroxysmal tachycardia been induced [Figure 4]. We continued to improve atrioventricular node till to the jump phenomenon disappeared. After that not only repeatedly grading frequency increment stimulation but also programmed S1S2 could not induce tachycardia. Then, we repeated these stimulations after intravenous infusion of isoproterenol, still, no tachycardia and atrioventricular conduction jump phenomenon recurred. These electrophysiological examination suggested the success of ablation.
|
atrioventricular nodal reentrant tachycardia (avnrt), atrioventricular reentrant tachycardia (avrt), radiofrequency catheter ablation (rfca)
| Not supported with pagination yet
| null |
PMC4513297_01
|
Male
| 18
|
An 18-year-old male patient presented with pain in the nape of the neck since 12 months duration which was not improving with medication. The patient had stiffness of the neck with decreased range of movement in all directions. Routine hematological investigations were within the normal limit, and he was screened for rheumatologic problems without revealing any pathology. On magnetic resonance imaging of cervical spine [Figure 1], there were irregular lytic bony destruction in anterior arch and left lateral mass of C-1, left occipital condyle, left half of C-2 vertebra with prevertebral and left para vertebral collection, mild erosion in the odontoid process, with cranial migration of odontoid with tip up to the level of foramen magnum, there was compression of cord at this level with cord edema, suggestive of Koch's. We did a transoral decompression of abscess with biopsy along with posterior decompression of cord and occipitocervical fusion [Figure 2]. Biopsy of the pathological material shows the background of extensive necrosis and inflammatory granuloma containing lymphocytes, plasma cells, epithelioid cells, and multinucleated Langhan's type of giant cells suggestive of TB. Postoperatively, he was advised for antitubercular therapy for 18 months. Now, the patient has relief of pain and leading a normal life.
|
craniocervical junction, posterior fusion, transoral decompression, tuberculosis
| Not supported with pagination yet
| null |
PMC6166387_01
|
Male
| 29
|
A 29-year-old man, a former semiprofessional handball player, had a traumatic rupture of the proximal side of the patellar tendon of the left knee in 2016 during a match. No associated disease was reported. Primary surgery was performed in another health facility through a median approach using 2 anchors for tendon repair protected by an additional ipsilateral semitendinosus graft (patellar and tibial tunnels). The patient came to our health facility following severe functional deficits after an iterative rupture without having experienced any new trauma 13 months after the initial surgery. The iterative rupture of the knee extensor mechanism was also an iatrogenic fracture of the transverse patellar tunnel (Figure 1). Clinically, walking was not possible, there was a lack of active extension and hemarthrosis with pain. There were no scar problems, no signs of deep or superficial infection, and no cutaneous wound. A huge gap was clinically observed between the patella and the patellar tendon. Considering the patient's age, his preinjury sports level, and lack of active extension, the decision was made to perform a revision procedure. An artificial ligament (LARS ) and two adjustable loops, free ends of the PULLUP BTB (SBM SAS, France), were used to enhance the patellar tendon repair.
A preoperative lateral standard X-ray was taken of the contralateral knee at 30 of flexion to measure the Caton-Deschamps index and patellar height (Figure 2). The patient was placed in a supine position under general anesthesia with a tourniquet at the proximal part of the thigh. The previous median approach was used. The patellar fracture and the site of the previous rupture were cleaned to remove fibrous tissue and hematoma. Previous anchors were left in place.
The first step was to place the ligament advanced reinforcement system (LARS polyethylene terephthalate fibers 6 mm ref. L030307 ACFAR 32 CK). A new transverse tunnel was drilled in the tibia, distally to the tibial tunnel of the initial surgery, with a 5.5 mm drill, and the LARS was inserted in the tibial tunnel. The artificial ligament was then passed through the lateral retinaculum and above the patella at the junction with the quadriceps tendon in a Pulvertaft manner and through the medial retinaculum to return to its origin (Figure 3(a)). Two longitudinal tunnels were drilled in the patella using a 2.4 mm drill. The loops of a PULLUP BTB (the plate was removed from the device) were first passed into the patellar tendon and then into the patella through the two longitudinal tunnels using a shuttle relay. Next, the free ends were pulled down in the opposite patellar tunnels. At the proximal side of the reconstruction, the two free ends of the PULLUP BTB were inserted into each braid to close the system (Figures 3(b) and 3(c)). The distal and medial ends of the LARS were tightened with a clamp in order to restore normal patellar height and secured with 2 staples. Then, the 2 PULLUP BTB loops were tightened (Figure 3(d)). The previous tendon rupture was closed and reinforced with separate X-knots using absorbable sutures. The skin was closed. The knee was placed in an articulated brace with compressive ice therapy for 24 hours, and a postoperative X-ray was taken (Figure 4).
For postoperative care, weight bearing was not allowed for 6 weeks and mobilization of the knee was immediately started between 0 and 45 for 3 weeks then from 0 to 90 from 3 to 6 weeks. No complication was reported during the postoperative period. At 3 months, the patient was pain free and could walk without the aid of crutches. He was able to resume handball practice at 6 months after a control MRI. At one-year follow-up, he was able to play handball with complete knee extension strength (compared with the contralateral side) and was able to return to a semiprofessional level. The range of motion of the knee was 0-0-130 . The MRI at 1 year showed complete healing of the patellar tendon and the bone (Figure 5).
| null | Not supported with pagination yet
| null |
PMC9800931_01
|
Female
| 72
|
A 72-year-old woman with a middle school education who presented with headache, dizziness, nausea, and vomiting was admitted to a local hospital. In August 2021, she was diagnosed with tuberculous meningitis and underwent antituberculosis therapy. However, her symptoms did not disappear. After being discharged from the local hospital, she visited the neurology department of our hospital. CSF analysis and India ink staining of the CSF revealed Cryptococcus, and she was diagnosed with CM. She received antifungal therapy [fluconazole (10 mg/kg/day) and 5-fluorocytosine (100 mg/kg/day)] for almost 2 months before being affected by rapidly progressive dementia caused by central nervous system infection. On 25 November 2021, she began to show signs of confusion, an inability to speak, limited limb movements, an inability to walk, an inability to eat independently, signs of nausea without vomiting, and signs of incontinence, but she could still open her eyes when called and lacked limb convulsions. She was then hospitalized in our department. A lumbar puncture and CSF analysis were performed, which revealed an ICP of >200 mmH2O, a white blood cell (WBC) count of 7.4 cells/mul. a glucose level of 1.83 mmol/L, a protein level of 2,496 mg/L, and a chlorine level of 112.3 mmol/L. which suggested an inflammation. India ink staining of the CSF revealed Cryptococcus and CSF culture revealed Cryptococcus neoformans. Moreover, multiple intracranial lesions with meningeal enhancement were observed on brain magnetic resonance imaging (MRI; Figure 1). During hospitalization, the consciousness and mental state of the patient gradually improved after antifungal treatment with fluconazole and fluoropyrimidine, and cognitive function assessments revealed that the cognitive dysfunction of the patient persisted (Mini-Mental State Examination: 11 points, MoCA: 7 points); hence, the patient was referred to the Department of Rehabilitation Medicine for appropriate treatment while she demonstrated a weakness of the lower limbs and gait disturbance. After 2 weeks of receiving rTMS treatment, the condition of the patient was reevaluated, which revealed that all cognitive functions had significantly improved. The patient was conscious and responded slowly. Her ICP reached >200 mmH2O, and she had a WBC count of 11.83 cells/mul, a glucose level of 2.37 mmol/L, a protein level of 2,111 mg/L, and avchlorine level of 114.6 mmol/L. Additionally, the brain MRI images taken on 03 March 2022 revealed a significant reduction in the ventricles (MRI; Figure 2). Though the CSF culture was negative and cognitive function improved after rTMS therapy, we did not found a significant decline in inflammatory signals.
Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in the article.
Before rTMS treatment, the cognitive scale assessment results of the patient (Tables 1-3) indicated that the patient had severe cognitive impairment, and the MoCA assessment results revealed that the cognitive function of the patient was severely impaired; visuospatial and executive functions, object naming function, and abstract thinking function were severely reduced; and language function, short-term memory, and delayed recall function were impaired. The findings of the tests conducted using LOTCA and RBMT were identical in that the patients had a functional impairment and dysfunction in thinking operations, learning imitation, attention, and concentration. The scores on the aforementioned scales met the requirements for significant cognitive impairment.
The patient underwent a second evaluation of her cognitive performance after 2 weeks of rTMS treatment. The results showed that the cognitive function of the patient was greatly improved, and each subscore of the evaluation was very close to normal. The comprehensive analysis of assessment results and clinical observations revealed that the visuospatial function of the patient was significantly improved, and she could distinguish between her left and right limbs and identify the corresponding relationship between the front and the back and the left and the right of a picture. Meanwhile, the executive function, which includes drawing and imitating sketching, also improved. Time cognition and language function also significantly improved, and the patient could accurately express the current year, month, and season; tell the current time according to the clock; and name simple things. Furthermore, the memory function of the patient significantly improved; she was able to remember some basic phrases and complex routes and could execute simple recall tasks after a delay. However, the recall of complex tasks still required further improvement. In terms of learning and attention, the patient could learn how to use a calculator and imitate and copy graphics; in terms of logical thinking, the patient could classify simple objects and respond to straightforward logical questions.
Anti-infective therapy should be primarily addressed for the treatment of CM so that the cognitive impairments and other symptoms of the patients might be improved after controlling the infection. Additionally, rTMS therapy might improve the cognitive performance of the patient in different categories, including memory function, time perception, visuospatial cognition, speech, and executive function. This case provides a novel treatment method for patients with CM in conjunction with cognitive impairment, and our findings serve as a foundation for future clinical investigations. However, it is essential to increase the patient sample size to conduct a thorough analysis because of the limitations of case studies on individual differences. Finally, further exploration and research could be conducted on the selection of precise stimulation targets and stimulation parameters in rTMS, as well as analysis combined with imaging examinations, neurophysiology, and other clinical techniques.
|
cognitive impairment, cryptococcal meningitis, inflammatory cytokines, rehabilitation, repetitive transcranial magnetic stimulation
| Not supported with pagination yet
| null |
PMC7115113_01
|
Male
| 39
|
A 39-year old man was presented with painful sensation, redness and blurred vision of both eyes for 3 weeks. He also had multiple violaceous skin lesions on both arms and legs. His medical history was unremarkable. He was a non-smoker, but he was social alcohol drinker. He did not have a history of tuberculosis contact. He recalled no family history of cancer, tuberculosis, nor autoimmune diseases.
Physical examination revealed a temperature 38.1 C, blood pressure 126/85 mmHg, pulse rate 90/minute, respiratory rate 16/minute, and O2 saturation 98% on ambient air. Ophthalmic examination revealed bilateral swollen lids, chemosis, ciliary injection, compatible with bilateral diffuse scleritis. He did not have an oral ulcer, saddle-nose deformity, sinus tenderness, or hair loss. Multiple pulpable painless violaceous papules were found along his legs and arms. The neurological, heart, lungs, abdomen, lymph nodes and musculoskeletal examinations were unremarkable.
The laboratory examination showed a hemoglobin of 10.3 g/dL, white blood cell count 11.9 x 103/muL (77% neutrophils, 11% lymphocytes), a platelet count 366 x 103/muL, CRP-QT 204.48, ESR 91 mm/hr, serum creatinine (Cr) 0.58 mg/dL, globulin 4.5 g/dL, albumin 3.6 g/dL, ALP 227 U/L (30-120), AST 39 U/L (0-32), ALT 25 U/L (0-25). Urinalysis revealed 2+ protein, dysmorphic RBC of 10-20 cells/HPF. A 24-h urine total protein was 1333 mg/volume. Hepatitis B, hepatitis C, and anti-HIV antibodies were negative. Serum VDRL, TPHA, RT-PCR for herpes virus, and QuantiFERON-TB gold tests were also negative. The c-ANCA (indirect immunofluorescent assay, IFA) and anti-PR3 antibodies (western blotting) were positive. Anti-RF antibody was positive 26.4 IU/L (0-20.0). Anti-MPO, ANA, anti-Ro and anti-La antibodies were negative.
Initially, infectious scleritis was suspected. An orbits CT showed left eye proptosis with bilateral scleritis, enlargement of bilateral lacrimal glands with mildly retrobulbar fat stranding and sinusitis at bilateral sphenoid and ethmoid sinuses. Nasal endoscopy revealed suppurative discharge in sphenoid and ethmoid recesses and nasal crust without mass. A pus culture was negative.
Chest radiography showed multiple pulmonary nodules in both lungs. A chest CT showed multiple lung masses with peripheral enhancements and perilesional ground glass opacities, the largest mass was 10.1 x 5.6 x 6.0 cm. Sub-centimeter mediastinal lymphadenopathy was noted (Fig. 1a). An abdominal CT showed 10.5-cm heterogenous enhancements of the spleen without space-occupying lesion. Liver and portal veins were unremarkable. Neither adenopathy nor ascites were seen.
Bilateral conjunctival debridement and administration of topical and systemic ceftazidime and vancomycin were done. The scleritis, however, did not improve, and no organisms were found, conjunctival recess resection was performed. Conjunctival tissues revealed focal multinucleated giant cells with necrotizing vasculitis. AFB stains were negative. The IgG4 to IgG ratio was 0.29:1. Tissues from violaceous skin lesions were compatible with leukocytoclastic vasculitis.
The CT-guided lung mass biopsies for cultures, PCR for Xpert MTB/RIF assay, PCR for 18s rRNA were negative. The lung tissues showed geographic necrosis with few multinucleated giant cells, special staining for AFB, GMS, PAS, and cultures were negative.
The overall findings were compatible with GPA by ACR/EULAR 2017 provisional classification criteria. Prednisolone 1 mg/kg/day and intravenous cyclophosphamide 800 mg were started. The scleritis and lung mass were improved. Bronchoscopy was done 6 days later, hypervascularization and inflammation were seen along the bronchus (Fig. 2a). A transbronchial biopsy was done at the lateral segment of right middle lobe, and showed organizing pneumonia foci, a few necrotic tissues, no chronic granulomatous inflammation, no neoplasm, no vasculitis lesions, special staining for AFB, GMS, PAS, and cultures were negative. The final diagnosis was GPA with diffuse scleral, sinopulmonary, splenic, and mild renal involvements.
After the 3rd dose of cyclophosphamide, the bilateral scleritis deteriorated, the right lung mass was enlarged, and the patient also had a new onset of bilateral sensorineural hearing loss. ESR was raised from 29 to 58 mm/hr. Bronchoscopy revealed progression of bronchial inflammation, and a transbronchial biopsy showed necrotic tissue with neutrophil infiltration. The bronchoalveolar lavage for mycobacterial, fungal cultures, and PCR for herpes were negative. Intravenous rituximab 375 mg/m2 was given due to clinical deterioration. During treatment, he developed severe secondary infection, clinical deterioration, and then expired.
| null | null |
|
PMC7115113_02
|
Female
| 58
|
A 58-year-old woman presented with progressive painless visual loss in both eyes for 1 month. She had low grade fever, non-productive cough, and weight loss for 6 months.
Her medical history was notable for right Bell's palsy and bilateral hearing loss 1 year ago and was improved after a short course of oral corticosteroid. She denied any history of tuberculosis contact. She acknowledged no family history of cancer, tuberculosis, nor autoimmune diseases.
Physical examination revealed body temperature 37.7 C, blood pressure 133/80 mmHg, pulse rate 96/minute, and respiratory rate 18/minute. Ophthalmic examination revealed peripheral ulcerative keratitis, necrotizing scleritis, impending corneal perforation on both eyes, right visual acuity (VA) 3/60, left VA 6/24, normal intraocular pressure (IOP). The examination of ear-nose-throat (ENT), cardiovascular system, lung, abdomen, extremities, lymph nodes, skin and neurological system were unremarkable.
The laboratory examination showed hemoglobin 12.2 g/dL, white blood cell count 12,930 cell/mm3 (74.6% neutrophils, 18% lymphocytes, 5.7% monocytes, 0.9% eosinophils), platelet count 927,000/mm3, Cr 1.0 mg/dL, BUN 15.2 mg/dL, globulin 3.7 g/dL, albumin 3.8 g/dL, and no hepatitis. Urinalysis revealed trace protein, WBC 0-1 cell/HPF, and RBC 0-1 cell/HPF. Serum VDRL, TPHA, hepatitis B, hepatitis C, and anti-HIV antibodies were negative. Anti-RF antibody was 124.60 IU/ml (0-20 IU/ml) but Anti-CCP antibody was <7 U/mL. ANA was positive for nucleolar pattern with titer 1:320. The c-ANCA (IFA) and anti-PR3 antibody (western blotting) were positive.
The chest radiograph revealed multiple mass-like lesions. A chest CT showed two heterogenous enhancing masses, one 3.6 cm in size located at the lateral segment of the right middle lobe and the other was 4.4 cm in size located at the superior segment of the right lower lobe. A CT-guided lung mass biopsy was done at right lower lobe lesion. The pathological findings were one focus of necrotizing vasculitis. The AFB stain, gram stain, GMS stain, PAS stain, and cultures were all negative.
One-gram methylprednisolone was given for 3 days as initial therapy, followed by monthly intravenous 800-mg cyclophosphamide and prednisolone for 6 months. During treatment, the ophthalmic and pulmonary lesions were improved. After 6 months of therapy, she developed new mass-like lesions at right lower lungs without ophthalmic lesion. Bronchoscopy was done and revealed an ulcerative lesion along the carina through the right main bronchus. All microbiological testing was negative.
A right lower lobe wedge resection was performed due to isolated pulmonary lesions, and showed epithelioid histiocyte aggregation with central necrosis, eosinophils and multinucleated giant cells, lymphoplasmacytic infiltration in the vascular wall, compatible with granulomatous inflammation with destructive leukocytic angiitis. Special strains for AFB, GMS and PAS revealed no microorganisms (Fig. 1b). The final diagnosis was GPA with diffuse necrotizing scleritis, central airway, and lungs involvements by ACR/EULAR 2017 provisional classification criteria. Azathioprine and oral prednisolone were given for maintenance of remission. She was in remission state.
| null |
Chest CT showed the largest pulmonary mass at lateral segment of right middle lobe.
|
|
PMC6785917_01
|
Female
| 13
|
A 13-year-old female athlete presented to the office with left foot pain of 2 weeks duration. The pain had started gradually as she transitioned directly into basketball following her soccer season, moving from soccer cleats to basketball shoes. She participated at a high level for both sports for her age. She had no history of similar pain, but had sprained her ankle 1 month ago. She had no recent dietary changes, and there were not any concerns about her diet from her parents. The pain was located in the arch and described as tingling, worse at night, and after practice, but improved in the morning. She tried putting inserts in her shoes without relief. On exam, she was well nourished and in no acute distress. She had no visible swelling, and the alignment of her ankle, knees, and hips appeared normal. She had good arches of her feet that were symmetric bilaterally, and her gait revealed no abnormalities. Range of motion of the foot and ankle were normal. She had tenderness over the proximal second and third metatarsals on both the dorsal and plantar surfaces. She also had tenderness over the dorsal and plantar surfaces of the cuneiforms, including the arch of the foot. X-rays were obtained in the office and were negative. Due to concern for a stress injury, a magnetic resonance imaging (MRI) was obtained. The MRI showed a stress fracture involving the distal and plantar aspect of the middle cuneiform, with a stress phenomenon noted at the base of the second and third metatarsals. Figures 1 and 2 show this finding.
She was placed in a boot for all weight-bearing activities. At 4 weeks, she was only having occasional pain. The decision was made to continue the boot for 2 more weeks. At follow-up, 2 weeks later (6 weeks total), the patient was still having pain. She admitted to participating in impact activity outside the boot at the gym since her last appointment. After discussion with the patient and her father, she was made partial weight-bearing in the boot with crutches with instructions to wean off the crutches once pain free. At a recheck 2 weeks later (8 weeks total), she was pain free walking in the boot without the crutches. She was weaned out of the boot over the next 2 weeks (10 weeks total) and then began a gradual return to impact activity without set back.
|
athlete, cuneiform, foot, stress fracture
| Not supported with pagination yet
| null |
PMC7468610_01
|
Female
| 40
|
In October 2016, a 40-year-old woman was admitted to another hospital for a diagnosis of BC. One month prior to admission, a routinary mammography had shown the presence of a mass of 15 mm in diameter confirmed by a subsequent MRI scan. She was treated with right quadrantectomy and axillary dissection, and the histopathology provided a diagnosis of grade III invasive poorly differentiated ductal carcinoma. Immunohistochemistry was positive for estrogen and progesterone receptors (75%), with a high Ki67 proliferation index (30%) and mild expression of HER-2 (+1). However, fluorescent in situ hybridization for the HER2 gene was negative (staging pT1N2M0). In November, she started adjuvant chemotherapy regimen with doxorubicin 60 mg/m2/day and cyclophosphamide 600 mg/m2/day for 4 cycles, followed by maintenance with paclitaxel 80 mg/m2 since April 2017 and endocrine therapy with tamoxifen. Peripheral blood cell count at the start of chemotherapy showed leukopenia (white blood cells count 1.0 x 109/L), not further investigated, with mild anemia (hemoglobin 11.6 g/dl) and normal platelets count (168 x 109/L). In June 2017, during breast adjuvant radiotherapy, the patient was persistently pancytopenic (hemoglobin 9.6 g/dl, white blood cells 0.9 x 109/L, and platelets 101 x 109/L). Bone marrow was cellular (+3) and showed 82% of CD34/CD117/CD13+ and CD33/CD7/CD11b/CD14/cMPO blasts of small-medium size, round nucleus, and basophilic cytoplasm with rare granules, consistent with a diagnosis of AML. Cytogenetic analysis showed a normal karyotype (46, XX), and the gene mutation status was wild type for FLT3/ITD and TKD, AML1/ETO, Cbf/MYH11, NPM1, PML-RARalpha, BCR-ABL1, and MLL. The patient was treated with ICE induction chemotherapy, consisting of a combination of idarubicin 10 mg/m2 days 1, 3, and 5, cytarabine 100 mg/m2 days 1-7, and etoposide 100 mg/m2 days 1-5, but proved refractory. In September 2017, the patient was referred to our centre and underwent a salvage chemotherapy with the G-CLAC schedule: clofarabine 30 mg/m2 days 1-5, cytarabine 2000 mg/m2 days 1-5, and granulocyte colony stimulating factor (G-CSF) until hemopoietic recovery. On day +13 from chemotherapy, the patient was pancytopenic (hemoglobin 8.5 g/dl, white blood cells 0.750 x 109/L, neutrophils 0.2 x 109/L, and platelets 20 x 109/L) and presented a septic shock, associated with acute abdominal pain with positive Murphy sign and positive blood cultures for Escherichia coli, Staphylococcus haemolyticus, and Candida glabrata. She received antibiotic and antifungal treatment (meropenem, tigecycline, and caspofungin) and supportive care with fluids and inotropic agents. An abdominal ultrasound and an MRI allowed to make a diagnosis of acute cholecistytis and in October 2017 (on day +30 from chemotherapy), after a full hematologic recovery, she underwent a laparoscopic cholecystectomy. The microbiologic examination of pericholecistic liquid revealed a multidrug-resistant Pseudomonas aeruginosa. In the same period, she became a KPC-Klebsiella pneumoniae rectal carrier. On day +35, the bone marrow aspiration showed a complete remission (CR). For this reason, in November 2017, on day +56 from chemotherapy, she underwent an allogeneic hematopoietic stem cell transplantation (HSCT) from the haploidentical brother after a preparative regimen with TBF (thiotepa 5 mg/kg days -7, -6; fludarabine 50 mg/m2 days -5, -4, -3; busulfan 3.2 mg/kg days -5, -4, -3). No major toxicity was observed during the aplastic phase, and the engraftment was obtained on day +17 for PMN and on day +25 for platelets. On day +20, the bone marrow aspirate confirmed a CR with complete donor chimerism, and on day +30, the patient was discharged.
Two months after discharge, she was readmitted to the hospital due to an acute left chest pain, fever, and neutropenia (hemoglobin 8.9 g/dl, white blood cells 0.62 x 109/L, neutrophils 0.34 x 109/L, and platelets 21 x 109/L); chest computed tomographic scan showed a basal bilateral pneumonia associated with diffuse pulmonary nodules and bronchial obliteration. A microbiological examination of bronchoalveolar lavage proved the presence of a Nocardia spp. Despite targeted antibiotic therapy with high-dose sulfametoxazole-trimethoprim in association with broad-spectrum and specific antibiotics in different timing (amikacin, ceftadizime-avibactam, levoxacin, ceftolozane-tazobactam, tigecycline, imipenem, and linezolid), pulmonary nocardial lesions persisted (and progressed toward) with the formation of a pulmonary organized abscess (Figure 1). Due to the persistence of fever and worsening of clinical conditions, the patient was submitted to a lower pulmonary lobectomy by left lateral thoracotomy; histological examination confirmed the presence of a Nocardia spp. She had a good postoperative course with disappearance of fever and complete resolution of the pneumonia.
Currently, the patient is alive in continuous CR for AML and BC, with a follow-up from diagnosis of 29 months and 37 months, respectively. She is in good general conditions with an excellent quality of life.
| null | Not supported with pagination yet
| null |
PMC7276589_01
|
Male
| 14
|
A 14-year-old male presented to the emergency department 15 min after a hyperextend and axial load force injury. The boy was trying to push a heavy object during a basketball game, resulting in an open volar fracture epiphysiolysis of the distal radius and fracture on the distal one-third diaphysis of the ulna. The patient's right hand was dressed with gauze and a bandage that had been applied by the physician of the sporting match. After removing the dressing, the distal radius metaphysis was found to be protruding through a transverse volar laceration in the wrist area, 5 cm in length. Immediately, the wound was irrigated with 4 L of isotonic sodium chloride solution and the fracture was reduced under local anesthesia. Care was taken not to harm the median nerve, the ulnar nerve, and the ulnar artery which were very close to the fracture. The wound was dressed after debridement and a short arm cast was applied. The patient's neurovascular status was found to be intact. Intravenous cefazolin and an antitetanus serum were administered. An X-ray of the wrist was performed and the boy was taken to the operating room.
|
open fracture, case report, distal radius, epiphysiolysis, pediatric
| Not supported with pagination yet
| null |
PMC5109572_01
|
Male
| 39
|
In 2015, a 39-year-old man presented to our hospital with a four-week history of headache and a two-week history of low-grade fever. He was previously healthy and had not been taking any medications. He was a current smoker (15 pack-years) and occasionally consumed alcohol. Although he had traveled to Hawaii in 2005, Dalian in China in 2008, and Hong Kong in 2010, he had no other history of overseas travel. He had been working as electrical engineering technician, and had not been exposed to any imported timber or animals.
A physical examination revealed mild neck stiffness and a low-grade fever of 37.2 C. A chest X-ray showed a tumor of approximately 50 mm in size in the right lower field, and a chest computed tomography (CT) scan showed a 48x35 mm tumor with spiculation and notching on the right S9/10 (Fig. 1a and b). A gadolinium-enhanced brain magnetic resonance imaging (MRI) scan showed multiple brain tumors with ring enhancement (Fig. 1c and d). The patient's white blood cell (WBC) and platelet counts were high [WBC, 12,900/mm2 (normal range 4,000-9,000/mm2); platelet, 444,000/mm2 (normal range 150,000-370,000/mm2)], and the blood chemistry findings were normal, including the levels of glucose, hemoglobin A1c, tumor markers, immunoglobulin, and autoimmune antibodies.
Based on the chest CT and brain MRI findings, we suspected lung cancer with multiple brain metastases, and performed a bronchoscopic examination. A histopathological examination of the transbronchial lung biopsy (TBLB) specimen from the right S9/10 revealed numerous fungal elements that appeared as encapsulated yeast with clear halos (Fig. 2a). The yeast was observed after periodic acid-Schiff and Grocott methenamine silver staining (Fig. 2b). A lumbar puncture had an elevated opening pressure (33 cm H2O), and the cerebrospinal fluid sample contained 264/mm2 cells, 84.1% of which were lymphocytes. India ink staining of the patients' cerebrospinal fluid revealed encapsulated Cryptococci as a halo against a black background (Fig. 2c). Additional serological tests revealed that the patient was negative for HIV-1/2 antibodies and positive for cryptococcal antigen; the CD4+ lymphocyte level in the patient's peripheral blood was normal. The cryptococcal strain isolated from the bronchial lavage and cerebrospinal fluid was identified as C. gattii genotype VGIIa using multi-locus sequence typing (MLST), based on a DNA sequence analysis of a set of polymorphic loci.
As a result, the patient was diagnosed with pulmonary and cerebral cryptococcosis induced by C. gattii genotype VGIIa infection. He was treated with liposomal amphotericin B (5 mg/kg/day for more than 8 weeks), flucytosine (6,000 mg/body for more than 8 weeks). Percutaneous lumbar drainage was performed more than 15 times within the first month to manage his increased intracranial pressure. His subjective symptoms and radiological abnormalities improved gradually. After induction therapy, we are planning consolidation therapy and maintenance therapy with oral fluconazole.
| null | Not supported with pagination yet
| null |
PMC7154234_01
|
Male
| 39
|
We report on a 39-year-old Filipino man who presented to our facility with a 2-week history of headache, easy fatigability, and on-and-off gum bleeds that stopped spontaneously associated with palpitation tremors and unintentional weight loss. He had a history of hypertension diagnosed 2 years ago. Upon presentation, the patient was conscious, alert, oriented, afebrile, and vitally stable. He seemed slightly pale but without signs of thyroid eye disease. His neck exam showed enlarged thyroid glands but no palpable lymphadenopathy. His hands were sweaty moist and warm with coarse upper-extremity tremors. His chest abdomen and neurological exams were all normal. His blood works and peripheral smear were suggestive of pancytopenia with severe macrocytic anemia and reticulocytopenia.
The patient's initial blood test results showed a hemoglobin level of 5.5 g/dL (lab reference range: 13-17), his white blood cell level was 2.2 x 103 U/L (reference range: 4-10 x 103), and his platelet count was 30 x 103 U/L (reference range: 150-400).
Other lab works, including iron profile, folate, vitamin B12, haptoglobin, lactate dehydrogenase, and liver function test, were normal. Hepatitis B and C virus, HIV screen, parvovirus B19 serology, and autoimmune disease work-up were done as part of the pancytopenia work-up, all returned normal. CT of the chest/abdomen and pelvis with contrast showed no organomegaly evidence of malignancy. Peripheral blood smear showed severe macrocytic anemia with anisopoikilocytosis including scattered macrocytes, ovalocytes, tear drop target cells, some spherocytes and schistocytes, basophilic stippling, and few polychromatic cells. We found leukopenia with moderate neutropenia, mild toxic features, few and reactive lymphocytes, and severe thrombocytopenia. Bone marrow exam suggested hypocellular bone marrow with low numbers of megakaryocytes and decreased granulopoiesis with orderly maturation up to the segmented stage. Blast cells were 3%. Erythropoiesis appeared active with a mixture of normoblastic and megaloblastic maturation. No significant dysplasia was found, normal cytogenetics and no increased blast cells.
The patient was started on supportive transfusions with packed red blood cells and platelets. He required almost weekly transfusions. Then, the patient was started on cyclosporin 125 mg BID daily. During his hospital stay, the patient was found to have hyperthyroidism with suppressed TSH of <0.01 mIU/L (reference range: 0.45 4.50) T4 45.4 pmol/L (9-20), T3 15.35 pmol/L (2.6-5.7), and anti-TBO (thyroid peroxidase negative and anti-thyroglobulin antibody negative). A scan of his thyroid glands showed diffuse uptake suggestive of Gravies' disease. The patient was started on propranolol and methimazole, but he was not compliant with his medications so radioactive iodine ablation was done. After which the patient became hypothyroid and was started on levothyroxine replacement 100 mug daily.
After radioactive iodine ablation and thyroid disease control, the patient's red blood cell and platelet transfusion requirement markedly decreased, and his blood counts improved. Cyclosporine was gradually tapered until it was completely stopped after 16 months. Currently, our patient does not require any red blood cell or platelet transfusions. His anemia symptoms have resolved. His follow-up includes close blood count monitoring. During his most recent follow-up, he had a hemoglobin level of 15 g/dL, white blood cell count of 5.4, and platelet count of 38. Repeat bone marrow exam after 2 years of diagnosis suggested cellular bone marrow with trilineage hematopoiesis and areas of reduced cellularity.
|
anemia, hyperthyroidism, thrombocytopenia
| Not supported with pagination yet
| null |
PMC6374815_01
|
Female
| 70
|
A 70-year-old woman presented with a 10-month history of left midfoot pain without any trauma. She was diagnosed as having osteonecrosis of the tarsal navicular based on the findings of plain radiographs from the previous hospital. She was initially treated with an insole. However, the conservative treatment was ineffective for her symptoms. Therefore, surgery was performed.
At the time of presentation, her left foot was swollen and had point tenderness at the dorsal side of the talonavicular joint. The preoperative Japanese Society for Surgery of the Foot (JSSF) midfoot scale score was 79 points. Radiographs showed increased radiodensity and dorsal protrusion of the tarsal navicular. Sclerotic collapse was also noted at the lateral aspect of the tarsal navicular (Figure 1). Computed tomography (CT) scans showed diffuse sclerosis and marginal irregularities of the tarsal navicular (Figure 2). Magnetic resonance imaging (MRI) showed low signal-intensity areas on both T1-weighted images and T2-weighted images in the marrow of the tarsal navicular. Gd-based MRI showed increased uptake in the peripheral tarsal navicular, which was representative of hypervascular areas (Figure 3). She was diagnosed with spontaneous osteonecrosis with a Maceira classification of Stage 3.
Arthrodeses of the talonavicular and naviculocuneiform joints were selected as the treatment because both joints had cartilage damage on imaging. The articular surfaces of the talus and medial cuneiform that were adjacent to the tarsal navicular and necrotic areas of the tarsal navicular were excised. The blood supply was visible from the marrow of the residual tarsal navicular. The bone defect (5 cm x 1 cm) was reconstructed with a tricortical bone graft harvested from the iliac crest. Arthrodesis was performed using an LCP Distal Radius Plate (SYNTHES) with 6 2.4 mm locking screws from the medial aspect of the foot (Figure 4). Histopathologic findings showed diffuse empty lacunae without any infection, consistent with avascular necrosis (Figure 5). The ankle was immobilized in a cast for 4 weeks after surgery. Partial weight bearing with a patellar tendon-bearing (PTB) orthosis was permitted after the removal of her cast.
A radiograph taken 1 year after surgery showed union of the naviculocuneiform joint, but she did not have satisfactory union of the talonavicular joint. The patient had referred pain with the backing out of the most proximal screw. Therefore, the most proximal screw was removed. A radiograph taken 5 years after surgery showed nonunion with mild osteoarthrosis of the talonavicular joint (Figure 6). However, she just had tenderness of the talonavicular joint and had no pain in her usual daily life. The final follow-up JSSF midfoot scale score was 97 points.
| null | Not supported with pagination yet
| null |
PMC10203767_01
|
Male
| 22
|
A 22-year-old male patient had been occasionally experiencing left chest pain and dyspnea for several years, but the symptoms had resolved spontaneously, and he had no history of medical examination. He presented to his home physician with persistent complaints of respiratory distress and high-grade fever for one month after the onset of left chest pain and dyspnea, and was found to have a pneumohydrothorax on chest radiography (Fig. 1A). For the failure of conservative treatment, he underwent thoracoscopic decortication at a local hospital (Fig. 1B). The apical part of the left upper lung, which could not be peeled off, was resected. A pathological finding of Aspergillus organisms in the pleura (Fig. 1C and D), and a serological positiveness of Aspergillus antibodies led to the diagnosis of spontaneous pneumothorax and Aspergillus empyema. The patient had no history of pulmonary aspergillosis, and no organisms were present in the lung parenchyma in the lung resection specimen. Laboratory and physical findings were negative for the presence of any disease that could cause opportunistic infections, and there was no history of any other noteworthy illness since birth and no family history of immunodeficiency diseases. It was strongly inferred the patient had developed a chronic pneumothorax cavity as a result of repeated pneumothoraces, although there was no history of the other diseases that could form cavitary lesions, such as tuberculosis. Just before the onset of symptoms, his apartment had been closed for an extended period for the summer vacation, and upon his return, he discovered that his place was heavily infested with mold due to the high summer temperatures and humidity. It was presumed that the patient developed the current pneumothorax in this moldy environment, resulting in the dissemination and infection of Aspergillus in the previously existing chronic pneumothorax cavity through the bronchopleural space. The postoperative course after the decortication and the wedge resection was good, and he was discharged home on the eighth postoperative day. However, one month after the operation, re-infection occurred and persistent air leak developed, which prompted admission to our hospital. Chest computed tomography (CT) revealed a large apical pleural space in the left without a fungus ball, and an irregularly thickened pleura in the upper thoracic cavity, suggesting the infection of the pleura and air space (Fig. 2). Pleural fluid culture detected Aspergillus fumigatus, and serum Aspergillus antibodies were again positive. Other findings on CT included a very mildly infiltrated left upper lung parenchyma with intrapulmonary infection at the bottom of the cavity. The patient had no evidence of intrapulmonary infection on imaging or histology at the time of the initial surgery, suggesting that the fungus in the pleural and pneumoperitoneum had spread to the lungs after the previous surgery; hence, the patient was diagnosed with apical pleural aspergillosis because the main body of infection was located in the pleura and airspace.
It was determined that surgical intervention for the infection site in the pleura was necessary for curative treatment. Since the intrapulmonary infection was controlled with antifungal medication, lobectomy with extensive muscle filling was considered an excessive intervention. Because the dead space was confined to the apex of the lung, we decided to perform cavernostomy and minimal dead space filling as the first-line procedure.
A 12-cm anterolateral incision was made in the third intercostal space, and cavernostomy was performed on the Aspergillus cavity, providing a clear view from apex to bottom (Fig. 3). The thickened parietal pleura, forming the cavity wall, was deemed unresectable, while the thickened lung parenchyma at the cavity bottom maintained compliance. To control infection, the pyothorax cavity was scraped with a sharp curette to remove Aspergillus fragments, and the alveolar fistula was closed by suturing the pleura as a pledget. The cavity volume was estimated at 90 mL. The extraperiosteal air-plombage method (Kinchu method) was used to minimize cavity volume. Intercostal muscle and partial serratus anterior muscle transposition flaps, attached to the second and fourth ribs, were guided into the cavity. Two thoracic drains were placed in the cavity, one outside the pleural tent, and the wound was closed.
Postoperatively, the thoracic drain was maintained for three weeks while the patient underwent intravenous voriconazole (VRCZ) therapy (4 mg/kg every 12 hours for 4 weeks), which was eventually transitioned to oral VRCZ (400 mg/day) for approximately a year. One year after the surgery, microbiological results remained negative for the organism. The patient had good lung reexpansion, with full extension into the bony thorax (Fig. 4). With regard to scapular dysfunction, the patient developed slight limitation in movement when raising the left upper limb straight toward the head and putting it on the ear, but there has been no other movement disorder of the upper limb or scapula, and no difficulty in daily life. There is no thoracic deformity or disfigurement.
|
aspergillus empyema, cavernostomy, decortication, minimally invasive surgery, pleural aspergillosis, spontaneous pneumothorax
|
One month after the decortication, re-infection with Aspergillus occurred and persistent air leak developed. Chest computed tomography revealed parietal pleural thickening in the apical thoracic cavity.
|
|
PMC4699733_01
|
Female
| 66
|
A 66 year-old woman with a history of atrial fibrillation, hypertension, gastritis, and hypothyroidism presented to our clinic in July 2014 with progressive dysphagia for solids and liquids over 18 months. Her symptoms occurred daily and resulted in an 11-kg weight loss over this period of time. She denied heartburn, regurgitation, choking or coughing during eating, chest pain, vomiting, abdominal pain, or change in bowel habits. Her medications included levothyroxine, metoprolol, pantoprazole, and warfarin. In June 2013, she underwent a barium swallow, upper endoscopy, and conventional manometry. The barium swallow showed a mildly dilated esophagus with tertiary contractions, delayed emptying of the esophagus, and a narrowed gastroesophageal junction (Fig. 1). The upper endoscopy demonstrated mild-moderate antral gastritis, normal appearing esophageal mucosa, and it was noted that the patient might have a tight gastroesophageal junction. The patient then underwent conventional manometry which was consistent with NSEMD. The patient was initiated on pantoprazole 40 mg daily. Unfortunately, after completing these tests she missed 3 follow-up appointments and was seen in our clinic only one year later.
Given her progressive dysphagia symptoms and weight loss, a high resolution esophageal manometry (HREM) was performed (Fig. 2). The study showed a normal median integrated residual pressure of 8 mmHg, normal mean resting pressure of 21 mmHg, normal mean residual pressure of 7 mmHg, and mean DCI of 10 770 mmHg sec cm. Of the 10 swallows, 30% were hypercontractile (> 8000 mmHg sec cm), 50% were normal and 20% simultaneous. Overall, the findings were consistent with jackhammer esophagus based on the Chicago Classification of Motility Disorders. Pantoprazole was increased to 40 mg twice daily with plan to add a pain modulator if her symptoms recurred.
Unfortunately, she was lost to follow-up again until she was seen in our clinic a year later in June 2015. She continued to have worsening dysphagia, lost an additional 7 kg, and reported new onset of occasional post-prandial chest tightness. At this point, a timed barium esophagram was performed (Fig. 3). This study demonstrated diffuse esophageal dilatation with retained secretions and barium with only 0-20% change in esophageal volume after 5 minutes. In addition, tertiary peristaltic waves within the distal esophagus, limited emptying of the contrast into the stomach and an air-fluid level were also noted. A repeat HREM was performed (Fig. 4) revealing a median integrated residual pressure of 71.5 mmHg, mean resting pressure of 97 mmHg, and mean residual pressure of 90 mmHg. There was panesophageal pressurization with every swallow and 80% of the swallows demonstrated prolonged pan-pressurization, all consistent with type II achalasia. Therapeutic options were discussed with the patient, and she elected to undergo Heller myotomy. The patient was referred to General Surgery but presented urgently to GI clinic 2 weeks later for worsening dysphagia, dehydration, weakness, inability to tolerate oral intake, and an additional 7-kg weight loss. The patient was admitted to the hospital and was noted to develop acute kidney injury. The patient underwent aggressive volume resuscitation. In addition, a nasogastric tube was placed and she was started on tube feeds to optimize her nutritional status. A week after discharge the patient underwent Heller myotomy with Dor fundoplication. On a follow-up visit with her general surgeon, she reported that she was tolerating liquids without any difficulties. The plan at that time was to initiate a soft diet.
|
esophageal achalasia, esophageal motility disorders, esophagus, manometry
| Not supported with pagination yet
| null |
PMC10030056_01
|
Male
| 1
|
As an industry that accounts for 21 billion euros annually, football has evolved enormously, whilst remaining rooted in a capitalist rationale. This is shown by the constant change that competition structure has undergone to make the game ever more economically advantageous for the most powerful contemporary football clubs. The history of the Champions League itself is a good example: when in 1955 the French newspaper L'Equipe conceived the notion of the former European Cup, this project was born without the approval of UEFA, which was only one year old at the time.
This being the case, we might question whether a slow but continuous process of Americanisation of the competition-spectacle model exists in Europe, in which the maximisation of profit is the primary goal of the participants. The various reforms that the format of the European Cup, known as the Champions League since 1993, has undergone, follow this logic. As Holt (, p. 35) states, the clubs were "increasingly dissatisfied by the level of income accrued by UEFA through the Champions League, and also the manner in which it was distributed". In the United States, based on a system of closed competitions, drafts are organised, salary limits are imposed and the commercialisation of audio-visual and advertising rights is centralised, taking advantage of the strength of the cartel with the aim of favouring a competitive balance.
This is the wider context within which the debate surrounding the creation (or not) of a European Super League of football has been contextualised. Indeed, since 1988, a number of proposals have been put forward, driven primarily by large media groups: Mediaset in 1988, Media Partners in 1998 and News Corporation in 2012. Shortly after the conglomerate founded by Rupert Murdoch articulated its own proposal, Badia reflected that the "the pressure exerted by the large media groups for the big European clubs to leave their domestic league and concentrate on a European Super League has become more present than ever and is gaining ever greater purpose, more based on the notion that the model of such a Super League would provide the participants and owners with significant negotiating capacity to increase their ordinary income (broadcasting, marketing and matchday)" (, p. 22).
Investment funds and banks have also been behind the European Super League project. Holt (, p. 35) explains how JP Morgan was involved in the Media Partners' attempt to create a Super League in 1998 with an investment of 1.2 million pounds. In this vein, in 2020 reports published by the Financial Times that CVC Capital Partners could have been interested in financing a global club super league project were joined, according to Sky News, by an initial proposal with JP Morgan as the main financial partner. The first visible targets were two English clubs: Liverpool and Manchester United. In recent years, two other clubs, Juventus and Real Madrid, have also been particularly active in promoting this new competition. JP Morgan had negotiated financing the promotion group with between 5 and 6 billion euros in order to start the new competition.
The launch of the Super League has thus been on the table of the big European football clubs for years, which have been a permanent pressure group to get UEFA to change the structure of the Champions League, to create greater competitive balance and from which they could receive more income for their participation. In the 2019-2020 season, the winner of the Champions League won around 111 million euros : out of the 2.04 billion euros that UEFA had to distribute among the participants. In relation to the proposal for the new competition, which was to be funded by JP Morgan, the Spanish newspaper Marca suggested that the winner could end up earning some 1 billion euros, a figure that would be impossible for UEFA to match, even with the arrival of new sponsors such as Barclays.
On the night of April 19, 2021, hours before the UEFA executive committee approved the new pyramid of its club competitions from season 2024-25, the break was completed. Twelve clubs, led by the President of Real Madrid, Florentino Perez, took the step and announced this new Super League: AC Milan, Arsenal FC, Atletico de Madrid, Chelsea, FC Barcelona, Inter Milan, Juventus, Liverpool, Manchester United, Manchester City, Real Madrid and Tottenham Hotspur. These big clubs opted for a championship model where there was more competitiveness between the participating teams and an even more commercial approach. This involved implementing a semi-closed competition, which relativised the maximisation of the result, in which there would be fifteen permanent participants and five more that varied depending on their performance in the previous seasons, similar to the way in which the basketball Euro-League already functions, which began in the season 2000-01 and is controlled by the private company Euro-league Basketball, with headquarters in Barcelona. This competition would have to coexist with national leagues.
However, two days later, the big six English clubs publicly distanced themselves from the project, citing the public and political pressure unleashed when it became public, at the same time that the prestigious economic news agency Bloomberg reported on a possible new $6 billion funding package for UEFA from the British investment firm Centricus Asset Management. One week later, Real Madrid, FC Barcelona and Juventus were left alone to defend, to different degrees, the project. While this may fail, the underlying debate will continue since the structural problems of European football (the economic and competitive imbalance) remain unresolved.
Although the Super League project is recent, it has already attracted academic attention. Just a few months after the failure of the project, different authors began publishing editorials or short commentaries in prominent journals highlighting the research perspectives that it has opened. Furthermore, in tandem with the Super League project, a PhD dissertation was published highlighting the need to revise the competitive structure of European competitions in order to improve the competitive balance between the clubs.
The most common justification among academics for the football Super League is based on the analysis that some researchers, especially from the field of sports economics, have made of the economic and competitive imbalance that exists between the clubs of the major European football leagues.
Jordi Badia also makes an interesting contribution from the communication sciences with the following summary (, p. 4): "The position of these authors has oscillated between those that believe that the current situation of combining the UCL with the domestic leagues is optimal, and those that consider that the richest clubs should compete exclusively in a European Super League of American format". Badia aligns himself with the latter, because he understands that those who defend the combination of the European and domestic competitions fail to take into account an essential factor: "The decisive weight that the competitive balance of the championships has on the clubs' economic viability" (, p. 4).
On the one hand, this author concludes that as soon as these clubs take part in the UCL, the revaluation of [their] intangibles becomes as, or more, important than the immediate revenue that the continental competition generates. On the other hand, this fact adds to the growing sporting difference between the main European clubs and the other participants in domestic competitions. According to Badia, who conducted a longitudinal analysis of results between 1996 and 97 to 2020-21 seasons, after the Bosman ruling and the rise of satellite televisions (Pay-TV) between 1994 and 1995, the economic inequality among the clubs is a progressive and unstoppable trend. Hence, economic inequality increases the difference in sport performance among clubs. "This difference in sport performance affects the competitiveness of the domestic leagues. It is only a matter of time, therefore, that this loss of competitiveness ends up affecting the interest of the spectators and, consequently, the commercial value of both, domestic leagues and their clubs, decreases" (, p. 163).
Based on competitive balance theory, FIFA is the first to endorse the fusion of competitions in emerging markets: the possible African Super League, the merger of the leagues of Mexico and the US or of Belgium and the Netherlands. Furthermore, "continental Super Leagues, if organised by club consortiums and not by confederations, would provide the perfect feeder tournaments for its own flagship competition, which it could then brand, promote and sell as "The Best", short-circuiting the existing continental cups (slow-killing them, in fact), and thereby weakening the economic and political power of confederations, two of which [Gianni] Infantino's FIFA perceives as a threat to its desire of total dominance, South American CONMEBOL and European UEFA". With regards to the European football competitions, things change when a new championship that escapes the control of UEFA is proposed globally in Europe, which is considered the cradle of modern football.
|
european super league, competitive balance, football (soccer), geopolitics, sports industry
| Not supported with pagination yet
| null |
PMC2647660_01
|
Female
| 9
|
Another approach to assessing the validity of the Lewis/Webster reading is to set it against qualitative evidence of local experience. This section examines public health in a region of the South West of England containing two County Councils, Somerset and Gloucestershire, and three County Boroughs, Bristol, Bath, and Gloucester. Hitherto the detailed work on local government health services has concentrated on cities in the Midlands and North, to the neglect both of Southern cities and counties with sparsely settled rural populations, so this region provides a valuable counterpoint. Pastoral agriculture dominated in Somerset, with its celebrated dairy industry, along with tourism and coal production; Gloucestershire's economy was also agricultural with some coal mining and textiles. Bristol (interwar population c.400,000) was England's seventh largest city and the South-West's major outport, as well as a commercial, manufacturing and service centre. Gloucester (c.50,000) was a river port and cathedral city and Bath (c.70,000) a wealthy spa and leisure town.
Taking Lewis's criticism of the failure of diphtheria immunisation first, local sources suggest that action was inhibited by the legacy of popular antivaccinationism. Gloucestershire for example had long been associated with opposition to compulsory vaccination against smallpox, with public protests continuing up until the last epidemic of variola minor, in 1923. Meanwhile in Somerset MOH William Savage (who Lewis admires as a <<thoughtful commentator>>) moved slowly for fear that <<(...) the <<anti>> people will whip up a certain amount of opposition>>. In Bristol the MOH embraced the science of Schick testing and child immunisation on the American model by the early 1920s, but the political environment inclined the health committee to a voluntary scheme, with resulting low levels of take-up. Moreover in rural areas the powers of public vaccination were situated not with the county council, but with smaller subsidiary authorities, the urban and rural districts. Thus when Gloucestershire's MOH, Kenneth Cowan, initiated a county-wide immunisation scheme this foundered on local resistance. Some districts rejected the scheme, and in others its implementation was remitted to individual GPs who followed inconsistent practices with regard to the numbers of doses. These considerations suggest that it is unreasonable to attribute all blame for Britain's lag in adopting diphtheria immunisation to public health departments. Given the anti-vaccinationist past, it is arguable that only a strong lead from the state would have ensured success, but this was not forthcoming.
Nor is the charge that the relationship between poverty and ill health was ignored during the interwar depression borne out. A striking example is a long essay on the <<vicious circle>> of poverty in the 1934 report of Bristol's MOH, Robert Parry, in which he implicated lack of <<family means>> in leading to <<slumdom>> and poor public health. Nor was there neglect of new research on nutrition. Frustrated with the poor guidance from central government on the classification of malnutrition in school medical inspections, and consequent subjectivity in assessments, Parry initiated a panel study of local 9-year olds. Taking as baseline their classifications as <<well>> or <<poorly>> nourished at age 5, researchers conducted physical and mental tests on the children and interviewed their parents, concluding that <<we can unhesitatingly say that it is the economic state of the family>> which explained malnutrition. This hardly tallies with Lewis's assumption that the <<community watchdog>> role was neglected. One might also expect, if Lewis's judgement is correct, to find that Parry was an enthusiast for social medicine. But although he was a supporter of Ryle, he regarded social medicine as <<(m)ere change of name>>, and essentially similar to <<preventive medicine>> in which he held the university chair at Bristol.
Lewis's argument that the activities of public health doctors were becoming increasingly indistinct from those of general practitioners is also hard to validate. One obvious difference was that environmental health remained within the MOH's remit in the interwar period. Historians have hitherto neglected this, assuming that practical aspects of the work had been devolved to the sanitary inspector's teams, but the MOH still retained overall direction. Some issues were relatively minor. In Gloucester for example the health department fought a long-running battle in the 1920s to persuade a parsimonious council to provide metal dustbins for waste collection. Elsewhere though, the nineteenth-century struggle to provide constant supplies of fresh running water continued. In Somerset for example, MOH William Savage moved quickly to identify places which still lacked mains connections, after the County Council gained powers from district authorities in 1929. Levels of funding were raised and regional planning was introduced, though even in the 1940s some dairy farming areas still relied on springs and wells.
More generally, the MOsH in the five authorities regarded housing improvement as a major health priority. Again the sanitary inspectorate was the workforce, but it was the MOH who was: <<the centre of the stage, the author of the plot and "villain" of the piece, as the principal witness and the butt of cross-examination>>. In rural areas an active programme of visiting by the MOH and housing inspector preceded demolition of <<hovels and quite insanitary houses>>, while health officials advocated the building of low rent public housing and attacked overcrowding. In the cities the MOH was involved in inspection, slum clearance, the issuing of improvement and closure notices, and the planning of public building programmes. <<Of all the causes which tend to undermine the health of the people>>, stated Bath MOH, James Blackett, in his 1920 report to the council, <<a shortage of suitable housing accommodation is by far the greatest>>. His argument was that damp and overcrowding led to TB, the spread of infectious diseases and high infant mortality. Thus, even in prosperous Bath, health officials highlighted social determinants of morbidity, and used the housing sections of annual reports to encourage local politicians to initiate improvement. As to the scale of these initiatives, consider the example of Bristol. In the mid-1930s the health department conducted about 1,400 housing inspections annually, with a yearly average of 980 houses found unfit for habitation. Overall the interwar corporation built about 15,000 new houses, of which about 25% were for families removed through slum clearance.
By coincidence, one of Lewis's key witnesses for the argument that MOsH were losing their preventive role was based in the region. She notes the criticism which William Savage, MOH for Somerset, launched in 1935 of the administrative burdens imposed by the LGA, which he saw as <<not our proper business>>. Hospital work and responsibility for mental health, he argued, detracted from the development of new preventive strategies, and Lewis concludes from this that MOsH were failing to keep abreast of new practices and remained wedded to traditionalism. Was Savage's analysis reliable, and his perspective representative? Consideration of his long and distinguished career in Somerset (1909-1937) suggests not. Born in 1873 he was close to retirement by 1935, describing himself as <<an old timer bred in the old tradition>>. His public health textbook of 1941 demonstrates that his goal was not to reinvent preventive medicine, but to preserve for the MOH the time-honoured environmental duties increasingly devolved to sanitary inspectors. Indeed, a Ministry of Health surveyor in 1931 wrote that Savage was disinclined to delegate and was unusually committed to environmental health, functioning as a <<sort of super sanitary inspector>>. His substantial published output in the 1930s confirms this orientation, dealing predominantly with issues of milk and food safety and bovine tuberculosis (matters close to his heart in cheese-producing Somerset), while his textbook concentrated on water supply, disposal of sewage and effluent, river pollution and housing inspection. Savage may also have been singularly ill-disposed towards the post-LGA administrative duties because of difficulties peculiar to Somerset. His efforts to assert control over the transferred Poor Law institutions in order to <<depauperise sickness>> were unsuccessful, as was his recommendation that the county should build a new central hospital rather than developing existing stock. These observations point to a potential problem with Lewis's methodology, heavily reliant on articles in the professional journals, which when contextualised may have a different significance.
Claims of administrative <<overstretch>> are further contradicted by regional evidence that public health departments experimented with new preventive strategies. One example is the advance of health education, then termed <<health propaganda>>. To a limited extent this followed national leadership, with <<health weeks>> and distribution of journals and posters, but it emanated principally from local initiative. It is ironic in the light of Savage's complaints about bureaucratic distractions that Somerset County Council provides a prime illustration. A full-time worker gave talks to Women's Institutes, Mothers Unions, schools, district nursing associations, village communities, girl guides, boy scouts and so on. Films and a travelling exhibition accompanied these health education efforts, whose explicit rationale was to promote healthy living and counter the ignorance deemed to cause much ill health. Clearly innovative health promotion was not confined to better known <<progressive>> authorities like Bermondsey. Another example is the use of TB screening programmes which began during wartime. The first step was Bristol's purchase of a <<mass miniature radiography unit>> used to screen selected groups, such as cohorts of school leavers, council staff, civil servants and BBC radio employees. A van was then purchased, which made the equipment available for temporary loan to the neighbouring authorities, with Bath for example screening government employees in the city. Screening and health promotion assumed greater significance as an activity of local government public health departments under the NHS, but they were rooted in the earlier period.
Behind the critique which Lewis and Webster level is the assumption that interwar public health might have done more to further social justice during the economic recession. Certainly the case is well made with respect to the inadequacy of food supplements in the depressed areas and the failure of public housing adequately to address the needs of the very poor. However, if we turn from a narrow definition of preventive medicine to consider the activities of public health departments more broadly it is possible to discern a significant advance in equity. First, much local government health work was funded through local taxation based on property values, which, with some provisos (related to geographical variations and to exemptions), was increasingly progressive. And while user fees were introduced for some services, they did not loom large as a source of revenue and were not aggressively pursued. For example, in the five South West authorities on average only 8% of the SMS was funded by recoupments from parents.
Second, the growing range of personal health services meant more equitable access. After 1929 the appropriated municipal hospitals admitted patients for acute medical care on the basis of local citizenship and they added significantly to the volume of available provision. In Bristol for instance the ex-Poor Law Southmead Hospital rapidly developed from long-stay institution to acute care hospital: 75 per cent of patients stayed for less than four weeks by 1938, and the number of maternity confinements increased from 127 in 1928 to 3,131 in 1942. There were also experiments in bringing services to previously under-served rural areas. Gloucestershire initiated an <<Extension of Medical Services Scheme>>, creating a network of <<out-stations>> in remote places, with regular clinics linked to general hospitals through formal referral networks. In Somerset a different approach was tried, with a system of "flying clinics" (ie without a fixed location) for rural areas. This developed from a series of nurse-led <<Mothers and Babies Afternoons>> to include a doctor and the health propaganda officer. In the cities similar initiatives were underway. Bristol pioneered the building of health centres aimed principally at infants and children and intended to provide a nexus for MCW and SMS work, to ensure joined-up working and a referral system to underpin inspections. These were not health centres on the Peckham model, but considering the entrenched opposition of private practitioners to any such initiative, they too represented an important extension of access.
Finally, with respect to patient dignity, the removal of many services from the Poor Law lessened the shame of accessing public medical services, and eroded, though did not destroy, the historic division between a <<respectable>> voluntary sector for the self-supporting and a lesser public sector for the dependent poor. The appropriation of Bristol's Southmead explicitly aimed to <<remove the stigma of pauperism>> so that the poor would receive <<hospital treatment (...) under the same conditions as the rest of the citizens>>. Even in the public assistance institutions, oral history shows that management styles were becoming more compassionate, professional and progressive.
| null | Not supported with pagination yet
| null |
PMC10213385_01
|
Female
| 5
|
A 16.5-year-old Indian female presented with secondary amenorrhea for six months. She had spontaneous thelarche at 9 years of age and menarche at 11 years of age, following which she had regular menstrual cycles for five years. She had a history of pulmonary tuberculosis 2 years prior, for which she received antitubercular pharmacotherapy for six months, after which she was declared cured. She was born at term from a non-consanguineous marriage and her perinatal history and her childhood development were unremarkable. At the time of presentation, she was a student of the tenth standard with average scholastic performance. She was lean, had no clinical evidence of hyperandrogenism and had received no treatment before her consultation. There was no history of recent weight loss, chronic stress or malnutrition and she had no history of galactorrhea, headache, seizures or visual deficit.
On examination, her height was 147 cm (between 3rd to 10th percentile; Height SDS: -1.59 SD, Indian Academy of Pediatrics 2015 growth chart references, Upper segment: Lower segment ratio = 0.9:1), her target height being 165 cm; her body weight was 55 kg (between 75th to 97th percentile, Weight SDS = + 0.51), BMI 23.8 kg/m2 (between 75th to 97th percentile, BMI SDS: +1.06). She had sinus tachycardia with a heart rate of 120/min and had stage 1 hypertension with clinic BP of 136/88 mm Hg. There was mild scoliosis with convexity to the right. She had a grade 1b goitre and grade 2 acanthosis nigricans. She had multiple cafe-au-lait macules with dimensions ranging from 5 to 25 mm distributed over her arms, thighs and backs (Figure 1), multiple lentigines over her face (Figure 2) and axillary freckling. She had cubitus valgus (Figure 3) and short fourth metacarpals. Systemic examination including cardiovascular, neurological, respiratory and abdominal examination revealed no abnormalities. Tanner's sexual maturity rating for her was B5P2A0 and she had unambiguous female genitalia with no clitoromegaly or hirsutism.
Initial investigations aimed at evaluating the etiology for short stature revealed normal results for most routinely tested parameters (Table 1). X-ray of her left wrist and hand showed a bone age of 17 years, which corroborated her chronologic age (Greulich and Pyle's atlas). She had subclinical hypothyroidism with TSH 5.6 mIU/ml, normal free T4, and TPO antibody was positive. She had evidence of primary ovarian failure with low estradiol inspite of high FSH and LH.
The clinical findings of proportionate short stature, secondary amenorrhea, cubitus valgus, multiple lentigines over face, scoliosis, hypertension and high FSH levels prompted further evaluation for Turner Syndrome. Karyotype of peripheral blood cells revealed mosaic TS with isochromosome Xq - 45, X [27]/46,X,i(X)(q10)[03] (Figure 4). Following a diagnosis of TS, relevant investigations to screen for comorbidities and complications known to be common in TS were done (Table 1).
The index case had a total of six cafe-au-lait macules out of which only four had diameters exceeding 15 mm. She also had evidence of axillary freckling. There were no subcutaneous or plexiform neurofibromata and upon slit lamp examination, she had no Lisch's nodules or distinctive osseous lesions like sphenoid dysplasia or tibial pseudoarthrosis. There was no known history of neurofibromatosis in any of her first-degree family members. Thus, she did not fulfil the classic criteria for a diagnosis of NF1, which was chiefly due to the smaller size of her cafe-au-lait macules than the cut-off of 15 mm required for diagnosis in the post-pubertal age. However, due to strong clinical suspicion, clinical exome sequencing was done which revealed a heterozygous single base pair deletion in exon 21 of the NF1 gene (chr17: g.29556477delA), which was a pathogenic variant consistent with NF1. Home blood pressure monitoring confirmed persistent stage 1 hypertension. Screening for phaeochromocytoma through 24-hour urinary fractionated metanephrines (metanephrines and normetanephrines) was done with normal results. MRI of the brain revealed no optic nerve glioma or CNS tumours.
She was started on estradiol valerate 2 mg daily and medroxyprogesterone 10 mg daily for 10 days every month which induced regular menstrual cycles. She was counselled regarding the poor prospect of future fertility and offered the option for oocyte cryopreservation. During therapy with estrogen and progesterone, she was closely monitored for any new appearance of neurofibromata or worsening of visual acuity or headache for the possibility of optic glioma expansion though she did not develop any of these in her two years of follow-up. She was advised against daily estrogen-progesterone combined pills as a therapeutic strategy to reduce progesterone exposure since there is evidence suggesting the permissive role of progesterone on neurofibroma expansion.
Her bone age was 17 years and X-ray of her knees revealed fusion of her upper tibial and distal femoral epiphyses and therefore she was not initiated on recombinant growth hormone therapy. She was given calcium 500 mg/day and cholecalciferol 2000 IU/day for bone health. She was started on metoprolol 25 mg once daily for her hypertension and sinus tachycardia to achieve a target heart rate of 60/min and SBP less than 130 mm Hg. She was periodically monitored with echocardiography and cardiac MRI for aortic root diameter and aortic size index, annual testing of liver function and metabolic profile, pure tone audiometry and BMD-DXA.
Written informed consent was obtained from the patient and her parents for publication of this case report and images of the patient and her genetic tests.
|
nf1, neurofibromatosis-1, neurofibromatosis-noonan syndrome, turner syndrome
| Not supported with pagination yet
| null |
PMC5548691_01
|
Male
| 35
|
The patient was a 35-year-old male with no history of major illness or family history of immunodeficiency. He had been doing well until he developed general fatigue, low-grade fever, and vomiting 2 weeks before this admission. Four days before admission, he went to a general hospital because of a persistent headache and a high fever of 39C. Nasopharyngeal swab tests for influenza A and B were both negative, and he was given ibuprofen. At midnight on the day of admission, he suddenly groaned, foamed at the mouth, and lost consciousness, and his wife witnessed his eyes turn upward. He was taken to hospital by ambulance and suffered 5 or 6 generalized seizures. Diazepam and phenobarbital were administered, and he finally was transferred to our hospital.
On physical examination, the patient was found to have a body temperature of 38.9C, a pulse rate of 94 beats/min, and a blood pressure of 131/81 mmHg, and marked hyperhidrosis and dark reddish skin eruptions were noted on the right forehead and scalp. No abnormalities of the chest, abdomen, or extremities were observed. A neurological examination revealed consciousness disturbance (Japan Coma Scale score: 100; Glasgow Coma Scale score: 6 [E1V1M4]), neck stiffness, and hyporeflexia of the bilateral lower extremities.
A chest X-ray and electrocardiogram demonstrated normal results. Brain magnetic resonance imaging (MRI) showed high-intensity lesions in the bilateral hippocampi and periventricular white matter on fluid-attenuated inversion recovery images (Fig. 1a-c). Diffusion-weighted imaging and apparent diffusion coefficient mapping showed high-intensity signals emanating from the lesions, which were suggestive of vasogenic edema (Fig. 1d and e). Contrast-enhanced brain MRI did not show any abnormal enhancement (data not shown).
The patient's white blood cell count was 12,420/mm3 (neutrophils: 88.7%, lymphocytes: 4.8%, monocytes: 4.9%, eosinophils: 0.1%, basophils: 0.3%, and atypical lymphocytes: 0.0%), and a test for C-reactive protein was negative (0.14 mg/dL). His creatine kinase was elevated (2,225 IU/L), which was considered to have been caused by the general seizures. Other routine blood and urine biochemical analyses did not detect any significant abnormalities.
A cerebrospinal fluid (CSF) analysis performed on admission revealed a normal pressure (16 cmH2O), a colorless appearance, pleocytosis (35/mm3) with a predominance of lymphocytes (94%), a high protein level (76.5 mg/dL), a normal glucose level (113 mg/dL; plasma glucose: 160 mg/dL), and a normal IgG level (4.6 mg/dL; IgG index: 0.50).
Polymerase chain reaction (PCR)-based tests of the CSF sample produced a positive result for HHV-7 DNA, but negative results for herpes simplex virus, varicella-zoster virus, Epstein-Barr virus, cytomegalovirus (CMV), and HHV-6. Repeated CSF tests confirmed the positive HHV-7 DNA result and the results of PCR were verified with positive and negative controls. A quantitative analysis of the HHV-7 DNA detected 3,800 copies/mL in the CSF specimen obtained on the 3rd hospital day, and a blood sample was also positive for HHV-7 DNA. The patient's serum HHV-7-IgG and IgM levels (fluorescence antibody technique) were 80x and <10x on admission, and his serum HHV-7-IgG level had increased on the follow-up examination (peak: 640x after 5 months), but his serum HHV-7-IgM level remained at <10x. The patient's CSF HHV-7-IgG and IgM levels were <1x and <1x, respectively. All viral DNA assays were performed by a commercial laboratory (SRL, Tokyo, Japan) (Fig. 2).
A culture of the patient's CSF did not show any growth of bacteria, mycobacteria, or fungi. PCR-based tests of the patient's CSF for mycobacterium tuberculosis and avium/intracellulare were negative, and a cytological examination did not show any malignant cells. A serological test for human immunodeficiency virus was negative. The rapid plasma reagin test for syphilis and the Treponema pallidum hemagglutinin test were also negative. Similarly, serological tests for autoantibodies including rheumatoid factor, antinuclear antibodies, and paraneoplastic antibodies (anti-Hu, Yo, and Ri) were all negative.
Tumor marker tests (carcinoembryonic antigen, alpha-fetoprotein, squamous cell carcinoma antigen, cytokeratin 19 fragment, and the soluble interleukin-2 receptor) all produced negative results. Contrast-enhanced chest and abdominal computed tomography (CT) exhibited normal findings. Brain single-photon emission CT (SPECT) and 67Ga-scintigraphy did not show any abnormalities. Electroencephalography (EEG) performed on admission showed slow waves in the frontal area, and the follow-up EEG demonstrated spikes in the left hemisphere.
Methylprednisolone (1 g/day, 3 days), intravenous immunoglobulins (IVIG) (5 g/day, 3 days), ceftriaxone (4 g/day, 7 days), acyclovir (1,500 mg/day, l4 days), glycerol (400 mL/day, 14 days), midazolam (20 mg/day, 14 days), and fosfluconazole (100 mg/day, 6 days) were administered. The patient's seizures and fever improved on the 2nd hospital day, and his consciousness gradually became clear. However, he demonstrated a severe memory disturbance. His revised Hasegawa Dementia Scale (HDS-R) score [a modified simple dementia scale in Japan, which is closely compatible with the Mini-Mental State Examination (MMSE)] was 12 points (full score: 30 points) on the 3rd hospital day and he could not recall any of 3 objects even after hints were given. We started to administer valproate (400 mg/day) on the 6th day. On the 9th day, myoclonus appeared; therefore, we added clonazepam (0.5 mg/day) and obtained a good response. His memory disturbance had disappeared by the 31st day [HDS-R score 29/30; MMSE 29/30; Wechsler adult intelligence scale-revised (WAIS-R) intelligent quotient (IQ) 86 verbal IQ (VIQ) 81, performance IQ (PIQ) 96)], and he left our hospital on the 37th hospital day. The patient's CSF cell counts normalized, and the HHV-7 DNA disappeared three months later (Fig. 2).
Since he suffered several seizures after being discharged, phenytoin and clobazam were added serially (Fig. 2). Carbamazepine was also used, but it was terminated because of a drug-induced lip eruption. At present, he is independent in terms of his daily life and ability to work. He has not shown any emotional or behavioral disturbances. He has been followed for 7 years at our clinic without any recurrence of his encephalitis. He is in good health and does not exhibit any signs of an immunocompromised state or autoimmune disorders.
Brain MRI showed a gradual improvement of the high-intensity areas in the hippocampus (data not shown). Blood tests for HHV-7 DNA continued to produce positive results throughout the follow-up period.
|
adult, encephalitis, human herpesvirus-7, immunocompetent
| Not supported with pagination yet
| null |
PMC8810502_01
|
Female
| 42
|
A 42-year-old woman presented for presurgical evaluation due to very frequent epileptic seizures. No febrile convulsions or significant medical antecedents in childhood were reported except for rare migraine attacks since puberty. Historically, the patient was operated at the age of 32 for autoimmune Hashitoxicosis (unfortunately, detailed medical documentation is missing) and was on treatment with 100 mug L-thyroxin. At the age of 36, she was diagnosed with HLA-B27-negative AS based on 6-month clinical manifestation of progressive (predominantly low) back and hip pain, 3-plane limitation of lumbar spine mobility, morning stiffness improving with motion, and confirmation by laboratory (elevated C-reactive protein and negative rheumatoid factor) and radiological findings (bilateral sacroiliac joints changes, syndesmophytosis, and enthesitis in neck and lumbar spine on X-ray). Anti-tumor necrosis factor (anti-TNF) therapy with etanercept (ETN) for 2 years led to AS remission and was discontinued due to tuberculosis (TBC) treated with 4-drug regimen for 4 months.
The epilepsy started at the of age 37 with six bilateral tonic-clonic seizures in sleep. A few months later, focal seizures with preserved or impaired awareness started, initially only few per month but later became weekly and in clusters. According to patient description, they did not change over time and occurred in awake state, and were characterized by goosebumps in the back of the neck, left shoulder, and left arm, followed by a cold sensation in the left hemibody, mostly in the upper extremity and shoulder, subsequent unpleasant, strong and sharp smell, "deja-vu"/"deja-vecu" experiences, and trembling of both hands, more on the right. Sometimes, because of a feeling of "breathlessness," she tried to breathe more deeply and frequently. Her relatives confirmed that usually she could warn at onset. Afterwards, motion arrest, eye closure, oral automatisms and facial flush were observed. Her right hand became stiff and immobile, while the left hand was squeezing. Postictally she could not speak and respond for minutes. Several anti-seizure drugs (ASDs) had no or minimal efficacy (oxcarbazepine and levetiracetam), and some of them had also marked adverse effects (valproate and carbamazepine). Therefore, the therapy consisted of lamotrigine (LTG) and low-dose clonazepam (CZP). Standard electroencephalograms (EEGs) were reported as showing left temporal epileptic focus, and four brain magnetic resonance imaging (MRI) examinations were interpreted as demonstrating left hippocampal sclerosis (HS). Memory problems appeared 2 years after epilepsy onset and increased 5 months before admission, when seizure frequency reached 30 to 40 per day.
During 3-day video-EEG monitoring, more than 150 seizures were recorded. They lasted 40-60 s and correlated to left temporal ictal change of initial attenuation and subsequent rhythmic epileptiform discharge (Figure 1A). In wakefulness, seizures occurred at fairly regular intervals of 3 to 4 per hour (Figure 1B). Rapid titration of topiramate and intravenous (IV) phenytoin did not reduce seizure frequency.
Brain MRI demonstrated bilateral mesiotemporal hyperintensity with clear left predominance and enlarged left amygdala (Figure 2A). Neuropsychological testing confirmed verbal memory deficit. Based on clinical history, presentation, and results from the examinations performed, we strongly suspected an autoimmune etiology. Serum and cerebrospinal fluid (CSF) autoimmunity testing by ELISA (Oxford University Hospitals Neuroimmunology Laboratory) was negative for anti-Caspr2, anti-NMDAR, anti-AMPAR-1/2, anti-GABA-b, anti-VGCC, anti-VGKC, and anti-LGI1 Ab, but very high anti-GAD-Ab titers of > 50,000 IU/ml (serum) and >10,000 IU/ml were (CSF) detected. Immunotherapy was immediately started with IV methylprednisolone (MPR), and later continued with IV immunoglobulins (IVIG) and cyclophosphamide, with only brief and transient effect despite decreased serum anti-GAD Ab-titers (15,000 IU/ml).
Control brain MRI did not show progression of mesiotemporal abnormalities but fluorodeoxyglucose-positron emission tomography (FDG-PET) revealed very restricted hypermetabolism in the left amygdala correlating to an ictal event during the examination, as reported by the patient (Figures 2B,C). Because of lacking efficacy of ASDs and immunotherapy, and after thorough discussions with the patient and relatives on the chances and risks of epilepsy surgery, we performed a very selective left amygdalectomy. Postoperatively, the patient was 3 months seizure-free but later continued to experience shorter focal aware/unaware seizures mostly with behavioral arrest, decreased/absent responsiveness, and milder dyscognitive features but without the initial vegetative/somatosensory and the later motor signs. Seizure frequency stabilized at 3 to 4 per month with no change in ASDs, an outcome corresponding to Engel Class IIIA.
One year later, the patient was diagnosed with DM1 that was initially difficult to compensate, and transient increase in seizure frequency of up to 1-2 daily for about 2 months was also noted. Later on, with Insulin Apart 6 UI t.i.d. and Insulin Degludec 16 UI q.d., the condition with regard to both DM1 and TLE (weekly non-disabling focal seizures) was stable and did not require further treatment adjustments.
Approximately 4 years after the operation, rare episodes of dizziness and falls were reported and interpreted by the patient as probable seizures. They were not recorded on VEEG, but right temporal epileptiform activity was registered, and lacosamide (LCM) was added. The patient continued to report intermittent unsteady gait, diplopia, vertigo, and more frequent falls, related or not to LCM intake. No changes in the neurological exam, MRI, and EEG were found; therefore, these complaints were attributed to LCM and prompted its replacement with brivaracetam without any improvement. Gradually, over the next 5 months, the patient developed a full-blown picture of CA with severe locomotor ataxia, dysdiadochokinesis, dysmetria, nystagmus, and dysarthria. Brain MRI did not reveal obvious signs of cerebellar atrophy but only progress in left HS (Figure 3). Paraneoplastic auto-Ab testing was negative for anti-Hu (ANNA-1), anti-Ri (ANNA-2), anti-Yo (PCA-1), anti-PNMA2 (Ma2/Ta), anti-Tr (DNER), anti-amphiphysin, anti-CV-2, anti-Sox-1, anti-ZIC4, anti-recoverin, and anti-titin Ab, but high anti-GAD65 titers of 67 U/ml persisted (positive if >= 10 U/ml, strong positive >= 50 U/ml; [10 U/ml 180 IU/ml]).
|
gad, autoimmune, cerebellar, diabetes, epilepsy, insular, surgery, temporal
| Not supported with pagination yet
| null |
PMC3298010_01
|
Female
| 44
|
A 44-year-old native woman was admitted to our hospital for right upper quadrant discomfort and jaundice in July 2006. The patient had been healthy until 4 months prior to admission when upper abdominal pain of unknown origin first began; at that time, icteric eyes and skin with darkened urine were noted. She felt somewhat nauseous but did not vomit. There was no fever, cough, headache, diarrhea or other mental or physical complaints. Despite taking ursodeoxycholic acid capsules prescribed by her primary care provider for 20 days, she still felt malaise with no improvement in her symptoms. Three days prior to this hospitalization, she experienced a sudden onset of colicky pain in the stomach and back. The patient's past medical history and family history were unremarkable, and she had not been exposed to any drugs, toxins, or blood products. She had been a rural resident until marriage as an adult and resided in town. She had been in a monogamous relationship with her husband for more than 20 years and had no risk factors for HIV infection.
On physical examination, she was a well-developed woman with a body weight of 54 kg (body mass index: 20.6 kg/m2), although she had lost 10 kg over the last 4 months. Her vital signs were stable and within normal limits, and no positive physical exam findings except scleral icterus were present. Her complete blood count, coagulation factors, erythrocyte sedimentation rate, serum immunoglobulin, CEA, AFP and CA125 were normal, whereas anti-hepatitis viruses (A-E), anti-HIV and abnormal autoimmune antibodies were negative. Urine and stool tests were consistent with obstructive jaundice. The liver function tests and CA19-9 results are summarized in Table 1. Moreover, the flow cytometry lymphocyte subpopulations were normal at 49% CD3+, 21% CD4+, 23.4% CD8+, and 19.5% natural killer cells (TBNKTM, Latonia, KY). Her chest CT, gastroscopy and colonoscopy demonstrated no abnormalities, although abdominal ultrasonography revealed extra- and intrahepatic biliary dilatation. The outer CBD diameter was dilated to 16.1 mm as its maximum, but the lumen was narrowed to almost zero due to an irregular thickened wall (7.4 mm at its maximum). No findings that were consistent with bile duct calculus, liver neoplasm or cirrhosis were identified. Furthermore, an abdominal contrast-enhanced CT scan and an MRCP produced similar findings, i.e., a hilar biliary stricture with no evidence of abdominal masses or lymphadenopathy (Fig. 1). Accordingly, an ERCP was performed, which revealed an isolated irregular proximal CBD stricture with dilated intrahepatic biliary trees and no duodenal or ampullary tumors as well as negative brush cytology (Fig. 2A). After a multidisciplinary consultation with the gastroenterologists, radiologists and oncologists, a consensus diagnosis of primary sclerosing cholangitis rather than cholangiocarcinoma was made, partly due to the lack of malignant features consistent with cholangiocarcinoma in all of the medical images. Thus, the patient was transferred to the Gastroenterology Dept. and received a conventional diagnostic plan of glucocorticoid-free treatment. Unfortunately, within the first week posterior to the ERCP, her hyperbilirubinemia worsened, with TBil and DBil increasing to 203.5 mumol/L and 123.1 mumol/L, respectively. At that time, a repeat MRCP revealed a more indistinct biliary bifurcation and CHD, which was pathognomonic for malignancy (Fig. 1F). Therefore, a hepatic hilar cholangiocarcinoma (Klatskin tumor) emerged as the new presumptive diagnosis, and thus, an exploratory laparotomy was scheduled after comprehensive consent was obtained.
Intraoperatively, the liver was noted to be tough, green-brown in color, of generally normal size and without any masses or perihepatic enlarged lymph nodes. Thickened walls of the cystic duct, CHD, proximal CBD and initial part of the bilateral hepatic ducts were detected and ranged from 0.5-1.0 cm, which consequently narrowed the bile channels. Upon choledochotomy, packed gelatin-like exudates were found inside the lumen, and thus, a flexible choledochoscopy was performed, the results of which supported the above findings. The gallbladder and a strip of the morbid CBD were removed and submitted for frozen section pathological examination. Both samples demonstrated inflammatory granulomas with round refractive corpuscles based on hematoxylin-eosin staining. Therefore, the diagnosis of biliary cryptococcosis was made, and only T-tube drainage was performed.
Next, following the operation, the blood, bile, sputum, CSF, urine and stool samples were cultured simultaneously with ink staining at least in triplicate. All samples were negative for Cryptococcus, but the final paraffin pathology of the intraoperative biliary samples confirmed the definite diagnosis. Microscopic examination demonstrated numerous encapsulated yeast-like organisms measuring 7-10 mum in diameter scattered in multinucleated giant cells and fibrous tissues throughout the CBD and gallbladder wall. The periodic acid methenamine silver stain, periodic acid Schiff stain and India ink stain were all positive (Fig. 4), whereas the pathogen identified by polymerase chain reaction from the paraffin-embedded gallbladder was Cryptococcus neoformans (VNI type1). As soon as she completed an uneventful recovery, she was transferred to the Gastroenterology Dept. again for anti-fungal therapy (beginning on POD11), an itraconazole regimen consisting of 2 weeks of inpatient intravenous injections (Sporanox IV, Xian-Janssen Pharmaceutical Ltd., Beijing, China) followed by 10 weeks of per os outpatient therapy (Sporanox Capsule). At two weeks after discharge (i.e., 4 weeks after starting the therapy), her transaminases had returned to normal levels, whereas TBil/DBil levels normalized after an additional 4 weeks. Moreover, her T-tube was removed after a cholangiography (Fig. 2B).
During her follow-ups, she appeared to have some occasional abdominal discomfort, mainly in the right upper quadrant, but no jaundice. Regular repeat abdominal ultrasonographies revealed no extra- or intrahepatic biliary dilatation, and the thickness of CBD wall dropped to 2.2 mm (April 2009).
Unexpectedly, more than 4 years after her original presentation (September 2010), she was re-hospitalized for hyperbilirubinemia. Similar to the first admission, her examination was otherwise normal, and all body fluids were negative for Cryptococcus. The liver function test results from this presentation are listed in Table 1. Radiologic exams at this time revealed a thickened CBD wall, narrow CBD lumen and moderately dilated intrahepatic bile ducts, which were also similar to but slightly less severe than her initial presentation (Fig. 3). Instead of an ERCP, an EUS was performed; it demonstrated a benign change of her CBD entirely from the hilar bifurcation to the duodenal ampulla. A cholangioenterostomy was under deliberation; however, her icterus gradually and spontaneously vanished. By the 14th inpatient day, the patient had recovered and was again discharged. As of the time of this writing, another 16 months of follow-up have passed with no abnormalities detected.
|
biliary cryptococcosis, cholangiocarcinoma, cryptococcus neoformans, itraconazole, obstructive jaundice
| Not supported with pagination yet
| null |
PMC9125057_01
|
Female
| 61
|
A 61-year-old woman with a kidney-pancreas transplant in 2004 followed by a second kidney transplant in 2017 presented to the emergency department with recurrent falls in the setting of several weeks of diarrhea with generalized weakness and progressive bilateral lower extremity swelling. Prior to admission, the patient was having 6 to 7 loose bowel movements per day for 2 weeks. The number of episodes and volume were not affected by food intake. The large-volume, high-frequency diarrhea was debilitating and subsequently led to decreased oral intake and a decline in her overall nutritional status. Additional medical history was pertinent for type 2 diabetes. For her transplantation, she had been induced with anti-thymocyte globulin, and maintenance immunosuppression at the time of admission consisted of tacrolimus, mycophenolate mofetil, and prednisone. Social history was unremarkable. She lived alone, denied sick contacts, did not smoke or drink, and had not traveled in or outside the country.
On admission to our institution, vital signs demonstrated a temperature of 36.4 C, blood pressure of 131/68 mm Hg, heart rate of 64 beats per minute, respiratory rate of 16 breaths per minute, and oxygen saturation of 97% on room air. Her weight was 47.2 kg and body mass index was 17.32 kg/m2. Physical examination revealed a pale, frail, ill-appearing lady with generalized muscle wasting in her extremities, bilateral temporal wasting, and loss of her buccal pad of fat. She had 2 plus pitting lower extremity edema up to the hips. There was no fluid wave on abdominal examination.
Renal function tests demonstrated a creatinine of 4.5 mg/dL (increased from baseline 3.5 mg/dL), blood urea nitrogen of 65 mg/dL, and bicarbonate of 16 mmol/L. Serum albumin was 1.7 g/dL. Complete blood count demonstrated hemoglobin of 6.8 g/dL (baseline, 7.5-9 g/dL), mean corpuscular volume of 91.5 fL, and white blood cell count of 6.37K/cumm. Gastrointestinal (GI) polymerase chain reaction was tested and was negative on 2 occasions. Fecal calprotectin and fecal leucocytes were also negative. The stool was negative for cyclospora.
Due to her severe diarrhea and cytomegalovirus (CMV) plasma viral load of 1823 IU/mL, intravenous ganciclovir was started for a presumed diagnosis of CMV colitis. Colonoscopy showed a few sessile polyps with a few diverticula but was overall unremarkable. Colonic biopsies were negative for CMV on histopathological examination. Given her persistent symptoms despite ganciclovir therapy, an upper endoscopy was performed, which demonstrated erosive gastropathy and mucosal changes with nodularity in the first and second parts of the duodenum.(Figure 1A, B)
On hospital day 7, ova and parasite stool study returned 3+ positive for acid fast bacteria (AFB). Around the same time, blood cultures also turned AFB-positive. Histopathologic results from the duodenal biopsy demonstrated abundant AFB organisms present within lamina propria macrophages and acid fast organisms consistent with mycobacteria seen on Kinyoun stain (auramine-rhodamine-positive, periodic acid-Schiff with diastase-positive) (Figure 1C and D).
Transplant infectious disease team was consulted and she was started on a multidrug regimen to cover both NTM and mycobacterium tuberculosis, adjusting for renal function, with ethambutol, azithromycin, rifabutin, amikacin, isoniazid, and moxifloxacin.
Unfortunately, despite appropriate treatment and supportive measures, our patient continued to deteriorate. She developed respiratory distress and acute hypoxic respiratory failure requiring transfer to the intensive care where she was intubated. Chest imaging demonstrated bilateral multifocal pneumonia with bilateral pleural effusions. Bronchoalveolar lavage and pleural fluid analysis returned AFB-positive, confirming a diagnosis of disseminated NTM. The organism was later identified as Mycobacterium avium complex (MAC).
Over the next few days, her kidney function continued to deteriorate and she ultimately lost renal graft function all together. Her tenuous hemodynamics did not permit intermittent hemodialysis and she needed to be on continuous renal replacement therapy. After spending 2 months in the hospital with repeated trips to intensive care, she succumbed to the disseminated infection.
|
mycobacterium avium complex, diarrhea, nontuberculous mycobacteria, solid organ transplant
| Not supported with pagination yet
| null |
PMC9633737_01
|
Male
| 19
|
A 19-year-old man presented with peripheral spondyloarthritis characterized by typical magnetic resonance imaging (Fig. 1). The patient was prescribed 150 mg secukinumab monthly starting in May 2020 after a poor response to sulfasalazine and non-steroidal anti-inflammatory drugs. Before receiving biologic therapy, the patient was screened for latent TB and was found to have a negative QuantiFERON-TB test and a normal chest X-ray. The patient had a good response to secukinumab, and musculoskeletal symptoms improved significantly after several months of treatment.
In April 2022, the patient complained of fatigue, mild fever, productive cough, and weight loss lasting for 2 weeks. Physical examination revealed crackle rales in the left lung and no abnormalities in any other organs. Laboratory results demonstrated an elevated C-reactive protein level of 44 mg/dL and an erythrocyte sedimentation rate of 75 mm. Other hematological and biochemical tests, such as complete blood count, liver enzymes, renal function, glycemia, and thyroid function, were normal. The patient tested negative for human immune-deficiency virus, hepatitis B virus, and hepatitis C virus. Chest X-ray showed left upper lobe consolidation (Fig. 2), and a bronchoalveolar lavage test indicated a negative acid-fast Bacilli smear and detected M tuberculosis, which was found to be sensitive to rifampicin and isoniazid by a line probe assay. Bronchoscopic biopsy demonstrated caseating granulomatous inflammation (Fig. 3).
The patient was diagnosed with active pulmonary TB. The initial treatment for TB was 4 antibiotics: isoniazid, rifampicin, pyrazinamide, and ethambutol. Secukinumab was discontinued due to concerns regarding the reactivation of latent TB. Joint pain and back pain were eased by high doses of non-steroidal anti-inflammatory drugs. After 8 weeks of anti-TB treatment, the patient's fever and respiratory symptoms resolved, and the lesion on the chest X-ray improved. Joint pain and back pain were mostly controlled.
|
secukinumab, spondyloarthritis, tuberculosis
| Not supported with pagination yet
| null |
PMC7531270_01
|
Male
| 72
|
A 72-year-old man with a past medical history of AA, amegakaryocytic thrombocytopenia and chronic kidney disease (CKD) stage 3 presented in April 2020 with high fever, cough, and progressive fatigue.
Aplastic anemia had been diagnosed in 2014 and initially responded to therapy with horse anti-thymocyte globulin (ATG), methylprednisolone and cyclosporine A. Nine months later, his disease progressed and he was treated with rabbit ATG, methylprednisolone, and cyclosporine A. In late 2019, his disease progressed primarily as thrombocytopenia due to acquired amegakaryocytic thrombocytopenia (AAT). He received four doses of rituximab in early 2020 for treatment of AAT without response. Bone marrow biopsy performed 2 months prior to presentation was hypocellular with 15-20% bilineage hematopoiesis and near absence of megakaryocytes without evidence of dysplasia or increased blasts. While his diagnosis is most accurately classified as AAT at the time of presentation, he had been consistently pancytopenic for 4 months despite treatment with cyclosporine A and remained dependent on platelet transfusions. Family and psycho-social history were non-contributory.
Physical examination at the time of presentation was notable for fever of 103.7F and mild tachycardia (HR 104) with normal oxygen saturation (97%) without supplemental oxygen. He appeared in mild respiratory distress, but pulmonary examination was unremarkable. His initial laboratory studies were notable for severe lymphopenia, which was different from his baseline pancytopenia and elevated C-reactive protein (CRP) and IL-6 as noted in Table 1. Chest radiograph revealed a consolidation in the left midlung, possibly representative of pneumonia (Figure 1A).
The patient was treated empirically with hydroxychloroquine (400 mg twice a day for 1 day, followed by 200 mg twice a day for 4 days) for possible COVID-19 in the setting of immunosuppression. Ceftriaxone and azithromycin were added for possible community acquired pneumonia. On admission day 0, SARS-CoV-2 PCR nasal swab came back positive. For the first several days of the hospitalization, the patient had persistent fever (range 100-104F) and intermittently required oxygen up to 2 L/min via nasal cannula to maintain oxygen saturation above 94%.
By hospital day 5, he had developed progressive pancytopenia and required near daily transfusion of packed red blood cells and platelets. Due to persistent high fever, and now neutropenia, antibacterial coverage was broadened to cefepime, then meropenem, and antifungal prophylaxis with posaconazole was added. He was also started on tbo-filgrastim and eltrombopag for management of severe neutropenia and thrombocytopenia.
On hospital day 9, the patient developed lethargy and hypoxia requiring supplemental oxygen of 6 L/min via nasal cannula and subsequently Venturi mask at 50% FiO2. Repeat chest radiograph demonstrated significant worsening with bilateral pulmonary infiltrates (Figure 1B). Inflammatory markers, including ferritin and CRP were significantly increased and acute on chronic renal failure worsened (Table 1).
The clinical picture was consistent with cytokine storm due to severe COVID-19 and the patient promptly received tocilizumab 5 mg/kg (400 mg) on hospital day 11. The patient received a single dose of methylprednisolone 60 mg IV immediately prior to tocilizumab administration but did not otherwise receive corticosteroid treatment during his admission. The same day, the patient's fever resolved and he developed mild hypothermia (minimum 94.6F). Over the next several days, his temperature normalized, oxygen requirements steadily declined, inflammatory markers decreased and acute renal failure resolved. The patient remained pancytopenic and transfusion dependent. Meropenem was discontinued and he was transitioned to oral levofloxacin prophylaxis due to persistent neutropenia.
On hospital day 18, the patient developed mild recurrent fever (100.5F, repeat 100.9F). The following day, his fever increased significantly to 103.9F and his oxygen requirement increased to 10 L/min. Chest radiograph revealed new pulmonary infiltrates (Figure 1C). Cefepime was resumed and vancomycin and therapeutic voriconazole were added. Despite these efforts, the patient developed mild hypotension (BP 90/40) which resolved with fluid resuscitation. Antibiotics were further broadened to meropenem and the patient received a single dose of tobramycin. Inflammatory markers at this time were relatively stable.
By hospital day 20, the patient had again clinically improved. His blood pressure remained within normal range and his oxygen requirement decreased to 6 L/min by nasal cannula. The exact cause of his brief clinical deterioration was unclear but was presumed to be due to a secondary bacterial pneumonia. Over the subsequent days, he completed a 7-day course of meropenem and vancomycin. He remained afebrile on this treatment and his oxygen requirement slowly normalized. His repeat IL-6 level on day 28 was significantly elevated at 1223.8 pg/mL.
The patient was discharged home on hospital day 31. He remained pancytopenic and transfusion dependent at the time of hospital discharge. Notably, his SARS-CoV-2 PCR testing has remained persistently positive as of hospital day 29 (with the exception of a single negative result on day 17, which was thought to be a false negative). It is unclear whether detectable viral RNA represents persistent infectiousness or whether non-infectious RNA fragments are being detected. Twenty two days after hospital discharge, SARS-CoV-2 PCR was repeated and was negative.
|
covid-19, sars-cov-2, aplastic anemia, cytokine storm, tocilizumab
| Not supported with pagination yet
| null |
PMC6918496_01
|
Female
| 57
|
A 57-year-old woman was hospitalized due to dyspnoea, cough, and bilateral interstitial infiltrates on chest X-ray (Figure 1a). She moved from Venezuela to Germany a year prior to presentation. A chest computed tomography (CT) showed diffuse interstitial changes (Figure 1b). Flexible bronchoscopy revealed a black exophytic mass migrating through the left main bronchus wall. Subsequently, a peripheral lung biopsy was obtained using video-assisted thoracoscopy (VATS). Histological analysis of the peripheral lung biopsy by local pathology showed large interstitial granulomas, giant cells, and no necrosis or signs of vasculitis, compatible with sarcoidosis. In combination with elevated serum interleukin-2-receptor levels (1654 U/ml, normal 220-710 U/ml), pulmonary sarcoidosis was diagnosed and systemic corticosteroid therapy (1 mg per kg bodyweight) was initiated; 3 months later clinic and chest CT remained unchanged (Figure 1c), as did the lesion in the left main bronchus under bronchoscopy. A lesional biopsy revealed necrosis and inflammation. Viridans streptococci were isolated from the broncheoalveolar lavage (BAL), as well as low levels of unspecified yeast after 7 days of incubation. The latter was not found on microscopic analysis of the BAL or, at the time of testing, in the lesional biopsy. Meropenem was initiated but the patient developed progressive respiratory failure requiring noninvasive ventilation (NIV) and was transferred to our university clinic.
Subsequent re-examination of the initial (VATS) lung biopsy by our pathologists led to the detection of intracellular Grocott positive particles of 2-5 microm, accompanied by giant cell pneumonia, suggestive of a yeast infection. While initial stains showed small yeasts more in line with Histoplasmosis, more in depth examination of the samples showed multi-budded yeasts (Figure 1d) more closely resembling Paracoccidioides, albeit not matching the textbook description of a multipolar 'pilot's wheel' configuration. Due to this ambiguity, we were unable to differentiate between these fungi by histology alone.
Due to the histologic finding, and since the patient had recently moved from a region endemic for dimorphic fungal pathogens, serologic testing was performed for histoplasmosis using immunodiffusion (ID) and complement fixing (CF) methods, as well as for paracoccidioidomycosis (ID) and coccidioidomycosis (ID, CF). Histoplasma and Paracoccidioides antibodies were detected by ID. However, a lack of antibodies against Histoplasma in the CF-test was interpreted as a likely cross-reaction in Histoplasma ID and evidence for paracoccidioidomycosis. CF testing for PCM was not available at our laboratory. In addition, a specific qPCR assay targeting the ITS-1 region of Histoplasma and broad range PCR assays targeting the 28S and ITS-2 region were performed from the formalin-fixed, paraffin-embedded (FFPE) lung biopsy as described previously. While correct DNA extraction was shown by amplification of the human 18S rRNA gene, and an internal amplification control excluded PCR inhibition, both broadrange fungal PCRs amplified DNA. Negative Mastermix-, and extraction controls ruled out contamination with fungal DNA. Sequencing of both broad range PCR amplicons showed 99.7% pairwise identity with sequences of Paracoccidioides brasiliensis as the causative pathogen. The specific Histoplasma PCR did not amplify DNA.
In conclusion, the patient had paracoccidioidomycosis as suggested by the detection of antibodies, histology (Figure 1d,e) and PCR from FFPE tissue. Cerebral or abdominal involvement was ruled out by cMRI and abdominal ultrasound. Sarcoidosis was excluded after histopathological re-evaluation. We initiated a therapy using liposomal amphotericin B 150 mg once per day. Within 3 days, the respiratory situation improved and noninvasive ventilation was discontinued. After 3 weeks, therapy was switched to oral itraconazole 200 mg twice daily, and, after 8 weeks of antifungal treatment, oxygenation had returned to baseline and no additional oxygen supplementation was required. Chest CT showed decreasing pulmonary infiltrates (Figure 1f) which resolved completely after 6 months (Figure 1g). To monitor the lesion in the left main bronchus, another flexible bronchoscopy was performed after 6 months, showing a decrease in size of the fungal lesion (Figure 1h). However, in the BAL, yeasts were still detected by microscopy but fungal culture and PCR remained negative. Treatment with itraconazole 200 mg twice daily was continued.
|
venezuela, female, lung, paracoccidioidomycosis, sarcoidosis
| Not supported with pagination yet
| null |
PMC6157168_02
|
Female
| 31
|
A 31-year-old female patient complained a mass in her maxilla and mandible. The mass was gradually increasing in size over the last 6 months. The mass caused talking disturbances, swallowing difficulty, dilating gap between upper teeth, and narrowing gap between lower teeth. She also complained hard masses in her fingers and the middle of chest. There was a decrease in height (15 cm in 8 years) and weight (19 kg in 8 years). The patient was on end stage of renal failure and has been treated with hemodialysis 2 times per week for eight years since 2008. The patient was planned to be performed renal transplantation. Comorbidities were reported among this patient, which are chronic heart failure, grade II hypertension, lung tuberculosis on therapy, and hepatitis C. The physical examination showed facial asymmetry with mass in the palatum durum, measured 4 x 3 cm in size (Figure 5). Palpation revealed hard consistency originated from the bone, smooth surface, and no tenderness.
X-ray of the maxilla and mandible showed diffused decreasing bone density and multiple lytic lesion, which suggest metabolic bone disease or renal osteodystrophy or hyperparathyroidism (Figure 6). BNO showed compression fracture in thoracolumbar vertebrae and multiple calcification in soft tissue abdominal and pelvic region compatible with renal osteodystrophy. Bone survey also showed the decreased bone density. Facial CT with contrast showed diffuse hyperostosis, lytic lesion in the mandible bone, maxillary bone, cranium, and multiple calcification in the soft tissue which is compatible with osteorenal dystrophy (Figure 7). Thyroid ultrasonography showed bilateral multiple cysts in the thyroid, a firm lesion with calcification in the left parathyroid fossa, and oval lesion in the left pericarotid. Parathyroid scan showed an increased activity in the inferior aspect of left thyroid which was suggestive of parathyroid adenoma.
Laboratory examination showed the elevated ureum (202 mg/dL), creatinine (11.1 mg/dL), and normal calcium level (9.7 mg/dL). In November 2016, PTH level was >5000 pg/mL and phosphate level was 6.1 mg/dL.
Physical examination and laboratory examination lead us to brown tumor as the diagnosis due to secondary hyperparathyroidism. The treatment for this patient was subtotal parathyroidectomy with frozen section and intraoperative PTH evaluation. Intraoperative parathyroid hormone level was evaluated following subtotal parathyroidectomy. The result decreased to normal range 10.37 pg/mL (normal range: 10-65 pg/mL). Frozen section revealed hyperplasia of the parathyroid. Calcium level was evaluated every day for 7 days, and the result ranged from 5.0 mg/dL to 7.4 mg/dL. The parathyroid hormone on the 3rd month following the surgery was 4.31 pg/mL. Definitive pathology anatomy result revealed hyperplasia of the parathyroid with water clear cell parathyroid adenoma.
The masses in the maxilla, mandible, and sternum were observed to be significantly regressed. This patient then underwent renal transplantation approximately 4 months following parathyroidectomy. One month following the renal transplantation, PTH level was 6 pg/mL.
| null | Not supported with pagination yet
| null |
PMC5433578_01
|
Unknown
| 74
|
A 74 years old patient, diagnosed one month prior with giant cells arteritis (GCA), treated with methylprednisolone and azathioprine, presented with a superficial, soft, painless mass on the right frontal scalp region, noticed by the patient to have grown slowly in the previous week. From the medical history we report pulmonary tuberculosis (treated in 1975), Lyme borreliosis treated with intravenous ceftriaxone in March 2013, followed by moderate Clostridium difficile infection. The laboratory tests revealed increased erythrocyte sedimentation rate (ESR=120 mm/hour) and C-reactive protein (CRP=2.05 mg/dl), hyper-potassemia (5.9 mEq/l) and hypo-sodemia (132 mEq/l).
One week before, physical examination had shown an enlarged painful left temporal artery and a previously absent right frontal scar, most probably a cutaneous superficial atrophy due to vasculitis (Figure 1a, b). Therefore, the new frontal mass suggested a hematoma from a vasculitis-injured vessel effraction, or a superficial arterial aneurysm. Ultrasonography, performed with an Esaote MyLab Five machine with a 10-13 MHz multifrequence linear transducer, showed an aspect compatible with an abscess (Figure 2). A puncture from the scalp collection reveled pus; a drainage tube was inserted for complete evacuation. The microscopic examination revealed Gram-positive long thin branching roads and culture on Colombia blood agar was indicative of Nocardia spp. with white chalky colonies after 3 days of incubation (Figure 3). Because direct inoculation of Nocardia was less probable (no skin lesion was visible and the patient did not recall any injury), disseminated infection was suspected. The cerebral MRI and abdominal CT did not find any abscesses. The pulmonary radiography detected left apical and subclavicular nodules recalling previous healed pulmonary tuberculosis, but also multiple bilateral subclavicular nodular opacities, the latter not present on the x-ray performed one month before, suggestive of possible tuberculosis reactivation or pulmonary nocardiosis. A bronchoscopy with bronchoalveolar aspiration showed the aspect of suppurative bronchitis; culture identified Acinetobacter calcoaceticus, interpreted as colonization and not as infection, as no pneumonia was detected on X-ray findings. Pulmonary CT scan showed the image of a subcarinal mediastinal mass with polycyclic shape, 6 cm in diameter, other mediastinal adenopathies and minimal bilateral pleural effusion. The transbronchial biopsy was not successful, while diagnostic thoracotomy could not be performed, due to the altered status of the patient.
The patient underwent therapy with intravenous imipenem-cilastatin 2g/day for 21 days and Amikacin 1g/day for 14 days for Nocardia infection, associated with tuberculostatic therapy (rifampicin, izoniazide, ethambutol and pyrazinamide). Tuberculostatic therapy was initiated due to the presence of radiologic subclavicular nodular opacities in a patient with immunosuppressive therapy and history of pulmonary tuberculosis and was stopped when cultures for Mycobacterium tuberculosis were reported as negative.
The scalp collection completely resolved after surgical drainage and antibiotic therapy. Therapy for nocardiosis was recommended after discharge with long term trimethoprim-sulfamethoxazole (TMP 10 mg/kg/day), justified by the immunocompromised status and immunosuppressive therapy. However, after 2 months the patient developed drug-induced myelosuppression and a severe and complicated recurrent Clostridium difficile infection that led to multiorgan dysfunction and exitus.
|
giant cell arteritis, nocardiosis, subcutaneous abscess
| Not supported with pagination yet
| null |
PMC7481268_01
|
Female
| 14
|
A 14-year-old female previously diagnosed with HGPS was the firstborn child of non-related, healthy parents, with no previous family genetic disorders and a healthy 9-year-old sibling. The mother was aged 20 years during conception, and the father was aged 26 years. Normal weight and height data were recorded throughout pregnancy. According to the parents, the patient appeared to be a healthy newborn, and the patient's development and growth was normal until her second year of age.
|
hgps, genetic assessment, laminopathy, premature aging, progeria, treatment
| Not supported with pagination yet
| null |
PMC5611446_01
|
Male
| 34
|
Physiologic data:RR interval data of twenty healthy "Young" (21-34 years old) and twenty healthy "Elderly" (68-85 years old) subjects were obtained from the Fantasia module of the PhysioNet database (Goldberger et al.,). Each data corresponds to a 120 min recording of the subject's electrocardiogram (ECG) when in continuous supine resting, sampled at a frequency of 250 Hz. Each group of subjects has an equal number of men and women. Each RR interval is computed by an automated algorithm from annotated heartbeats of subjects (Iyengar et al.,). After extraction of RR series of all subjects from the database, each signal segment was selected from the beginning by varying length from 50 to 1,000 beats (total 8 different lengths:50, 100, 200, 300, 400, 500, 750, and 1,000 beats corresponding to average time durations of 0.78, 1.60, 3.23, 4.88, 6.52, 8.17, 12.30, and 16.44 min, respectively) for each subject.
RR interval time-series of "Arrhythmia" and "Healthy" subjects were obtained from the MIT-BIH module of the PhysioNet database (Goldberger et al.,). The Arrhythmia Database contains 48 ECG recordings obtained from 47 subjects (Moody and Mark,). The subjects included 25 men aged 32 to 89 years and 22 women aged 23 to 89 years. The recordings were digitized at 360 samples per second per channel with 11-bit resolution over a 10 mV range. Each beat of every record was then annotated independently using a slope sensitive QRS detector (Moody and Mark,). From this, the RR interval was then computed for each subject. The Normal sinus rhythm database contains 18 long-term ECG recordings of subjects who were found to have no significant arrhythmia; they include 5 men, aged 26 to 45, and 13 women, aged 20 to 50. After extraction of RR series of all subjects from the database, each signal segment was selected from the beginning by varying length from 50 to 1,000 beats (total 8 different lengths as mentioned in previous paragraph, which corresponds to average time durations of 0.69, 1.34, 2.67, 3.98, 5.23, 6.16, 9.89, and 13.24 min, respectively) for each subject.
In this study, we compared the characteristics and performance of DistEn as a entropy measure with ApEn and SampEn. The reason that ApEn and SampEn were used for comparison was because DistEn was initially proposed to address the dependence of the existing ApEn and SampEn methods on tolerance r.
Form (N - m + 1) vectors of length m each, given by
Take each vector of step 1 as a template vector and find its distance from every vector of , where the distance is given by
Then we define where, is the probability of a vector to lie within a distance r of the vector
The above steps are repeated for m+1, resulting in Phim+1(r) from which ApEn is defined as
ApEn is an approximation of the conditional probability (Pincus,; Pincus and Goldberger,) of two segments matching at a length of m+1 if they match at m. The embedding dimension m is the length of compared segments of the input time series and r is the threshold of distance, which is fixed to match segments when they are compared with each other. Let a time series of length N be defined as {x(n) : 1 <= n <= N}. For a given value of the embedding dimension m and tolerance r, ApEn is calculated using following steps:
In this study, we used m = 2, 3, 4, 5 and r = 0.1*SD to 1*SD with a step size of 0.1 * SD to calculate ApEn for all signals, where SD denotes standard deviation of the signal. For ease of calculation and visualization, each RR time-series was normalized to unitary variance before calculating ApEn.
SampEn is a modified version of ApEn to find the irregularity of a given signal (Richman and Moorman,). Here, self matches between vectors are avoided from the calculation and the same number of template vectors are used in m and m+1 dimensions. For a given time series data of length N, sample entropy is calculated as
where
being the probability of a vector to lie within a distance r of the vector ,1 <= j <= (N - m), j i.
Similar to ApEn, we used m = 2, 3, 4, 5 and r = 0.1 * SD to 1 * SD with a step size of 0.1 * SD to calculate SampEn for all signals, where SD denotes standard deviation of the signal. For ease of calculation and visualization, each RR time-series was normalized to unitary variance before calculating SampEn.
DistEn is initially developed from SampEn with an aim of improving the inconsistency and minimizing the dependence on input parameters. The novelty behind DistEn is the assumption that the inconsistency and parameter-dependence of SampEn-based measures come from the incomplete assessment of the distribution of inter-vector distances, and that they can be eliminated by taking full advantages of the distribution property (Li et al.,). By quantifying the Shannon entropy of the probability density of inter-vector distances:an assessment that completely and globally quantifies the distribution property, the so-developed DistEn displayed improved performance as we expected (Li et al.,).
For a given time series data of length N, embedding dimension m and bin number M the distribution entropy is calculated as follows.
Form (N - m) vectors of length m each, given by
Take each vector of step 1 as a template vector and find its distance from vector , where the distance is given by
A distance matrix D of size (N - m) * (N - m - 1) is formed by repeating this calculation for all ith template vectors, where 1 <= i <= (N - m).
The elements of distance matrix D are now divided into M number of equally spaced bins and the corresponding histogram is obtained.
Now, at each bin t of the histogram, its probability is estimated as ; 1 <= t <= M.
By the definition of Shannon entropy, the normalized DistEn of a given time series x(i), is defined by the expression where pt is the probability of each bin in the histogram.
In this study, we used m = 2, 3, 4, 5 and M = 50, 100, 200, 300, 400, 500, 750, 1000, 1500, 2000 to calculate DistEn for all signals.
In our study, we used area under the ROC curve (AUC) to test the efficiency of DistEn as a feature to distinguish signals of different levels of complexity (synthetic) and RR time series belonging to different classes (physiologic). The AUC is the probability that a classifier ranks a randomly chosen instance X higher than a randomly chosen instance Y, X and Y being samples taken from two independent populations. An AUC value of 0.5 indicates that the distributions of the features are similar in the two groups with no discriminatory power. Conversely, an AUC value of 1.0 means that the distribution of the features of the two groups do not overlap at all. The AUC value was approximated numerically using the trapezoidal rules (Hanley and McNeil,) where the larger the AUC value, the better the discriminatory performance. MATLAB R2014b Statistics toolbox was used to perform all statistical operations.
|
aging, approximate entropy, arrhythmia, distribution entropy, heart rate variability, sample entropy, short-term analysis
| Not supported with pagination yet
| null |
PMC5369860_01
|
Male
| 46
|
A 46-year-old male, with no significant medical or surgical history, was ambulanced to the emergency department in King Abdulaziz Medical City (tertiary care center) in Riyadh, Saudi Arabia, as a result of a motor vehicle accident. The patient had a main complaint of a right first MTP joint that is mildly swollen and painful. Upon examining the big toe, swelling and deformity were observed accompanied by a painful range of motion. Also, the patient was unable to plantarflex or dorsiflex the right hallux. The right big toe showed dorsal displacement with shortening of the first ray. The vascular and neurological examination findings were normal; and the skin was intact.
A review of the radiographic images showed dorsal dislocation of the first MTP joint of the right big toe (Fig. 1). In addition, the fibular and tibial sesamoids were displaced dorsolaterally. At the emergency department, multiple attempts at closed reduction of the dislocation were unsuccessful. With the failure of the reduction to be obtained and because of the coronavirus outbreak at the hospital during that time, so the patient was referred to another hospital to take care of him. After six months, the patient was referred to the orthopedic department from the family medicine clinic since he neglected his injury. His x-ray at that time showed diffused regional osteopenia of the dislocated joint. Also, flattening of the head of the 2nd metatarsal bone was noted (Fig. 2).
Six months following the injury, another attempt at closed reduction in the operation room while the patient was under general anesthesia (with ankle block) was unsuccessful, after which an open reduction was carried out. In order to gain access to the dislocated first MTP joint, a dorsomedial approach was used (Fig. 3). The lateral and medial sesamophalangeal ligaments were torn or pulled off forcibly from their distal attachment to the proximal phalanx base upon further investigation of the sesamoid complex. Then the sesamoids retracted proximally. No fracture of the two sesamoids was observed. After the reduction was achieved, the level of stability and the movement range were verified and checked to be was expected. The first ray length seemed to be regained and back to its normal function. Fixation of the MTP joint was carried out with the help of provisional Kirschner wire (k-wire) for more stability (Fig. 4).
Then after eight weeks, the wire was taken off, and weight-bearing was gradually allowed for the patient. After three months of the operation, the patient gained a full normal activity with no degrees of pain or forms of disability. The patient did not suffer from range of motion restriction (plantarflexion and dorsiflexion). After twelve months of the operation, persistent disuse osteopenia was observed by the X-ray. However, other complications were neither observed nor reported by the patient either (Fig. 5).
|
case report, delayed, dislocation, first metatarsophalangeal joint, surgery
| Not supported with pagination yet
| null |
PMC9850118_01
|
Female
| 37
|
A 37-year-old lady (para 2 living 2) reported to have last normal menses period 2 years ago. Presented with per vaginal discharge for 2 years which was copious, yellowish, and non-itchy, which made her use one pad each day. She reports to have an intra-uterine device inserted 2 years ago which was later removed. She denies any history of lower abdominal pain, postcoital bleeding or cough, fever, loss of weight and night sweats. During her illness, she has been treated with variety of antibiotics and antifungal medications without improvement. She was otherwise well and with no significant medical history. She has no known drug or food allergy. There is no any family history of gynecological or other malignancies that was reported. She has delivered two times through cesarean section, and there is no history of other gynecological procedures reported. She is non-smoker and does not consume any alcohol. During childhood, she received Bacillus Calmette-Guerin (BCG) vaccination.
On examination, she was alert, not pale, no lower limb edema, and had stable vital signs. The abdominal examination revealed normal contour, a pfannenstiel scar, soft and non-tender. There was no mass palpable, and uterus was not bulky. The rest of the systemic examination was normal.
Speculum examination was unremarkable except with blood oozing from the cervix. Bimanual examination was unremarkable, endocervical swab was done which was negative on gram stain and no organism growth on culture media.
Pap smear was not performed due to vaginal bleeding; however, a pap smear done 1 year ago revealed inflammatory cells.
Transvaginal ultrasound was performed which showed an anteverted uterus with a grossly abnormal endometrial echo pattern. The endometrium was heterogenous and had an irregular surface (Figure 1).
After the history and physical examination, we had a differential diagnosis of endometritis; the patient was then scheduled for diagnostic hysteroscopy. Preoperative investigations included a hemoglobin level of 11.4 g/dL, white blood cell 5.61/L, platelets 181/L, and CA-125 (Serum) 41.78 U/mL (0-35). The patient's hepatitis and HIV status were negative. A chest X-ray showed no evidence of pulmonary TB.
Intra-operative findings revealed a stenosed cervical canal and uterine synechiae was present. Release of uterine synechiae and endometrial biopsy via curetting was done. Blood loss was not excessive and the patient was discharged home the same day. Histopathology of the curettings showed numerous caseating and non-caseating granulomas with plasma cells, Ziehl-Neelsen stain for acid fast bacilli was positive (Figure 2).
Based on the history and results of endometrial biopsy, our provisional diagnosis was endometrial TB. The department of internal medicine was consulted and initiated the patient on one combined tab daily, consisting isoniazid, rifampicin, ethambutol, and pyrazinamide for 2 months (Intensive phase), then tab (isoniazid, rifampicin) for 4 months (Continuation phase).
Upon follow-up, she reports bleeding to have ended in the fourth week of her treatment. She has been doing well and disease-free after completion of the treatment.
|
tuberculosis endometritis, female genital tuberculosis, menstrual irregularity
| Not supported with pagination yet
| null |
PMC9244929_03
|
Male
| 0
|
A 6-month-old boy complained of fever and no other symptoms. Four days later, the fever persisted, then the patient had 1 episode of watery stool and 1 episode of generalized tonic-clonic seizure which lasted for 10 minutes. The patient was awake after the seizure ceased, and he was admitted to a district hospital later. On the first day of hospitalization, the patient experienced seizures 6 times and decreased level of consciousness with a pediatric coma scale (PCS) of 5. Physical examination revealed that the patient had fever of 38.5 C, stiff neck, and anisocoria; he was transferred to the Pediatric Intensive Care Unit (PICU) of the same hospital subsequently. Head computed tomography (CT) scan demonstrated signs of meningoencephalitis. Patient was eventually referred to the PICU of our hospital because of status epilepticus.
Upon arrival to the PICU, the patient was still in a coma with PCS of 5; the physical findings were persistent fever, anisocoria, sluggish pupillary light reflex, positive Babinski reflex, positive clonus, and positive Brudzinski sign. The initial hemogram was: Hb 11.1 g/dL; leukocyte count 8620/muL (69.8% neutrophils; 27.0% lymphocytes; 3.1% monocytes); platelet count 125 000/mm3. There was an increase in C-reactive protein (CRP) of 139 mg/L. A cerebrospinal fluid (CSF) analysis revealed: red and turbid in appearance, proteins 1.59 g/dL; glucose 1 mg/dL (serum glucose 117 mg/dL); blood cells 1120/muL (64% polymorphonuclear and 36% mononuclear); erythrocytes 7300/muL; lactate dehydrogenase (LDH) 2659 U/L; positive Nonne and Pandy tests; which was typical for bacterial meningitis. The patient was administered 100 mg/kgBW/day of ceftriaxone, paracetamol, mannitol, dexamethasone, and maintenance dose of phenytoin.
On the fifth day of hospitalization, the patient experienced Cushing triad. An evaluation head CT scan demonstrated signs of obstructive hydrocephalus and diffuse cerebral edema, a neurosurgery consult was made and ventricular tapping were performed. Interferon gamma release assay (IGRA) blood test of CSF was performed for any Tuberculosis suspicious, and result test was intermediate. Considering the clinical deterioration and the indeterminate IGRA test result, the tuberculous meningoencephalitis regimen protocol was then initiated.
CSF culture showed moderate number of gram-positive cocci in chains, consistent with S. pneumoniae. The isolate was susceptible to the following 10 antibiotics: ampicillin, penicillin G, clindamycin, ampicillin-sulbactam, ciprofloxacin, ertapenem, imipenem, meropenem, vancomycin, moxifloxacin; ceftriaxone was not tested due to lack of reagent. The bacteria were resistant to: azithromycin, oxacillin, amikacin, cefoxitin, doxycycline, tetracycline, trimethoprim/sulfamethoxazole, chloramphenicol. After 7 days of ceftriaxone administration, the antibiotic regimen was switched into 400 mg ampicillin/kgBW/day IV devided q6h of ampicillin-sulbactam according to the antibiogram. Three days following the administration of ampicillin-sulbactam, the fever significantly increased, then antibiotic was escalating to 40 mg/kgBW IV q8h. The patient experienced recurrent seizures and decerebration. Unfortunately, the patient died due to cardiorespiratory failure on day 17th of hospitalization (Table 1).
|
streptococcus pneumoniae, case series, developing countries, invasive pneumococcal disease, pediatric
| Not supported with pagination yet
| null |
PMC8472319_01
|
Male
| 49
|
A 49-year-old married Indian man presented to the Emergency Department of Hamad General Hospital in Qatar, with fever, headache, lower limb weakness, and urinary incontinence for 1 month. His complaints were also associated with fluctuating episodes of confusion and agitation for 4 days. He had not experienced vomiting, convulsions, loss of consciousness, diplopia, facial weakness, or dysphagia. He had no history of trauma; fall; cardiac, respiratory, genitourinary, gastrointestinal, or musculoskeletal abnormality; or drug intake or intoxication. Review of his past medical documents revealed visits for evaluation of cervical lymphadenitis with inconclusive results. On assessment of his living conditions and housing, the patient stated that he lives in a crowded house with multiple roommates, of which one had prolonged cough without professional diagnosis.
On examination, the patient looked pale, had stable vital signs, agitated, confused, had neglected appearance with poor hygiene and having an odour smelling like urine. On neurological examination, he was conscious, alert, but not oriented, with a Glasgow coma scale of 12/15. Signs of meningeal irritation were negative. His motor examination showed decreased strength in both lower limbs (4/5) as well as diminished reflexes. His cranial nerves, sensory, and cerebellar examination were equivocal. Other systemic findings were normal.
His 1-month history of headache, fluctuating hallucinations, and confusion led us to consider the possibility of CNS infection. All basic laboratory tests were performed, including complete blood count, urea and electrolytes, blood culture and sensitivity, and C-reactive protein (CRP). Lumbar puncture was performed under conscious sedation. Initial investigations showed a minimal increase in leucocyte count and CRP, but he had normal renal and liver functions. Chest X-ray imaging did not show any abnormality, and computed tomography finding of the head was normal. High lymphocytes, high protein, and low glucose were detected in the cerebrospinal fluid (CSF).
The patient was admitted under the care of the medical team and was empirically started on anti-TB medications and antibiotics. MRI, acid-fast bacilli, and QuantiFERON gamma assay were performed. MRI of the head and spine (Figures 1-3) showed extensive basal intracranial meningeal thickening and enhancement extending along the spinal canal circumscribing the whole spinal cord down to the conus medullaris region (Figures 2, 3). Intramedullary high signal intensity was observed along the cervical spinal cord from C3 down to the C7 vertebral level and the lower thoracic spinal cord opposite the tbl10-11 and tbl12 vertebral level, yet no abnormal post-contrast enhancement was detected (2). This picture was highly suggestive of extensive craniospinal TB meningitis.
The patient showed dramatic improvements in his orientation and conscious level after 7 days of receiving anti-TB management and was discharged from the acute medical ward to the rehabilitation center to address his lower limb weakness.
|
craniospinal tuberculosis, uncommon presentation
| Not supported with pagination yet
| null |
PMC3843933_01
|
Male
| 54
|
The proband was a 54-year-old French male patient with a personal history of dyslipidaemia and tuberculosis in childhood. From the age of 41 years, he slowly developed progressive weakness and atrophy of proximal upper-limb muscles and later (at 46 years of age) involvement of the lower limbs, associated with axial weakness. At that time, the walking test was unlimited, whereas at 50 years of age, he was only able to walk 50 m using a cane for support.
Then, the proband developed acute behavioural changes including irritability, severe anxiety and major depression, which led to him being hospitalised in a psychiatric hospital at 53 years of age. He developed gradual cognitive decline characterised by impairment of executive functions. The deterioration of upper- and lower-limb weakness and cognitive functions accelerated. The patient was eventually unable to walk and was confined to a wheelchair. His cognitive dysfunctions consisted of impaired frontal lobe functions, dominated by major behavioural changes (such as changes in affects, decline in social interpersonal conduct and perseveration) and aphasia (phonemic and semantic paraphasia). His score on the Frontotemporal Behavioural Scale was abnormal (3 items out of 4 were pathological). However, his memory was not impaired: the Mini-Mental State Examination (MMSE) was normal (29 out of 30 points).
An electromyography (EMG) (fig. 1) showed fibrillation potentials and positive sharp waves at rest, and myopathic or neurogenic features according to the muscles examined: polyphasic small-amplitude short-duration motor unit potentials and polyphasic long-duration with slow-frequency motor unit potentials were found together in the EMG study. These results suggested inclusion body myopathy, which was confirmed by a muscle biopsy revealing histopathological changes consisting of rimmed vacuoles. Plasma creatine kinase levels were mildly increased (262 IU/l, normal values 20-220 IU/l). Nerve conduction studies were normal.
Cerebral magnetic resonance imaging (MRI) was performed at 54 years of age and revealed frontal and internal temporal atrophy (fig. 2). Fluorodeoxyglucose (FDG) positron emission tomography (PET) scan showed severe bilateral hypometabolism, involving especially the frontal and temporal lobes (fig. 3). The occipital lobe, basal ganglia and cerebellar metabolism were normal. Behavioural-type frontotemporal dementia was therefore diagnosed based on the clinical frontal lobe dysfunction and the results of the cerebral MRI and PET scan.
|
frontotemporal dementia, inclusion body myopathy, psychiatric disorders, vcp mutation
| Not supported with pagination yet
| null |
PMC4722727_01
|
Female
| 77
|
A 77-year-old Caucasian woman was admitted to Panevezys regional hospital in Lithuania because of a 1-day history of skin and scleral jaundice and weakness. Her medical history included a laparoscopic cholecystectomy 15 years earlier for a gallbladder stone.
A clinical examination revealed only skin and scleral jaundice. The patient's laboratory test results were as follows: total bilirubin (TBIL) 338.59 U/L (normal <21 U/L), direct (conjugated) bilirubin (DBIL) 294 U/L (normal <5.3), alkaline phosphatase (ALP) 720.1 U/L (normal 40-129), alanine transaminase (ALT) 52.0 U/L (<=35), aspartate transaminase (AST) 142.1 U/L (normal <=35), gamma-glutamyl transpeptidase (GGT) 353.0 U/L (normal 9-40), and carbohydrate antigen 19-9 157.09 U/ml (normal 0-37). On the basis of ultrasound and computed tomography (CT) findings, cancer in the head of the pancreas with invasion to the common bile duct (CBD) was suspected. To alleviate jaundice and to perform brush cytology, percutaneous transhepatic bile duct drainage was performed. After this procedure, decreases in TBIL (187.92 U/L), DBIL (95.69 U/L), ALP (276.1 U/L), ALT (37.2 U/L), AST (73.0 U/L), and GGT (123.8 U/L) were noted.
A bile duct villous papilloma was suspected on the basis of brush cytology. Despite the results of these investigations, a chance of malignancy remained. The patient was referred to the local tertiary treatment center for further treatment. At the Vilnius University Hospital Santariskiu Klinikos, a multidisciplinary team reviewed a CT scan of the patient. The scan showed the presence of a CBD-obstructing, homogeneous, 5 x 3-cm mass below the fusion of extrahepatic ducts without any evidence of tumor metastasis in a common bile duct surrounding tissue, the liver, right and left intrahepatic ducts, and the pancreas. The patient's periportal lymph nodes were of normal size. On the basis of the tumor's radiological features, a preoperative diagnosis of extrahepatic bile duct malignant tumor was made (T1N0M0, type I, by Bismuth-Corlette classification) (Fig. 1).
The multidisciplinary team assessed the absence of tumor invasion in surrounding tissues and decided to perform a surgical resection of the tumor. However, there remained a possibility to perform a more radical operation, such as pancreaticoduodenectomy, if the cancer was observed to involve the distal part of the CBD.
A laparotomy was performed. The duodenum and the head of the pancreas were mobilized by using the Kocher maneuver. Importantly, no trace of tumor was found in the proximal common hepatic duct (CHD) and distal CBD parts. Only bile duct parts containing tumor were resected. To perform a Roux-en-Y hepaticojejunostomy, a small intestine loop was mobilized and divided 50 cm from the superior duodenal fold. The distal part was closed and anastomosed end to side to the remaining 0.5-cm length of the CHD. Sixty centimeters below this junction, an end-to-side jejunojejunal anastomosis was formed.
Macroscopic evaluation of the specimen revealed that the resected parts of the CBD and CHD consisted of dilated bile ducts with a papillary tumor protruding into the lumen. The tumor's size was 4.5 x 4 x 2.5 cm. The resection margin was 2 cm from one side and 2.5 cm from the other, and two investigated lymph nodes were identified as tumor-free (Fig. 2).
Histologically, the tumor consisted of branching papillary structures lined by tall columnar epithelium with slight nuclear atypia and scant fibrous stroma (Fig. 3a, b). Invasive growth was not detected. The adenoma was tested for microsatellite instability using immunostaining of MLH1, PMS2, MSH2, and MSH6 proteins. Positive nuclear immunostaining was observed in tumor cells (Fig. 3c), which showed no evidence of microsatellite instability.
The patient's postoperative course was uneventful. Her hospitalization period was 10 days. At two visits during the 18-month follow-up period, she had no complications or recurrence.
| null | Not supported with pagination yet
| null |
PMC6859392_01
|
Male
| 54
|
A 54-year-old Japanese man started to take oren-gedoku-to, a type of JHM, because it had been prescribed by a local clinic on Day 1 for the hot flashes he had been experiencing for 2 years. He developed a high fever and chills after taking oren-gedoku-to for 17 days. After developing dyspnea mainly when exercising, he decided to stop taking the medication on Day 17. A few weeks later he was admitted to his local clinic with symptoms of dyspnea on exercise, malaise and chills. When admitted to our hospital on Day 21, he was afebrile, and slight fine crackles were heard in his lower back.
Chest X-ray showed interstitial shadows in both lower lung fields, and computed tomography (CT) showed diffuse patchy and funicular opacities and volume loss predominantly in both lower lung areas (Fig. 1). Peripheral leukocyte and eosinophil counts were 6,500 cells/muL and 436 cells/muL, respectively, and the serum C-reactive protein level was 2.31 mg/dL. The hepatic transaminase levels (AST 41 U/L, ALT 39 U/L) were mildly elevated with slight elevation of the lactate dehydrogenase level (267 U/L), and KL-6 was within the normal ranges but SP-D was elevated (Table 1). The SpO2 was low at around 85% after minor exertion. Restrictive impairment and decreased single-breath diffusing capacity for carbon monoxide were demonstrated by pulmonary function tests. Lymphocytes and eosinophils were dominant (73.6% and 12.6% respectively), and the CD4/CD8+T-cell ratio was low in the bronchoalveolar lavage fluid (BALF) obtained on Day 25 (Table 1). Many alveolar macrophages existed, and mild lymphocytic interstitial inflammation was seen in the transbronchial lung biopsy (TBLB) specimens taken from the right middle and lower lungs (Fig. 2).
Although antibiotics and steroids were not administered, malaise, cough, hypoxemia and interstitial shadows gradually decreased up to Day 33. The patient was discharged from hospital on Day 43; the total hospitalization duration was 23 days (Fig. 3). We suspected that the patient had developed hypersensitivity pneumonitis from the Japanese summer, but he had no remarkable inhalation history, and there were no reports of deterioration in his housing conditions. After the patient stopped taking oren-gedoku-to, his fever and respiratory symptoms did not recur. Because lymphocytes obtained from the peripheral blood were positive in the DLST for oren-gedoku-to, we diagnosed him as having oren-gedoku-to-induced pneumonitis.
The JHM oren-gedoku-to, which is a mixture of four crude drugs-oren, ougon, oubaku and sanshishi-is often prescribed for hot flashes. We therefore next performed a DLST for each component, and only ougon was positive, resulting in a final diagnosis of ougon-induced pneumonitis (Table 2). His symptoms as well as radiograph findings and oxygenation recovered almost completely, and his peripheral eosinophil count returned to normal. Pulmonary function test findings also returned to normal, and his single-breath diffusing capacity for carbon monoxide recovered by Day 56 without using steroids or other anti-inflammatory drugs (Fig. 3).
|
ougon, drug-induced pneumonitis, lymphocyte stimulation test, oren-gedoku-to
| Not supported with pagination yet
| null |
PMC3791626_01
|
Female
| 31
|
A 31-year-old lady came to us with evidence of spontaneous abortion at 14 weeks of her pregnancy, which was conceived following in vitro fertilization. An ultrasound scan done showed an empty uterine cavity, indicating a complete abortion. She had fever at the time, and hence a course of antibiotics was given. Her hemoglobin levels were low, for which she was given a unit of packed red blood cells.
She was a booked case with us and had a past history of two episodes of ascites (OHSS) following the embryo transfer. The first episode was within 12 days of embryo transfer, and the second episode was at 9-10 weeks of gestation. Both episodes were diagnosed as OHSS and treated symptomatically with albumin infusion. At 14 weeks of gestation, she had fever and recurrence of ascites. Ascites did not subside even with albumin and Cabergoline; hence other causes of ascites were evaluated by Mantoux test and chest X-ray, which were negative for tuberculosis.
Her bleeding per vaginum persisted, for which a scan was done again which showed some retained products of conception for which she underwent dilatation and evacuation. The tissue obtained was sent for histopathology examination and came back as only degenerated products of conception and negative for mycobacterium tuberculosis by PCR.
When the ascites did not disappear after the regular treatment, the ascitic fluid was tapped thrice. It was green in color, leading to suspicion of presence of bile salts or pigments in it though her liver function tests were normal. When analyzed for the same, there were no bile salts or pigments found. Upon culturing it, no growth was found after 7 days. The ascitic fluid was negative for malignancy, and Ca 125 was normal. Her hematocrit persisted at the same values (19.8%) as before. She had lost significant weight within two weeks. The ascitic fluid was further investigated with acid fast staining, which showed no acid fast bacilli. A PCR sent for mycobacterium tuberculosis came back negative. Further evaluation showed its Adenosine DeAminase (ADA) level was 78 IU/L and rose to 110 IU/L in 3 days (normal: <39 IU/L). Based on the ADA levels, she was started on antitubercular treatment with HRZE (Isoniazid + Rifampicin + Pyrazinamide + Ethambutol), which finally resolved the ascites within a week.
| null | Not supported with pagination yet
| null |
PMC7492961_01
|
Male
| 8
|
An 8-year-old male patient was sent for management after 3 days of an enamel-dentine-pulp fracture in the upper left central incisor (number 21) and crown-root fracture with pulp exposure in the upper right central incisor (number 11) (Figures 1-4). Trauma occurred during a football match. The patient denied spontaneous pain on presentation. Through intraoral examination, pulp exposure was diagnosed in both incisors, as well as mild pain to percussion, absence of pain to palpation, absence of periodontal pockets higher than 3 mm, mobility class I, and positive response to cold test in both incisors that were performed on the labial surface. The size of the pulp lesion was registered (nearly 2 mm in tooth 21 and 4 mm in tooth 11) with proliferated pulp reaction in tooth 11. Radiographic examination showed no root fractures or periradicular radiolucency.
The pulpotomy procedure was explained to the parents and the patient who agreed to comply with it. The concerned area was anesthetized with local infiltration of 1.7 ml lidocaine HCL 2% with 1 : 100000 epinephrine. After local anesthesia, the palatine fragment was removed from tooth 11. After this, nearly 2 mm depth of visible pulp tissue and adjacent dentin of both incisors (11 and 21) were removed, with a sterile diamond cylindrical drill on a high-speed handpiece with water irrigation. A hole about 2 mm deep remained, enough to hold the wound dressing and sealing material. Bleeding was controlled with water squirted out of a syringe, and MTA (Pro Root, Dentsply, Tulsa Dental, OK, USA) was applied with a spatula-shaped hand instrument, followed by wet cotton pellets used to adjust it onto the visible pulp space. A layer of MTA 2 mm thick was positioned over the exposed pulp tissue. After 10 minutes, a glass ionomer (Vitrabond Plus, 3M ESPE, USA) was applied over the MTA, and the patient was dismissed (Figure 5). After 2 weeks, the patient was asymptomatic, with no pain to palpation, absence of periodontal pockets higher than 3 mm, mobility class I, and positive response to the cold test. The glass-ionomer restoration was partially removed, and directly bonded composite restoration was performed (IPS Empress Direct, IvoclarVivadent, Schaan, Liechtenstein) with gum retraction (Ultrapak 1, Ultradent, Utah, USA) (Figure 6).
Teeth were followed clinically and radiographically two weeks after treatment and subsequently during the first year at three months, and afterwards, at six-month intervals. During the 6-month follow-up and from 1 to 5 years, the situation remained unchanged with no evidence of radiolucent lesions in radiographic examinations (Figure 7). Cone-beam computerized tomography (CBCT) was used to monitor healing during the 5-year follow-up, allowing 3D visualization of the periapical area and discarding the existence of root resorption (Figure 8).
Follow-up shows a continuation of root development and the creation of hard tissue at the site of the incision (Figure 7). Light discoloration in the crown of 21 was evident at 3 years follow-up (Figure 9), but it did not disturb the patient or his family, so it was not addressed.
| null | Not supported with pagination yet
| null |
PMC7417062_01
|
Female
| 70
|
A 70-year-old woman presented to the Emergency Department (ED) of our hospital with gradually worsening anorexia, and dyspnoea.
She was in her usual state up to 2 months prior when she developed dyspnoea and anorexia, leading to her first visit to the ED and hospitalization. During the initial evaluation, chest computed tomography (CT) was performed, which showed a large left pleural effusion and minimal right-sided pleural effusion, with no parenchymal abnormalities.
Thoracocentesis yielded a pleural exudate with a high leucocyte count (15.790/mcl) with 67.5% monocytes, and markedly elevated adenosine deaminase (ADA; 269.4 U/l) and LDH (3.590 IU/l) levels.
Pleural biopsy reported a non-specific inflammation of the pleural tissue without malignancy, granulomas or acid-alcohol resistant bacilli. Pleural fluid cultures were negative.
A presumptive diagnosis of pleural TB was made, based on elevated ADA levels, and empirical therapy was started with isoniazid, rifampicin, ethambutol and pyrazinamide. The patient reported an improvement in her general wellbeing and was discharged.
Over the following 2 months, the patient reported recurring progressing asthenia and anorexia. On the day of readmission, sudden-onset dyspnoea and general malaise developed, motivating a new admission to the ED.
Chest radiography showed an enlargement of the cardiac silhouette and a large-volume right pleural effusion. CT further showed a small left pleural effusion. Both CT and echocardiography showed a circumferential pericardial effusion with no further sonographic signs of tamponade risk. No other lesions besides pleural and pericardial effusions were found on the CT scan.
Blood tests yielded a normal haemogram, with elevated C-reactive protein (88.7 mg/dl) and LDH (335 IU/l) levels with no additional remarkable findings.
Pleural effusion analysis showed an elevated leucocyte count, ADA levels of 203.5 U/l and LDH elevation (1.535 IU/l).
Intravenous methylprednisolone was started at admission due to the pericardial effusion, resulting in rapid improvement of both the pericardial and pleural effusions.
Despite improvement, a small pleural effusion remained visible on chest radiography and a new thoracocentesis was performed during the hospitalization. This time, flow cytometry analysis was requested that identified the presence of anomalous lymphocytes, the immunophenotypic characteristics of which supported the diagnosis of diffuse large B cell lymphoma (DLBCL).
Empirical TB therapy was thus stopped, and the patient was referred to the Haematology department for treatment.
Due to the loss of muscle mass and autonomy, the patient was discharged to a rehabilitation facility to improve functional status before any treatment options were considered.
|
adenosine deaminase, lymphoma, pleural effusion, tuberculosis
| Not supported with pagination yet
| null |
PMC10248407_01
|
Male
| 49
|
We described the case of a 49-year-old man with a 1-year history of intermittent cough and sputum production who had worsened over the last 4 days with fever and right chest pain. The patient recently had a history of unexplained weight loss of 5 kg. He has smoked 20 cigarettes per day since the age of 19. The patient had periodontitis and tooth loss. The patient had no history of travel or tuberculosis exposure and no food or drug allergies.
On admission, his temperature was 37.9 C, pulse rate 81 beats/min, respiratory rate 22 breaths/min, blood pressure 132/80 mmHg, and oxygen saturation 95% on room air.
Pulmonary percussion found dullness in the right lower lung, and pulmonary auscultation revealed decreased breath sounds in the right lower lung. The remaining physical examination was normal.
The laboratory findings were as follows: white blood cell (WBC) count 22.98 x 109/L with 85.5% segmented neutrophils, 7.6% lymphocytes, neutrophil count 19.9 x 109/L, and lymphocyte count 1.76 x 109/L, and his ultrasensitive C-reactive protein (hs-CRP) was 248.56 mg/L (normal range 0-5 mg/L). Serum tumor marker screening showed a slightly elevated neuron-specific enolase (NSE) of 17.05 ng/ml (normal range <16.3 ng/ml). The squamous cell carcinoma antigen (SCCA), cytokeratin-19-fragment (CYFRA21-1) and carcinoembryonic antigen (CEA) levels were normal. The other laboratory test results were normal. Chest computed tomography (CT) revealed an irregular mass in the right middle lobe of the lung near the interlobar fissure with right atelectasis and thickening of the right pleura with encapsulated effusion and pneumatosis (Figure 1A). Thoracentesis and catheter drainage were performed immediately after chest ultrasound confirmed pleural effusion. Analysis of the drained purulent fluid suggested an exudative process [fluid protein 54.9 g/L, serum protein 69.8 g/L (normal: 65-85 g/L), fluid lactate dehydrogenase 3,014 U/L, serum lactate dehydrogenase 220 U/L (normal 120.0-250.0 U/L)]. Other pleural fluid analysis showed WBC 3,800/muL with 80% neutrophils, fluid glucose 0 mmol/L, and fluid adenosine deaminase 81.8 U/L. Pleural fluid smear, Gram staining, bacterial culture, acid-fast bacilli culture and smear, and cytology were all negative. A blood culture was also negative. Empyema was diagnosed. He received intravenous imipenem/cilastatin 1.0 g once every 8 h. After repeated pleural effusion drainage and anti-infective treatment for 3 weeks, re-examination of lung CT showed no significant change in the amount of pleural effusion (Figure 1B).
The patient requested to be discharged after his fever improved. Half a month after discharge, he was admitted to our hospital due to progressive worsening of cough and shortness of breath. On April 1, a contrast-enhanced CT scan showed uneven enhancement of the mass in the lateral segment of the right middle lobe, mediastinal lymphadenopathy with visible enhancement, and a right hydropneumothorax (Figure 1C). In addition, the laboratory test results included WBC 11.56 x 109/L, neutrophil ratio 71.84%, and CRP 219.35 mg/L. Ultrasound re-examination showed that there was a localized anechoic area of 5.3 cm x 7.7 cm x 3.4 cm in the right thoracic cavity, and he underwent pleural puncture and drainage again. The pleural drainage fluid was sent for NGS. The NGS results only detected Fusobacterium nucleatum. Subsequently, the patient underwent bronchoscopy. Fiberoptic bronchoscopy showed complete airway obstruction due to whitish endobronchial membranous lesions in the lateral segment of the right middle lobe (Figure 2A). A cauliflower-like mass was seen and biopsied after clamping the whitish endobronchial membranous lesions (Figure 2B). Histological examination of the biopsy specimens demonstrated a highly differentiated squamous cell carcinoma in the right lung (Figure 2C). He was finally diagnosed with empyema caused by Fusobacterium nucleatum and squamous cell carcinoma of the lung. The patient's cough and shortness of breath were improved after draining 1,800 ml pleural effusion for 3 days, and cefoperazone sodium/sulbactam sodium 3 g was given intravenously twice a day for 2 weeks. Repeat chest CT revealed absorption of the fluid and gas in the right pleural cavity (Figure 1D). Meanwhile, blood biochemical examination showed that infection indexes, such as white blood cells and CRP, had returned to normal. Regrettably, the patient refused antitumor therapy.
|
fusobacterium nucleatum, empyema, lung cancer, next-generation sequencing, squamous cell carcinoma
|
CT scan images. (A) Chest CT showed an irregular mass at the right middle lobe of the lung near the interlobar fissure with right atelectasis and encapsulated effusion and pneumatosis.
|
|
PMC10248407_01
|
Male
| 49
|
We described the case of a 49-year-old man with a 1-year history of intermittent cough and sputum production who had worsened over the last 4 days with fever and right chest pain. The patient recently had a history of unexplained weight loss of 5 kg. He has smoked 20 cigarettes per day since the age of 19. The patient had periodontitis and tooth loss. The patient had no history of travel or tuberculosis exposure and no food or drug allergies.
On admission, his temperature was 37.9 C, pulse rate 81 beats/min, respiratory rate 22 breaths/min, blood pressure 132/80 mmHg, and oxygen saturation 95% on room air.
Pulmonary percussion found dullness in the right lower lung, and pulmonary auscultation revealed decreased breath sounds in the right lower lung. The remaining physical examination was normal.
The laboratory findings were as follows: white blood cell (WBC) count 22.98 x 109/L with 85.5% segmented neutrophils, 7.6% lymphocytes, neutrophil count 19.9 x 109/L, and lymphocyte count 1.76 x 109/L, and his ultrasensitive C-reactive protein (hs-CRP) was 248.56 mg/L (normal range 0-5 mg/L). Serum tumor marker screening showed a slightly elevated neuron-specific enolase (NSE) of 17.05 ng/ml (normal range <16.3 ng/ml). The squamous cell carcinoma antigen (SCCA), cytokeratin-19-fragment (CYFRA21-1) and carcinoembryonic antigen (CEA) levels were normal. The other laboratory test results were normal. Chest computed tomography (CT) revealed an irregular mass in the right middle lobe of the lung near the interlobar fissure with right atelectasis and thickening of the right pleura with encapsulated effusion and pneumatosis (Figure 1A). Thoracentesis and catheter drainage were performed immediately after chest ultrasound confirmed pleural effusion. Analysis of the drained purulent fluid suggested an exudative process [fluid protein 54.9 g/L, serum protein 69.8 g/L (normal: 65-85 g/L), fluid lactate dehydrogenase 3,014 U/L, serum lactate dehydrogenase 220 U/L (normal 120.0-250.0 U/L)]. Other pleural fluid analysis showed WBC 3,800/muL with 80% neutrophils, fluid glucose 0 mmol/L, and fluid adenosine deaminase 81.8 U/L. Pleural fluid smear, Gram staining, bacterial culture, acid-fast bacilli culture and smear, and cytology were all negative. A blood culture was also negative. Empyema was diagnosed. He received intravenous imipenem/cilastatin 1.0 g once every 8 h. After repeated pleural effusion drainage and anti-infective treatment for 3 weeks, re-examination of lung CT showed no significant change in the amount of pleural effusion (Figure 1B).
The patient requested to be discharged after his fever improved. Half a month after discharge, he was admitted to our hospital due to progressive worsening of cough and shortness of breath. On April 1, a contrast-enhanced CT scan showed uneven enhancement of the mass in the lateral segment of the right middle lobe, mediastinal lymphadenopathy with visible enhancement, and a right hydropneumothorax (Figure 1C). In addition, the laboratory test results included WBC 11.56 x 109/L, neutrophil ratio 71.84%, and CRP 219.35 mg/L. Ultrasound re-examination showed that there was a localized anechoic area of 5.3 cm x 7.7 cm x 3.4 cm in the right thoracic cavity, and he underwent pleural puncture and drainage again. The pleural drainage fluid was sent for NGS. The NGS results only detected Fusobacterium nucleatum. Subsequently, the patient underwent bronchoscopy. Fiberoptic bronchoscopy showed complete airway obstruction due to whitish endobronchial membranous lesions in the lateral segment of the right middle lobe (Figure 2A). A cauliflower-like mass was seen and biopsied after clamping the whitish endobronchial membranous lesions (Figure 2B). Histological examination of the biopsy specimens demonstrated a highly differentiated squamous cell carcinoma in the right lung (Figure 2C). He was finally diagnosed with empyema caused by Fusobacterium nucleatum and squamous cell carcinoma of the lung. The patient's cough and shortness of breath were improved after draining 1,800 ml pleural effusion for 3 days, and cefoperazone sodium/sulbactam sodium 3 g was given intravenously twice a day for 2 weeks. Repeat chest CT revealed absorption of the fluid and gas in the right pleural cavity (Figure 1D). Meanwhile, blood biochemical examination showed that infection indexes, such as white blood cells and CRP, had returned to normal. Regrettably, the patient refused antitumor therapy.
|
fusobacterium nucleatum, empyema, lung cancer, next-generation sequencing, squamous cell carcinoma
|
CT scan images. (B) Chest CT showed no significant changes in the amount of right-sided pleural effusion after thorough pleural drainage and anti-infective treatment for 3 weeks.
|
|
PMC10248407_01
|
Male
| 49
|
We described the case of a 49-year-old man with a 1-year history of intermittent cough and sputum production who had worsened over the last 4 days with fever and right chest pain. The patient recently had a history of unexplained weight loss of 5 kg. He has smoked 20 cigarettes per day since the age of 19. The patient had periodontitis and tooth loss. The patient had no history of travel or tuberculosis exposure and no food or drug allergies.
On admission, his temperature was 37.9 C, pulse rate 81 beats/min, respiratory rate 22 breaths/min, blood pressure 132/80 mmHg, and oxygen saturation 95% on room air.
Pulmonary percussion found dullness in the right lower lung, and pulmonary auscultation revealed decreased breath sounds in the right lower lung. The remaining physical examination was normal.
The laboratory findings were as follows: white blood cell (WBC) count 22.98 x 109/L with 85.5% segmented neutrophils, 7.6% lymphocytes, neutrophil count 19.9 x 109/L, and lymphocyte count 1.76 x 109/L, and his ultrasensitive C-reactive protein (hs-CRP) was 248.56 mg/L (normal range 0-5 mg/L). Serum tumor marker screening showed a slightly elevated neuron-specific enolase (NSE) of 17.05 ng/ml (normal range <16.3 ng/ml). The squamous cell carcinoma antigen (SCCA), cytokeratin-19-fragment (CYFRA21-1) and carcinoembryonic antigen (CEA) levels were normal. The other laboratory test results were normal. Chest computed tomography (CT) revealed an irregular mass in the right middle lobe of the lung near the interlobar fissure with right atelectasis and thickening of the right pleura with encapsulated effusion and pneumatosis (Figure 1A). Thoracentesis and catheter drainage were performed immediately after chest ultrasound confirmed pleural effusion. Analysis of the drained purulent fluid suggested an exudative process [fluid protein 54.9 g/L, serum protein 69.8 g/L (normal: 65-85 g/L), fluid lactate dehydrogenase 3,014 U/L, serum lactate dehydrogenase 220 U/L (normal 120.0-250.0 U/L)]. Other pleural fluid analysis showed WBC 3,800/muL with 80% neutrophils, fluid glucose 0 mmol/L, and fluid adenosine deaminase 81.8 U/L. Pleural fluid smear, Gram staining, bacterial culture, acid-fast bacilli culture and smear, and cytology were all negative. A blood culture was also negative. Empyema was diagnosed. He received intravenous imipenem/cilastatin 1.0 g once every 8 h. After repeated pleural effusion drainage and anti-infective treatment for 3 weeks, re-examination of lung CT showed no significant change in the amount of pleural effusion (Figure 1B).
The patient requested to be discharged after his fever improved. Half a month after discharge, he was admitted to our hospital due to progressive worsening of cough and shortness of breath. On April 1, a contrast-enhanced CT scan showed uneven enhancement of the mass in the lateral segment of the right middle lobe, mediastinal lymphadenopathy with visible enhancement, and a right hydropneumothorax (Figure 1C). In addition, the laboratory test results included WBC 11.56 x 109/L, neutrophil ratio 71.84%, and CRP 219.35 mg/L. Ultrasound re-examination showed that there was a localized anechoic area of 5.3 cm x 7.7 cm x 3.4 cm in the right thoracic cavity, and he underwent pleural puncture and drainage again. The pleural drainage fluid was sent for NGS. The NGS results only detected Fusobacterium nucleatum. Subsequently, the patient underwent bronchoscopy. Fiberoptic bronchoscopy showed complete airway obstruction due to whitish endobronchial membranous lesions in the lateral segment of the right middle lobe (Figure 2A). A cauliflower-like mass was seen and biopsied after clamping the whitish endobronchial membranous lesions (Figure 2B). Histological examination of the biopsy specimens demonstrated a highly differentiated squamous cell carcinoma in the right lung (Figure 2C). He was finally diagnosed with empyema caused by Fusobacterium nucleatum and squamous cell carcinoma of the lung. The patient's cough and shortness of breath were improved after draining 1,800 ml pleural effusion for 3 days, and cefoperazone sodium/sulbactam sodium 3 g was given intravenously twice a day for 2 weeks. Repeat chest CT revealed absorption of the fluid and gas in the right pleural cavity (Figure 1D). Meanwhile, blood biochemical examination showed that infection indexes, such as white blood cells and CRP, had returned to normal. Regrettably, the patient refused antitumor therapy.
|
fusobacterium nucleatum, empyema, lung cancer, next-generation sequencing, squamous cell carcinoma
|
CT scan images. (C) Contrast-enhanced CT scan showed uneven enhancement of the mass in the lateral segment of right middle lobe, mediastinal lymphadenopathy with visible enhancement, and a right hydropneumothorax on April 1.
|
|
PMC6374571_01
|
Female
| 32
|
A 32-year-old woman presented to the Emergency Department with an initial complaint of pain and swelling in her lower extremities for 54 h, more severe in her left knee over the last 48 h. She denied any history of recent or past trauma to that region. She further added that she had previously experienced similar pain in her elbows and ankles. She did not seek any medical help at the time and her symptoms subsided with an over-the-counter nonsteroidal anti-inflammatory drug (NSAID).
On physical examination, the patient was afebrile with normal blood pressure, pulse rate and respiratory rate. The right lower extremity was normal with no edema, deformity or restricted range of motion. On the left side, she showed signs of tenderness in both the medial and lateral aspects of her left knee. She did not have any hepatosplenomegaly, and no other systemic abnormalities were found. She was prescribed painkillers and sent home.
The patient presented again 10 days later with more severe pain in different joints, mostly her ankles, wrists and knees. She reported that the symptoms had not improved since her last visit and she was unable to come earlier because of her job. The physician in charge found blanching erythematous plaques over her body, most noticeably over the trunk and back. Subcutaneous nodules were also found on both arms. No murmurs were heard on auscultation and her neurologic examination was without any abnormalities. She was immediately admitted for further examination with a suspected diagnosis of acute rheumatic fever. A throat culture was found negative for group A streptococcus but she had elevated titers of antistreptolysin O (ASO) and antideoxyribonuclease B (ADN-B). She also tested negative for human immunodeficiency virus (HIV), Chlamydia trachomatis, Neisseria gonorrhoeae and syphilis. She showed signs of acute infection with an elevated white blood cell count of 14.6/mm3 with an elevated erythrocyte sedimentation rate (ESR) of 62 mm/h. Her hemoglobin was normal at 13.2 g/dl. She was scheduled for a cardiac electrocardiogram and echocardiogram that revealed no abnormalities. The patient was started on aspirin and her symptoms gradually subsided over the next 48 h. She was discharged on high dose aspirin and oral penicillin and scheduled for regular cardiac follow-ups. At each visit, her white blood cell count, ESR, bleeding profiles, ASO and ADN-B levels were monitored closely and a cardiac echocardiogram was performed.
Acute rheumatic fever has a worldwide incidence of 19 cases per 100 000, and in the United States the prevalence ranges from 2 to 14 cases per 100 000. The pathogenesis of ARF is still not fully understood as previous studies have been limited by the lack of a suitable animal model. It is strongly believed that streptococcal pharyngeal infection from certain subtypes is the main factor causing this condition, and five chromosome patterns, A to E, have been identified on the emm genes that code for the M and the M-like surface proteins responsible for the virulence factor. Acute rheumatic fever is only associated with streptococcal pharyngitis. Many outbreaks of impetigo causing glomerulonephritis have also been documented, but they almost never caused ARF. Activation of the innate immune system following group A streptococcus pharyngeal infection leads to bacterial antigens presenting to the patient's T cells. Certain studies have also proved that susceptibility to ARF is most likely polygenic. Initiation of tissue injury is related to molecular mimicry. Antibodies directed against bacterial antigens can cross-react with host antigens. alpha-Helical protein structures found in M protein and N-acetyl-beta-D-glucosamine that form a part of the carbohydrate antigen of GAS also share epitopes with myosin. Molecular mimicry is also believed to be the cause of chorea as the antibodies target both NABG and lysoganglioside.
Diagnosis of ARF is currently based on the Jones criteria. The presence of either two major criteria or one major criterion and two minor along with positive evidence of recent group A streptococcal infection is required to establish the diagnosis of acute rheumatic fever. Recent updates in diagnostic criteria also encouraged the use of echocardiography to confirm the diagnosis of carditis. Major diagnostic criteria include carditis, arthritis, chorea, subcutaneous nodules and erythema marginatum. About 50-65% of patients with ARF have clinically detectable carditis that leads to valvular regurgitation. Carditis usually develops over weeks to months. The most commonly affected valve is usually the mitral valve, followed by the aortic valve. Congestive cardiac failures can occur if untreated and severe carditis develops. Arthritis presents as migratory and asymmetrical. It involves mostly the large joints, causing edema, pain, rubor and limited movement. Up to 15% of ARF patients present with chorea, and it is more common in females and adolescents. The onset of chorea usually occurs after a long latent period when most of the inflammatory symptoms are resolved. Patients present with involuntary jerky movements such as clumsiness and hemichorea and also have deterioration in performing daily activities such as feeding or have an unsteady gait. Erythema marginatum is a rare presentation that occurs in less than 5% of cases, appearing as an annular erythema on the upper and lower extremities and torso. The edges are raised and the rash is not painful or itchy. Subcutaneous nodules are another rare finding that forms part of the major criteria. They present as small painless mobile nodules on extensor surfaces of wrists, elbows, knees and ankles. They usually appear during the first few weeks and can even be found in the spine. Minor criteria of diagnosis include symptoms such as fever, arthralgia, first degree heart block, elevated acute phase reactants and presence of prior group A streptococcus infection. Arthralgia is not used as a minor criterion if polyarthritis is already present. It is often missed by physicians since the patients start self-medicating themselves with anti-inflammatory drugs such as NSAIDs, as was seen in this case report. First degree heart block can be used as a minor criterion if there is no evidence of carditis. Nonspecific symptoms such as fever also form part of the minor criteria. Laboratory findings such as an elevated ESR can also be used as a minor criterion along with evidence showing a recent group A streptococcal infection. A fourfold rise or fall in titers is diagnostic for a recent streptococcal infection. Commonly tested antibodies include deoxyribonucleic (anti-DNase B) antibodies and anti-streptolysin O titer (ASOT).
There are multiple conditions that may mimic symptoms resembling ARF that need to be ruled out. These include congenital heart disease, viral myocarditis, infective endocarditis, other causes of pericarditis such as connective tissue disease, innocent cardiac murmurs, septic arthritis, reactive arthritis, juvenile idiopathic arthritis, sickle cell disease, gout, leukemia, Wilson disease, Huntington cholera, and intracranial tumor. Proper imaging and laboratory tests can help rule out these conditions to establish a definitive diagnosis of ARF.
The treatment plan of ARF includes antibiotic therapy, anti-inflammatory treatment and management of cardiac symptoms. The goals should include symptomatic relief, eradication of the group A streptococcal infection, prophylaxis against future infection and proper education to prevent future episodes. The most convenient way to treat GAS pharyngitis is with the use of long acting penicillin G. Symptomatic relief or arthritis symptoms can be provided with nonsteroidal anti-inflammatory drugs such as aspirin or naproxen. Other options also include using low dose glucocorticoids for patients who are allergic to aspirin or naproxen. Carditis can be prevented with early echocardiography and prevention of complications.
In conclusion, acute rheumatic fever is a condition that can be treated appropriately if detected early. While it was difficult to detect the diagnosis on the patient's first visit, her second visit showed appropriate symptoms for the diagnosis. Once ARF is considered, appropriate laboratory examinations and cardiac testing should be done. Early detection and treatment can prevent the risk of complications.
| null | Not supported with pagination yet
| null |
PMC3621243_01
|
Female
| 28
|
A 28-year-old woman, second gravida, presented at 26 gestational weeks, with 20 day history of intermittent fever and generalized bodyaches, 2 months history of nocturia, and 1 month history of urinary urgency and frequency. She was treated locally with chloramphenicol and norfloxacin. She presented with septic shock and a presumptive diagnosis of acute pyelonephritis was made. The WBC total count was 26,000 (95% neutrophils, 5% lymphocytes), hemoglobin 7.5 g/dl, serum urea 154 mg/dl, serum creatinine 4.5 mg/dl, serum sodium 130 meq/l, serum potassium 5.5 meq/l, serum chloride 115 meq/l, and serum bicarbonate 7 meq/l. The arterial blood gas showed partially compensated metabolic acidosis (pH 7.05, HCO3 09 mmol, PCO2 21 mmHg, and PO2 112 mmHg).
The urine showed plenty of pus cells, and all her blood and urine cultures were sterile. Her ultrasonogram of the abdomen showed enlarged, heterogeneous appearing kidneys with hyperechoic renal pyramids suggestive of medullary nephrocalcinosis [Figure 1] with no perinephric stranding. There was no evidence of hydronephrosis or calculus. She was empirically treated with meropenem 500 mg IV once daily and vancomycin 1 g IV once in 5 days. She remained febrile even after 72 h of antibiotics, and her urine and blood grew no organism. Injection amikacin was added after appropriate dose modification. She received four sessions of hemodialysis over 5 days for her severe renal failure and severe metabolic acidosis. He renal function rapidly improved by the end of 1 week and she became afebrile 48 h after adding amikacin. Amikacin was discontinued after 7 days due to fear of fetal ototoxicity, and meropenem and vancomycin were stopped after 10 days by which time her serum creatinine had improved to 1.8 mg/dl. Her fetus showed normal growth for the gestational age and she was discharged to come after 2 weeks with repeat urine culture and follow-up. Four days before her planned visit, she had developed bilateral loin pain. There was left renal angle tenderness and firm palpable swelling was noticed locally. Ultrasound showed bilateral perinephric collection with left psoas abscess and MRI scan showed significant bilateral psoas and perinephric abscesses [Figure 2]. A 10 F pigtail catheter was inserted in both the psoas abscesses. The "milk-coffee" colored pus showed 3+ acid fast bacilli (AFB) and culture grew E. coli, and there was a delayed growth of Enterococcus sp. The same organisms were isolated in repeated sampling on both sides. HIV was negative and her CD3 count was also within normal limits. Her corrected serum calcium and magnesium were 9.4 g/dl and 1.8 mg/dl, respectively, and her parathormone level was 71 ng/ml, low for her level of renal function. Her 24-h urine calcium was 112 mg/day and her urine calcium creatinine ratio was 0.12.
The patient was started on isoniazid, rifampicin, ethambutol, pyrazinamide, and pyridoxine for TB, and Inj. piperacillin and tazobactum for E. coli and ampicillin for Enterococci as per the antibiotic sensitivity. She developed oligohydraminos and intrauterine growth retardation during treatment. At 34 weeks, she developed labor pain spontaneously and delivered a 1.54-kg male baby. The child was given Bacillus Calmette-Guerin (BCG) vaccination and isoniazid prophylaxis. There was no relapse of psoas abscess 1 month after removal of both her pigtail catheters. Her renal function, which was stable, worsened after pigtail catheter insertion and after starting of ATT. The serum creatinine rose from 1.4 mg/dl to 1.9 mg/dl. No cause was identified and no change in therapy was made. Her renal function improved after 10 days to 1.2 mg/dl at the time of her delivery without any change in her medications. Four months after discharge, the serum creatinine improved to 0.8 mg/dl and the ultrasound showed no evidence of nephrocalcinosis.
|
acute pyelonephritis, abscess, nephrocalcinosis, pregnancy, psoas
| Not supported with pagination yet
| null |
PMC1361263_01
|
Male
| 44
|
The patient studied in this report is a 44-yr-old male diagnosed with acute myeloid leukemia (M2 subtype) in December 2000. He responded to induction chemotherapy and achieved complete remission before undergoing allogeneic HSCT. In April 2001 he received a myeloablative preparative regimen consisting of 120 mg/kg cyclophosphamide and 1,400 cGy total body irradiation followed by infusion of G-CSF-mobilized blood stem cells from an HLA-identical female sibling. He remains in complete remission 2 yr after HSCT. His prophylactic immunosuppressive treatment included tacrolimus and methotrexate. 5 wk after transplantation, he developed acute GVHD of the skin (grade 2 GVHD). Acute GVHD responded to treatment with prednisone, daclizumab, and mycophenolate mofetil but did not resolve completely. He was subsequently treated for limited chronic GVHD with involvement of skin and oral mucosa with a combination of mycophenolate mofetil, tacrolimus, and prednisone. Chronic GVHD persists 2 yr after transplantation and the patient remains on immunosuppressive drugs.
Synthetic peptides were obtained from New England Peptide. Peptides were either unpurified (93 screening peptides), or >85% purity (DBX34-47, DBY34-47, DBX30-48, DBY30-48, DBX120-136, DBY118-134, DBX538-554, DBY536-552). Each peptide was reconstituted at 10 mg/ml DMSO and stored at -20 C.
Patient and allogeneic EBV-transformed B cell lines were generated by incubating PBMCs with supernatant from EBV-producing B95-8 marmoset cells. Once established, cell lines were maintained in RPMI 1640 supplemented with 10% heat-inactivated FCS, 4 mM glutamine, 1 mM sodium pyruvate, 10 mM Hepes, and antibiotics. T cell lines and clones were cultured in RPMI 1640 supplemented with 10% heat-inactivated human AB serum, glutamine, sodium pyruvate, Hepes, and antibiotics (referred to as T cell medium) in the presence of 100 U/ml recombinant IL-2. Medium was replenished twice weekly with fresh IL-2. Immature DCs were obtained as previously described. In brief, 40-60 x 106 PBMCs were incubated for 15 min in a 75-cm2 Costar culture flask (Corning Inc.). Nonadherent cells were removed by thoroughly washing the flask three times with medium. The remaining adherent cells were incubated for 1 wk in medium supplemented with 1,000 U/ml IL-4 (R&D Systems) and 1,000 U/ml GM-CSF (R&D Systems). Cells were then harvested, washed, and used as immature DCs. Maturation of DCs was achieved by incubating cells for 24 h with 25 ng/ml poly-IC (Amersham Biosciences) or 100 ng/ml LPS (Sigma-Aldrich).
A T cell line was generated by stimulating 6 x 106 PBMCs collected from the patient 16 mo after HSCT in the presence of peptide DBY34-50 at a final concentration of 25 mug/ml in the presence of 100 U/ml IL-2. 1 wk after stimulation, CD4+ T cells were immunopurified from the cell line using anti-CD4+ microbeads (Miltenyi Biotec) and subsequently cloned by limiting dilution on feeder cells consisting of irradiated patient EBV B cells (1.5 x 104 cells/well; 60 Gy) and allogeneic PBMCs (7 x 104 cells/well; 35 Gy) in the presence of 100 U/ml recombinant human IL-2. All of the T cell clones generated were assessed for specific reactivity with the DBY peptide. Clone 2F9 displayed strong reactivity and was further expanded under the same culture conditions with the addition of PHA (Invitrogen).
ELISPOT assays were performed as previously described. In brief, 1-2 x 105 PBMCs were incubated in duplicate cultures in the presence of 10 mug/ml different peptides in multiscreen-IP microplates (Millipore) previously coated with anti-IFN-gamma mAb (Mabtech). After an overnight incubation, plates were washed six times in PBS and incubated with a biotinylated anti-IFN-gamma mAb (Mabtech) at room temperature for 75 min. The plates were washed again and incubated with streptavidin alkaline phosphatase (Mabtech) for 45 min at room temperature. The plates were finally washed and spots were revealed using nitroblue tetrazolium and brom-chlor-indolyl phosphate color development substrates (Promega). Spots were counted in duplicate wells and results were presented as number of spot forming cells/106 PBMCs.
IFN-gamma secretion by T cell clone 2F9 was assessed by incubating 5 x 104 responder cells in 96-well, U-bottom microplates with 5 x 104 stimulator cells in 200 mul T cell medium containing 50 U/ml IL-2. Stimulator cells consisted of autologous or allogeneic EBV B cells pulsed with synthetic peptides (10 mug/ml or as otherwise stated) for 1 h at 37 C and washed twice to remove excess peptide. After 18 h of incubation, 50 mul/well supernatant was harvested and IFN-gamma was measured using an ELISA kit (Endogen) according to the manufacturer's instructions. In experiments with DCs, 10,000 immature DCs/well were incubated in 100 mul medium with or without LPS or poly-IC for 24 h at 37 C. 4 x 104 2F9 cells were then added to the culture in the presence of 50 U/ml IL-2, and secretion of IFN-gamma was measured after 18 h of incubation.
Target cells were labeled with 51Cr for 1 h at 37 C, washed three times, pulsed with the corresponding peptide for an additional hour at 37 C, washed three times again, and then plated at 2,000 cells/well in conical-bottom, 96-well microplates. Clonal 2F9 cells were added at varying effector/target ratios and 51Cr release was measured in the supernatant after 4 h of incubation at 37 C. In cold target inhibition experiments, HLA-DR*1501 female or male unlabeled matured DCs were incubated with 51Cr-labeled targets at various ratios for 1 h before adding the T cell clone.
Recombinant DBX and DBY protein production and purification has been described. In brief, full-length DBX and DBY cDNA were cloned using the Gateway cloning system (Invitrogen) into pDEST42 prokaryotic expression vector introducing V5 and 6 HIS Tag epitopes at the protein C terminus. After transformation in Escherichia coli, DBX and DBY expression was induced with isopropyl-beta-d-thiogalactopyranoside and proteins were purified from bacterial lysates by affinity chromatography on nickel columns (Invitrogen). For Western blotting, 5 mug of each protein was run by electrophoresis on a 4-12% polyacrylamide gel in denaturing conditions using the NuPAGE system (Invitrogen), transferred on a nitrocellulose membrane, and blocked for 1 h in blocking buffer (1X TBST, 5% nonfat dry milk). Membranes were incubated overnight with either horseradish peroxidase (HRP)-conjugated anti-V5 epitope mAb (Invitrogen) or patient serum diluted at 1:10 in blocking buffer, and then with HRP-conjugated goat anti-human IgG antisera (Jackson ImmunoResearch Laboratories). Proteins were subsequently detected using the "Western Lightning" chemiluminescence HRP reagents (PerkinElmer).
Antibodies to DBX and DBY recombinant protein, as well as DBX and DBY peptides, were measured by ELISA. In brief, high protein adherence Nunc-Immunoplates (Nunc) were coated overnight with either 5 mug/ml purified recombinant protein or 10 mug/ml synthetic peptides in carbonate-binding buffer, washed in TBST, and then blocked with 2% nonfat dry milk in TBST. Serum diluted in blocking buffer was then added to the plates and incubated either overnight at 4 C for the recombinant protein-coated plates, or for 3 h at 20 C for the peptide-coated plates. Plates were then washed in TBST, incubated for 1 h at 20 C with alkaline phosphatase-conjugated goat anti-human IgG antisera (Pierce Chemical Co.), diluted in blocking buffer, washed again, and developed using pNPP alkaline phosphatase substrate colorimetric reagents (Sigma-Aldrich). Optical density was read at 405 nm.
| null | Not supported with pagination yet
| null |
PMC10310954_01
|
Male
| 32
|
A 32-year-old man was admitted to a local hospital with paroxysmal episodes of unconsciousness for 6 days and had convulsions 2 days ago. The patient also reported suffering from diplopia for 2 months, accompanied by headache, nausea, and vomiting. Lumbar puncture showed a cerebrospinal fluid (CSF) pressure of >330 mmH2O and protein level at 440 mg/dL, CSF WBC count was 1 x 106/L, and CSF color was yellow. The patient was diagnosed with encephalitis and was given antiviral and anti-inflammatory treatments, but there was no significant improvement in his condition. He was later transferred to our hospital for further treatment. His past medical history showed "lumbar disk herniation" for more than 10 years. In the recent 2 months, his symptoms had worsened, accompanied by discomfort in the right lower limb. Respiratory infection and diarrhea were not indicated in the medical history before this disease. Family history showed that the patient's maternal grandfather had a history of tuberculosis 30 years ago, which was cured. The patient's sister had died of intracranial glioma at 6 years of age, and a pathological test was not performed. At the time of admission, the patient was conscious and showed fluent speech, and muscle strength of both upper limbs was grade V and of lower limbs was grade V minus. The Babinski sign was negative bilaterally. Both T-SPOT and PPD tests were positive. A Cranial magnetic resonance imaging (MRI) scan showed diffuse meningeal enhancement caused by infection (Figure 1A). The patient was therefore diagnosed to have tuberculous meningitis. Following admission, he was treated with mannitol and glycerin fructose to reduce intracranial pressure, and anti-tuberculosis treatment was started.
During hospitalization, the patient complained of lower back pain and increased weakness in both lower limbs. On the 7th day, the patient had seizures, and his body temperature increased to 38 C. The patient still has headaches and vomiting. Physical examination showed that the patient was somnolent but arousable to stimulus, had limited abduction of both eyes, had sensitivity to pupillary light reflexes, showed grade IV muscle strength in both the upper limbs, had a stiff neck, showed grade II muscle strength in both the lower limbs, and had hypesthesia of both lower limbs, and the bilateral Babinski sign showed all toes were downgoing. MRI scans of the thoracic and lumbar spinal cord were again performed on the 8th day following hospitalization. The results showed extensive thickening and enhancement of the thoracolumbar spinal meninges (Figure 1B). Electroencephalogram showed increased slow waves in frontal and temporal lobes (Figure 1C). Two lumbar punctures were performed on day 2 and day 7 of hospitalization, and the results showed a significant increase in intracranial pressure and protein level and a decrease in chloride level (Table 1). Acid fast stain and tuberculosis cultures were negative. Though the anti-tuberculosis treatment showed no improvement in the patient's condition, the treatment was continued as his body temperature remained high, up to 38.5 C. The next-generation sequencing of CSF for detecting associated infections and three subsequent lumbar punctures provided no additional diagnostic information (Table 1). After 18 days of anti-infective treatment, the patient's symptoms of high intracranial pressure did not improve, but his body temperature returned to a normal level. The intracranial pressure remained high, with high protein and low chlorine levels. However, we noticed that there was no significant increase in the white blood cell count, and the cytology of the patient's cerebrospinal fluid showed an increase in the number of activated monocytes while malignant cells were not detected (Figure 1D). These results lead us to consider the presence of tumors in the spinal cord. A positron emission tomography-computed tomography (PET-CT) scan was performed, and the results showed multiple hypermetabolic areas scattered in the spinal canal at the level of the T2-L2 vertebral body, thus indicating the possibility of intramedullary tumors with intraspinal metastasis (Figure 1E).
On the 21st day of hospitalization, the patient's condition worsened. He was unconscious, his pupillary reflex disappeared, and he was diagnosed with a cerebral hernia. The patient was medicated to reduce intracranial pressure, and an emergency ventriculoperitoneal shunt was performed. The patient regained consciousness after surgery. On the 27th day, the thoracolumbar MRI scans were re-examined (Figure 1F), showing a significant increase in abnormal intramedullary signals at the thoracic (T) 12-lumbar (L) 1 vertebra level compared to that seen in the first scan. After that, the patient was transferred to the neurosurgery department. On the 35th day of admission, the patient underwent partial resection of space-occupying lesions in the spinal canal at T12-L1 of the vertebral body and spinal nerve adhesion release. The histopathological examination of the neuroepithelial tumor removed from the spinal tissue revealed a malignant tumor. The immunohistochemistry results showed GFAP (+), Oligo-2 (+), H3K27M (+), Ki-67 (+), IDH-1 (-), and EMA (-) (Figure 2). Combined with conventional pathological morphology and immunophenotype, the tumor was consistent with DMG, H3 K27-altered (WHO grade IV). The final diagnosis was DMG, H3 K27-altered. The patient was then transferred to the radiotherapy department and was recommended for local radiotherapy plus temozolomide chemotherapy. After radiotherapy, the patient's consciousness was better than before, and he could answer simple questions correctly. Abduction of both eyes was still limited, and pupillary reflex was found sensitive. His upper limb muscle strength improved, and the patient was discharged after his condition stabilized. No relevant disease assessment was performed during follow-up, and the patient died 6 months after discharge due to disease progression.
|
h3 k27-altered, adult, case report, central nervous system infection, diffuse midline glioma, spinal cord neoplasms
| Not supported with pagination yet
| null |
PMC5660782_01
|
Female
| 43
|
A 43-year-old Hispanic woman presented with recurrent asymmetrical bilateral eye redness, tearing, and pain for the past 2 years, with more severe symptoms in the right eye. Her ocular history was negative prior to this presentation. Her past medical history is significant only for rosacea, diagnosed clinically more than ten years ago by a dermatologist. She denied illicit drug use, exposure to patients with tuberculosis, or promiscuous sexual activity. Her family history is negative for ocular diseases and for autoimmune diseases. Aside from her eyes and her skin complaints, her review of systems was negative. Her right eye visual acuity was 20/40 and intraocular pressure was 8 mm Hg. Right eye slit lamp exam showed iris plugging a perforated cornea superonasally with associated extensive neovascularization (Figure 1) and a shallow but formed anterior chamber (Figure 2).
The cornea in her left eye appeared normal. Signs of bilateral blepharitis due to ocular rosacea such as eyelid telangiectasias, thick meibomian gland secretions with digital compression, and collarettes on the base of upper and lower eyelashes were present. The patient consented to an emergent, full thickness, tectonic corneal patch graft. Postoperative management consisted of topical steroid (prednisolone acetate ophthalmic solution 1%) every two hours that was tapered slowly after one-week period and topical moxifloxacin four times daily for a total period of two weeks. Concurrently a dermatologist initiated rosacea treatment consisting of minocycline 100 mg orally twice daily and benzoyl peroxide/erythromycin gel daily. At week one after grafting, the visual acuity was 20/100 (Figure 3).
The patient's bilateral eye signs and symptoms improved significantly one month after initiating rosacea treatment.
| null | Not supported with pagination yet
| null |
PMC5660782_02
|
Male
| 51
|
A 51-year-old African American man presented with bilateral eye pain and redness of four months' duration. Patient denied ocular problems previously. His prior medical history was significant for rosacea, for which he was not receiving any medical treatment. His review of systems was positive for bilateral eye pain and tender facial papules and pustules. He denied family history of autoimmune diseases or eye diseases. He denied promiscuous sexual activity, exposure to tuberculosis, or illicit drug use. His external exam was consistent with multiple medium to large erythematous papules, pustules with rhinophyma (Figure 4).
A skin biopsy was done and it revealed disrupted follicles with surrounding mixed suppurative and granulomatous dermatitis most suggestive of acneiform processes including ruptured folliculitis and rosacea. The patient was started on doxycycline 100 mg twice daily and topical metronidazole 1% cream twice daily.
His visual acuity was 20/400 in the right eye and 20/30 in the left eye. Slit lamp exam showed severe bilateral conjunctival injection, corneal pannus associated with inferonasal peripheral corneal thinning, and stromal scarring. His right eye showed an inferior 3 mm light-blocking infiltrate with overlying 3 mm epithelial defect.
Peripheral ulcerative keratitis (PUK) was suspected and oral prednisone 60 mg was started. An extensive autoimmune workup was done. All results were negative. Cornea was scraped for gram stain, bacterial cultures, and fungal cultures. The patient was started on topical hourly fortified vancomycin (25 mcg/mL) and tobramycin (15 mcg/mL). The patient continued to worsen and within two weeks developed right eye inferior corneal perforation, for which adjacent conjunctival resection, glue, and a bandage contact lens were applied (Figure 5). Topical fortified vancomycin (25 mcg/mL) and tobramycin (15 mcg/mL) were given at a lower rate of six times daily. Oral treatment consisted of doxycycline 100 mg twice daily and 60 mg of prednisone for one week that was tapered by 20 mg on a weekly basis. Close follow-up showed slow improvement afterwards. After four weeks of treatment, the corneal infiltrate resolved. The patient provided his informed consent prior to publishing this information.
| null | Not supported with pagination yet
| null |
PMC10359671_01
|
Male
| 61
|
A 61-year-old truck driver was referred for a painless mass on the left ring finger that lasted for 1 year; the lesion began to expand aggressively during the last 3 months. It started at the finger base and gradually expanded to involve the entire finger, which markedly limited hand function (Fig. 1a). There was no precipitating factor, swelling elsewhere, or history of malignancy. The patient reported an unintentional weight loss of 15 kg without other constitutional symptoms.
An examination revealed a 10 x 10 cm circumferential mass on the left ring finger extending from the distal interphalangeal joint to the fourth metacarpal head. It was firm to hard in consistency and was multilobulated with an irregular surface. The mass was warm, non-tender and attached to the overlying skin with dilated veins. The little and middle fingers were splayed due to the mass effect. Sensation over the ring finger was reduced, but intact circulation.
A plain radiograph showed a large soft tissue mass of the left ring finger that displaced neighbouring fingers (Fig. 1c). There was extensive destruction of the proximal and middle phalanges with soft tissue calcification. Local magnetic resonance imaging (MRI) showed an expansile mass arising from the proximal phalanx with a soft tissue component occupying the adjacent web spaces (Fig. 1d). The mass showed heterogeneous isointense in T1W and T2W and enhancement post-contrast. There was a non-enhancing cystic area, which may represent a cystic component or necrotic area. An area of persistent hypointensity was observed on T1W, T2W, and blooming on GRE and may represent calcification. It eroded the fourth metacarpal head and infiltrated the adjacent lumbricals and interossei muscles. Computed tomography scan of the lung showed no evidence of lung metastasis. The clinical and radiological differential diagnosis at that point of time was chondrosarcoma or osteosarcoma.
A tissue biopsy showed a low-grade spindle cell lesion. Subsequently, the patient underwent rays amputation of the ring and small fingers and wound closure by means of a local ulnar-based flap originating from the small finger (Fig. 1b). Histopathological examination revealed an ill-circumscribed and destructive bone lesion composed of mostly uniform plump spindled cells that have an indistinct moderate eosinophilic to amphophilic cytoplasm. The intervening stroma was highly collagenized. No osteoid was seen. Thick fibrous septae were present in between the tumour cells. No significant nuclear atypia or mitosis was observed (Fig. 2). Tumour cells were focally positive for SMA but negative for desmin, EMA, BCL2, TLE-1, SATB2, STAT6, CK7, CD34, and B-catenin. The overall morphology was in favour of DF of bone. However, the surgical resected margin was involved with tumour cells. At 6 months of follow-up, there was no local recurrence and he had good overall hand function with acceptable hand grip strength (Fig. 3). Nevertheless, a long-term follow-up was necessary to evaluate for local recurrence.
|
benign, desmoplastic, fibroma, phalanges
| Not supported with pagination yet
| null |
PMC6884965_01
|
Female
| 52
|
A 52-year-old female with a history of anti-tuberculosis therapy for six months was referred to our department because a new mass appeared on chest CT during a regular routine checkup. Six months ago, the patient complained of right chest pain for one year. At that time, the CT showed a 1.7x1.4cm nodule in the right-superior pulmonary lobe (Figure 1A and B). There were no apparent lesions in the right-inferior lobe (Figure 2A and B). She denied history of smoking, malignancy, diabetes and other serious illness. Since both interferon-gamma release assays (IGRAs) and tuberculin skin tests were positive, a diagnosis of pulmonary tuberculosis was made. The patient started an induction chemotherapy consisting of isoniazid, rifampicin, ethambutol, pyrazinamide and levofloxacin. Due to the liver damage, the regimens were changed to isoniazid, rifapentine and ethambutol. Her symptoms were gradually improved. A week before this admission, CT scans revealed a new occupying lesion (3.4cm x 2.3cm) in the right-inferior lobe (Figure 2C and D), whereas the original nodule located in the superior lobe demonstrated improvement (Figure 1C and D). The patient denied symptoms of fever, cough, chest pain or weight loss. White blood cell (WBC) counts were 4.28x109/L with 17.80% lymphocytes. Tuberculin skin test remained positive. Other laboratory results including erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), tumor markers, galactomannan test (GM-test), (1, 3)-beta-D glucan test (G-test), autoantibodies related to connective tissue disease were within the normal ranges. HIV test was negative. CT-guided percutaneous lung biopsy demonstrated caseating granulomas (Figure 3). The patient was diagnosed as paradoxical reaction and was continued on anti-tuberculosis therapy. The size of new pulmonary mass was reduced to 1.5cm x 1.3cm one month later (Figure 2E and F) and was further reduced to 0.9cm x 1.1cm (Figure 2G and H) three months later. Currently, the patient is asymptomatic, and the original therapy continues.
|
mass, paradoxical reaction, tuberculosis
|
Case 1. CT-guided percutaneous lung biopsy shows caseous necrosis and granulomatosis surrounded by epithelioid and multinucleated giant cells. Hematoxylin and eosin, x400.
|
|
PMC6884965_02
|
Female
| 35
|
A 35-year-old female was admitted to a community hospital because of polypnea, cough, sputum and intermittent fever four months ago. She has a medical history of HBV infection. The patient denied a history of smoking. Local CT scans revealed right pleural effusion and pleural thickening. Diagnostic thoracentesis showed tuberculous exudative fluid. A diagnosis of tuberculous pleuritis was speculated. The patient started anti-tuberculosis therapy with isoniazid, rifampicin, ethambutol and pyrazinamide. The patient's symptoms were gradually relieved, and the therapy was continued. Two weeks before, the patient developed cough and sputum. CT scans revealed a 3.9cm x 2.9cm mass in the right middle and inferior pulmonary lobe with no pleural effusion (Figure 4). WBC was 7.03x109/L with 80.90% neutrophils. HGB was 122g/L. Results of examinations of ESR, CRP, liver function, tumor markers, GM-test, G-test and autoantibodies related to connective tissue disease were normal. HIV test was negative. IGRAs and tuberculin skin test were positive. Sputum smears and culture were negative for acid-fast bacilli. CT-guided percutaneous lung biopsy showed plentiful infiltration of epithelioid cells and lymphocytes (Figure 5). The patient was diagnosed as PR paradoxical and was continued on the original anti-tuberculosis therapy. She recovered well during the follow-up. Local CT scans showed that the pulmonary mass was diminished three months later.
|
mass, paradoxical reaction, tuberculosis
|
Case 2. CT findings in the right middle and inferior pulmonary lobe. (A and B) A 3.9cmx2.9cm lobulated mass without pleural effusion was found was after four months of anti-tuberculosis treatment.
|
|
PMC6884965_02
|
Female
| 35
|
A 35-year-old female was admitted to a community hospital because of polypnea, cough, sputum and intermittent fever four months ago. She has a medical history of HBV infection. The patient denied a history of smoking. Local CT scans revealed right pleural effusion and pleural thickening. Diagnostic thoracentesis showed tuberculous exudative fluid. A diagnosis of tuberculous pleuritis was speculated. The patient started anti-tuberculosis therapy with isoniazid, rifampicin, ethambutol and pyrazinamide. The patient's symptoms were gradually relieved, and the therapy was continued. Two weeks before, the patient developed cough and sputum. CT scans revealed a 3.9cm x 2.9cm mass in the right middle and inferior pulmonary lobe with no pleural effusion (Figure 4). WBC was 7.03x109/L with 80.90% neutrophils. HGB was 122g/L. Results of examinations of ESR, CRP, liver function, tumor markers, GM-test, G-test and autoantibodies related to connective tissue disease were normal. HIV test was negative. IGRAs and tuberculin skin test were positive. Sputum smears and culture were negative for acid-fast bacilli. CT-guided percutaneous lung biopsy showed plentiful infiltration of epithelioid cells and lymphocytes (Figure 5). The patient was diagnosed as PR paradoxical and was continued on the original anti-tuberculosis therapy. She recovered well during the follow-up. Local CT scans showed that the pulmonary mass was diminished three months later.
|
mass, paradoxical reaction, tuberculosis
|
Case 2. CT findings in the right middle and inferior pulmonary lobe. (A and B) A 3.9cmx2.9cm lobulated mass without pleural effusion was found was after four months of anti-tuberculosis treatment.
|
|
PMC6884965_02
|
Female
| 35
|
A 35-year-old female was admitted to a community hospital because of polypnea, cough, sputum and intermittent fever four months ago. She has a medical history of HBV infection. The patient denied a history of smoking. Local CT scans revealed right pleural effusion and pleural thickening. Diagnostic thoracentesis showed tuberculous exudative fluid. A diagnosis of tuberculous pleuritis was speculated. The patient started anti-tuberculosis therapy with isoniazid, rifampicin, ethambutol and pyrazinamide. The patient's symptoms were gradually relieved, and the therapy was continued. Two weeks before, the patient developed cough and sputum. CT scans revealed a 3.9cm x 2.9cm mass in the right middle and inferior pulmonary lobe with no pleural effusion (Figure 4). WBC was 7.03x109/L with 80.90% neutrophils. HGB was 122g/L. Results of examinations of ESR, CRP, liver function, tumor markers, GM-test, G-test and autoantibodies related to connective tissue disease were normal. HIV test was negative. IGRAs and tuberculin skin test were positive. Sputum smears and culture were negative for acid-fast bacilli. CT-guided percutaneous lung biopsy showed plentiful infiltration of epithelioid cells and lymphocytes (Figure 5). The patient was diagnosed as PR paradoxical and was continued on the original anti-tuberculosis therapy. She recovered well during the follow-up. Local CT scans showed that the pulmonary mass was diminished three months later.
|
mass, paradoxical reaction, tuberculosis
|
Case 2. CT-guided percutaneous lung biopsy shows plentiful infiltration of epithelioid cells and lymphocytes. Hematoxylin and eosin, x400.
|
|
PMC6884965_03
|
Female
| 17
|
A 17-year-old female was diagnosed as pulmonary tuberculosis during a physical examination at the local hospital six months ago. She adopted a therapy of isoniazid, rifampicin, ethambutol and pyrazinamide. During the follow-up, CT scans showed that the original lesions were absorbed gradually while a new 1.4cm x 0.8cm (Figure 6A and B) nodule appeared in the left-superior lobe six months later. The patient denied symptoms of fever, cough, chest pain or weight loss. Laboratory results of ESR, CRP, liver function, tumor markers, GM-test, G-test and autoantibodies related to connective tissue disease were normal. HIV test was negative. IGRAs and tuberculin skin test were positive. CT-guided percutaneous lung biopsy showed tuberculous granuloma. The patient was diagnosed as PR and was continued on the original anti-tuberculosis therapy. The pulmonary mass was reduced to 1.0cm x0.3cm (Figure 6C and D) two months later.
|
mass, paradoxical reaction, tuberculosis
| null |
|
PMC2810606_01
|
Female
| 38
|
A 38-year-old woman presented to the department of general surgery, Kasturba Medical College Hospital, Manipal, on 15th may 2002, complaining of repeated episodes of abdominal pain, vomiting and loss of consciousness for the past 2 years. She was a tailor by profession.
She had first suffered from an attack of severe abdominal pain 2 years previously, while standing in the bus terminus. Shortly afterwards, she vomited and fell down unconscious and remained so for 2 to 5 minutes. On regaining consciousness, she could not recollect the details of the episode. Medical help was sought but no alleviation was found for her symptoms. She had 6 such episodes over a period of 2 years. The last episode occurred 15 days prior to her hospitalization at our institution. Her abdominal pain was spasmodic and localized to the umbilical area and the lower abdomen. It was mild to moderate in intensity. However, she occasionally experienced severe pain in the epigastric region, associated with vomiting and a peculiar sensation which permeated throughout the same area. This was invariably followed by loss of consciousness for 2 to 5 minutes. After regaining consciousness, she could not recollect ictal events. During such episodes, she experienced neither incontinence of the bowel or bladder, nor generalized tonic-clonic seizures. Post ictal automatism was absent. Patient initially was not aware of the importance of the epigastric pain. Soon she understood the sequence of events and was able to avoid falling down.
In 1990, she was diagnosed with pulmonary tuberculosis and treated with a standard course of drugs for 6 months. She is married with two children. Both were normal vaginal deliveries. After the birth of her second child, she underwent post-partum sterilization in 1991. There was no other significant medical history.
On examination, she was found to be moderately built and moderately well nourished. Abdominal examination did not reveal any abnormality. The examination of the central nervous system was also normal. All other examinations were also within normal limits.
Hemogram was normal. ESR (Erythrocyte Sedimentation Rate) was 14 mm at the end of the first hour. Urine microscopy showed 2 - 4 WBC/hpf, and 0-1 RBC/hpf. Urine culture showed the presence of E-coli 105 cfu/mL. The ultrasound examination of the abdomen, kidney, ureter and bladder was normal. Blood, renal and liver functions were normal. Serum amylase and lipase were within normal limits. Screening for porphyria was done and it was also normal. In view of the presence of RBCs in the urine and spasmodic pain in the abdomen (in spite of the ultrasound examination of the abdomen being normal), she underwent an intravenous urogram. It was normal, but incidentally a needle was found in her abdomen at the L3 level on the left side (Fig. 1 and 2). However, this finding alone could not explain her fainting attacks. Psychiatric evaluation did not reveal any functional abnormality. She was further evaluated with a CT scan of the brain and EEG (Electro Encephalo Gram). The CT scan of the brain was normal. The EEG revealed the presence of right fronto-temporal sharp waves with secondary generalized spike wave discharges, especially precipitated by hyperventilation. She was prescribed 300 mg eptoin tablets, to be taken at night. The patient willfully expressed the desire to have the foreign body removed, in spite of our telling her that it might not be the cause of her abdominal pain. Laparotomy was done 15 days later and the needle was recovered from the root of the transverse mesocolon on the left side. It was brittle and it broke while we were trying to remove it (Fig. 3). The postoperative period was uneventful. Eptoin administration was continued. She is currently being followed-up. To date, she has not suffered from any further episodes.
| null | Not supported with pagination yet
| null |
PMC7533951_01
|
Male
| 23
|
A 23-year-old Pakistani male biomedical engineer, with no significant past or present medical history, presented to the emergency department (ED) with a 3-day history of severe hearing loss that was associated with fever and disorientation. The patient appeared anxious and agitated due to his apparent hearing impairment based on his father. According to him, the patient had reported a history of fever, worsening headache, neck pain, and flu-like symptoms 7 days prior to his presentation. It was treated as an upper respiratory tract infection with minimal improvement. One week before his presentation, he returned to Qatar after a short vacation in Islamabad, Pakistan, with a history of eating local food from roadside stalls. He denied hiking, camping, tick bites, mosquito bites, exposure to lice, mites, animals, and their body fluids.
His father also reported that the patient had diarrhea and abdominal pain while in Pakistan, 10 days prior to his presentation, but without nausea or vomiting. The patient denied history of earache, ear discharge, tinnitus, recent upper respiratory tract infection, vertigo, and dizziness. He denied history of smoking, alcohol intake, and illicit drug use.
In the ED, he was febrile with an oral temperature of 38.9 C, pulse rate of 95 beats/min, respiratory rate of 20 breaths/min, and blood pressure of 115/67 mm Hg. Upon physical examination, the patient appeared anxious and disoriented to place. He also showed bilateral impaired hearing ability, raising his voice in order to communicate and struggling to hear questions from the medical team. Otherwise, systematic examination was normal.
Initial investigation showed mild thrombocytopenia with normal white blood cell count and hemoglobin level. He had mild elevation in creatinine at 162 umol/L (normal <100 umol/L), hyponatremia at 127 mmol/L (normal range 135-145 mmol/L), elevated serum alanine aminotransferase (ALT) at 134 U/L (normal <41 U/L), elevated serum aspartate aminotransferase (AST) at 240 U/L (normal <40 U/L), and elevated C-reactive protein (CRP) at 185 mg/L (normal <10 mg/L). Alkaline phosphatase (ALP) and total bilirubin were normal. Malaria screening was negative. Initial imaging with chest X-ray and head CT (computed tomography) were normal.
Acute meningitis with or without encephalitis was the top differential diagnosis; lumbar puncture (LP) failed in the ED due to the patient's agitation and non-cooperation. Intravenous normal saline and empirical antibiotics (ceftriaxone 2 g every 12 h, vancomycin 1 g/day, and acyclovir 750 mg every 8 h) were initiated after sending cultures and investigations for sepsis workup. Intravenous dexamethasone (initial loading dose of 3 mg/kg, then 1 mg/kg every 6 h) was also added to the regimen, then shifted to oral prednisolone (60 mg/day) after 2 days.
Pure tone audiometry (PTA) was performed at presentation and 15 days later in the outpatient clinic. The result from the left ear audiometry is shown in Figure 1. It showed moderate bilateral sensorineural hearing loss, to nearly all frequencies during his inpatient admission (shown in Figure 1(a)). PTA was repeated after 15 days and showed marked improvement with mild hearing loss only to high frequencies (shown in Figure 1(b)). Tympanometry and tone decay test were normal upon admission.
One day after admission, the patient's blood culture demonstrated growth of Salmonella Typhi. Due to the high prevalence of extensively drug-resistant (XDR) strains in Pakistan, antibiotics were changed to meropenem (2 g every 8 h) and azithromycin (500 mg/day) to ensure coverage of possible drug-resistant strains. According to the World Health Organization (WHO), 5274 (64.4%) cases of XDR typhoid fever were reported out of 8188 typhoid fever cases from 1 November 2016 to 9 December 2018 in Pakistan. The XDR strains of Salmonella Typhi have shown resistance to first- and second-line antibiotics (e.g. fluoroquinolones) as well as third-generation cephalosporins (e.g. ceftriaxone). Therefore, current treatment options for Pakistani XDR strains of Salmonella Typhi are limited to oral azithromycin, and intravenous meropenem or tigecycline.
Two days after admission, blood culture sensitivity results showed Salmonella Typhi sensitive to ceftriaxone but resistant to ciprofloxacin. Therefore, antibiotics were de-escalated to intravenous ceftriaxone only, to complete the total duration of 14 days of antibiotics. Head MRI (magnetic resonance imaging) was normal and fluoroscopic guided LP was performed; results showed high protein level without leukocytosis. Cerebrospinal fluid (CSF) culture and viral screen, including herpes simplex viruses, were negative. Workup for tuberculosis from sputum and CSF was negative. Urine analysis and microscopy were normal.
Clinically, the patient improved quite rapidly; fever subsided and hearing loss gradually improved 5 days after the treatment had commenced. The patient's hearing loss continued to improve and almost completely resolved by the end of the first month of treatment. Audiometry was not done after 1 month because the patient had traveled back to his home country and was thus contacted over the phone. Oral prednisolone was gradually tapered down from 60 mg/day and stopped within 3 weeks.
|
salmonella typhi, adult, atypical, hearing loss, typhoid fever
| Not supported with pagination yet
| null |
PMC5491826_01
|
Male
| 48
|
A 48-year-old Japanese man with a 2-month history of general fatigue developed right back pain and edema. He was admitted to a local hospital because of a high level of serum C-reactive protein (CRP) (28.9 mg/dL) and hypoalbuminemia (1.7 g/dL). Soon after admission, he developed a high fever, difficulty of breathing, and oliguria. A computed tomography (CT) scan showed right adrenal hemorrhaging and bilateral thickening of the Gerota's fascia. After treatment with antibiotics and intravenous hydrocortisone, he was referred to our hospital. At that time, the platelet (PLT) count was 61,000 /muL, serum albumin (Alb) 1.5 g/dL, creatinine (Cr) 1.16 mg/dL, estimated glomerular filtration rate (eGFR) 54.3 mL/min/1.73 m2, Na 131 mEq/L, CRP 38.6 mg/dL, IgG4 44.1 mg/dL, and PCT 8.09 ng/mL (normal range, <0.5). Anti-cardiolipin (CL) antibodies, anti-CL/beta2-glycoptotein 1 (beta2GP1) antibodies, lupus anticoagulants, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA), and proteinase 3 anti-neutrophil cytoplasmic antibodies (PR3-ANCA) were negative. Urine culture and repeated blood cultures were negative. A CT scan showed bilateral pleural effusion, hepatosplenomegaly, right adrenal hemorrhaging, and systemic lymphadenopathy (Fig. 1A-C). He was treated with antibiotics, intravenous steroids, Alb infusion, and diuretics. His symptoms gradually improved. One month later, he was referred to our department for further examinations.
At the presentation, his body temperature was 36.7C. A physical examination showed cervical lymph node swelling and edema. A urinalysis showed hematuria (50-99 red blood cells/high power field) without proteinuria. His white blood cell (WBC) count was 14,800 /muL, hemoglobin (Hb) 9.8 g/dL, PLT count 36,000 /muL, activated partial thromboplastin time (APTT) 29.5 sec, prothrombin time-international normalized ratio (PT-INR) 1.23, fibrinogen (Fib) 305.0 mg/dL, fibrinogen degradation product (FDP) 28.0 mug/mL, and D-dimer 12.52 mug/mL. Serum total protein (TP) was 5.1 g/dL, Alb 2.6 g/dL, blood urea nitrogen (BUN) 20.0 mg/dL, Cr 0.42 mg/dL, aspartate transaminase (AST) 89 U/L, alanine transaminase (ALT) 132 U/L, alkaline phosphatase (ALP) 684 U/L, lactate dehydrogenase (LDH) 226 U/L, gamma-glutamyl transpeptidase (gamma-GTP) 412 U/L, total bilirubin (T. Bil) l.4 mg/dL, Na 139 mEq/L, K 2.5 mEq/L, and Cl 89 mEq/L. Serum CRP was 0.85 mg/dL, antinuclear antibodies (ANA) <20, IgG 1,301 mg/dL, IgA 162 mg/dL, IgM 41 mg/dL, C3 118 mg/dL, C4 27 mg/dL, CH50 60 U/mL, and IL-6 3.8 pg/mL (normal range, <4.0). Serum and urinary protein electrophoresis showed no monoclonal paraproteins. Blood culture and urine culture were negative. There was no evidence of infections caused by hepatitis B virus (HBV), hepatitis C virus (HCV), cytomegalovirus (CMV), Epstein-Barr virus (EBV), human herpesvirus 8 (HHV-8), HIV, human T-lymphotropic virus (HTLV)-1, toxoplasma, Aspergillus species, or Mycobacterium tuberculosis.
A cervical lymph node biopsy showed atrophic germinal centers with reactive interfollicular plasma cell proliferation (Fig. 2A and B). Immunostaining for CD21 showed follicular dendritic cell proliferation (Fig. 2C). Bone marrow aspiration and a bone marrow biopsy revealed normocellular bone marrow with mild fibrosis without atypical cells (Fig. 2D). On the basis of these pathological findings and symptoms (thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and organomegaly), he was diagnosed with TAFRO syndrome.
On day 22 after admission to our department, oral prednisolone (PSL: 50 mg/day) therapy was started instead of intravenous steroids, and the dose of PSL was gradually reduced to 30 mg/day. On day 46 after admission, a CT scan showed that the pleural and abdominal fluids had disappeared, the adrenal hematoma had improved, and systemic lymphadenopathy was diminished. His PCT level had decreased to 0.56 ng/mL. He was discharged on day 67. At that time, the PLT count was 260,000 /muL, serum Alb 4.2 g/dL, Cr 0.51 mg/dL, and CRP 0.11 mg/dL.
|
castleman disease, tafro syndrome, adrenal hemorrhage, procalcitonin, rituximab
| Not supported with pagination yet
| null |
PMC7111179_01
|
Female
| 59
|
A 59-year-old female presented to our clinic in November 2011 for a second opinion regarding complaints of right foot and ankle pain. She stated that the pain had started approximately 1 year earlier and denied any trauma preceding the event. The patient described the pain as both aching and sharp, rating it as 8 of 10 on a visual analog scale, and that it was aggravated with standing and walking. Temporary immobilization in a below-the-knee boot and nonsteroidal anti-inflammatory drugs did not alleviate her symptoms. Physical examination revealed pain on palpation of the right anterior tibia just proximal to the ankle joint. She denied any pain with passive range of motion to her right ankle joint. Her neurovascular status was fully intact, with no signs of vascular disease to her lower extremities.
The patient had a remote history of ulcerative colitis that had been in remission for nearly 20 years. During the acute stage of the condition, she had been taking high doses of oral corticosteroids. The rest of her medical history and family history were unremarkable. She did not use any tobacco products, although she reported drinking alcohol occasionally. She took iron and vitamin D supplements. She also reported allergies to gluten and sulfa medications.
The previous surgeon she had consulted had ordered a magnetic resonance imaging study and diagnosed AVN of the bilateral distal tibias (Fig. 1 ). Subsequent magnetic resonance imaging studies showed ON in the left calcaneus and right humerus. Only her right tibia was symptomatic. For preoperative planning and to rule out any pathologic fractures, a computed tomography scan was ordered (Fig. 2 ).
The patient was placed on the operating room table in a supine position with a tourniquet on the right proximal thigh and the right leg placed in a thigh holder. After induction of general anesthesia, the patient's right foot was inserted into an ankle distractor, and the ankle was accessed through standard anteromedial and anterolateral portals. Arthroscopy revealed abundant hypertrophied synovitis in the lateral aspect of the ankle joint. On debridement of the synovitis, an osteochondral defect measuring approximately 5 mm in diameter was noted on the anteromedial shoulder (Fig. 3 ). This was subsequently micro-fractured in standard fashion until active bleeding was noted from the subchondral bone of the lesion. On extensive examination using arthroscopy, it was also noted that the tibial articular surface showed no signs of fracture or penetration into the ankle joint. The portal incisions were then closed with 4-0 monofilament suture (Prolene , Johnson & Johnson Medical Ltd, Livingston, UK).
An 8-cm liner incision was made along the anterior aspect of the distal tibia just lateral to the course of the anterior tibial tendon (Fig. 4 ). Dissection was carried down to expose the anterior aspect of the distal tibia, and the periosteum was reflected in a medial and lateral direction. A cortical window measuring approximately 3 cm in length and 2 cm in width was then carefully reflected using a sagittal saw (Fig. 5 ). Care was taken to bevel the cut so as to stabilize the cortical window. Once the cortical window was removed, a combination of rongeurs and curettes was used to remove all the necrotic bone. Once all the necrotic bone had been removed, which was noted to be quite spongy in consistency, an approximately 10-cm3 deficit was left to fill (Fig. 6 ). Specimens of the necrotic bone were sent for both culture and histopathologic examination for further evaluation.
The defect was filled with AlloStem Stem Cell Bone Growth Substitute (AlloSource, Centennial, CO), and geneX bone putty (Biocomposites Inc., Wilmington, NC; Fig. 7 ). Once this composite was dry, the cortical window was placed back into its original location and secured with a Synthes/Depuy Mesh Plate (Synthes, Inc., West Chester, PA) to act as a buttress plate. The advantage of this plate was that it can be easily cut and contoured to fit the size of the defect. The plate was secured with a combination of 2.7-mm unicortical and bicortical locking screws, with only 1 screw going directly through the cortical window (Fig. 8 ). Excellent stability was noted before the wound was closed. The wound was then copiously flushed and closed in standard fashion. The patient was placed into a non-weightbearing posterior splint and subsequently discharged home the next day after staying in the hospital for a 23-hour observation period.
The patient was maintained non-weightbearing in the posterior splint for 2 weeks and then transitioned to a removable cast boot for an additional 5 weeks. While in the removable cast boot, the patient was instructed to begin range of motion exercises of her ankle. At 7 weeks, the patient began protective weightbearing in the removable cast boot and started physical therapy. At 9 weeks, the patient began to wean herself out of the removable cast boot into normal shoe gear. Radiographs and computed tomography scanning showed excellent consolidation of the defect with native bone incorporation (Fig. 9 ). The patient began light exercise activity at 11 weeks and returned to work. The cultures and pathology specimens confirmed the diagnosis of ON with no infection present (Fig. 10 ). The patient was subsequently discharged from care at 16 weeks postoperatively, with minimal swelling and no pain. She was instructed to continue with physical therapy to improve her ankle range of motion. The patient was interviewed by telephone at 24 months postoperatively and reported she was doing well with no pain in the right leg or ankle. Final radiographs were also taken at that time and showed excellent deficit incorporation with native bone visible (Fig. 11 ).
|
alcohol, ankle, avascular necrosis, corticosteroids, diaphyseal metaphyseal bone
| Not supported with pagination yet
| null |
PMC6126086_01
|
Male
| 56
|
A 56-year-old healthy man presented to the emergency department in the summer season with three days of fatigue and bilateral thigh pain. He was born in Puerto Rico but resided in the Northeast Region of the US, where he worked as a chef in a major metropolitan city. He had no sick contacts, recent travel, or alcohol or drug use.
Laboratory data on presentation demonstrated a creatinine (Cr) of 1.73 mg/dL, creatinine kinase (CK) of 3494 U/L and platelet count of 68x103/muL with initially normal liver function tests (LFTs). The patient was admitted for treatment of acute kidney injury from presumed rhabdomyolysis of unclear cause but subsequently developed low-grade fevers, leukocytosis, and worsening thrombocytopenia over the following days. His Cr worsened despite hydration and conservative management for which the patient underwent a renal biopsy on hospital day 4, with findings of acute tubular necrosis, interstitial hemorrhage, and capillaritis.
In addition to worsening renal function, he had an impressively rapid rise in his total and direct bilirubin with development of clinical jaundice over the subsequent days with laboratory values on hospital day 8 as follows: Cr of 4, total bilirubin of 41 mg/dL, and direct bilirubin of 38 mg/dL (Figure 1(a)). The GI consult service became involved in his care and on physical examination noted no evidence of chronic liver disease other than jaundice. The patient had no abdominal tenderness, hepatosplenomegaly, or asterixis. The bilirubin values were out of proportion to his other liver tests such as INR and albumin, which remained within normal values and AST/ALT and alkaline phosphatase values wavered between mildly elevated (<2 times the upper limit of normal) and normal values. Based on the kidney biopsy results and significant hyperbilirubinemia, testing was done for bacteremia, influenza, tuberculosis, HIV, tick-borne diseases, Hantavirus infection, acute viral hepatitis (A, B, C, E, CMV, EBV, and VZV) and vasculitis, which were all negative or normal.
An abdominal ultrasound and MRI liver protocol/MCRP showed a normal hepatobiliary system. Given his impressive rise in bilirubin out of proportion to other LFTs in combination with renal failure and rhabdomyolysis, the GI service recommended antibody testing for leptospirosis, for which serum IgM antibodies were checked on hospital day 5. The following day, he was started on empiric doxycycline in liaison with infectious disease consultation at 100 mg intravenously twice daily. In the interim, a liver biopsy was done showing liver parenchyma with marked canalicular and intracellular cholestasis, accentuated in perivenular zone, and rare foci of bile duct injury and ductular proliferation. There was no evidence of significant steatosis, fibrosis or intracellular iron deposition with trichrome, reticulin, PAS-D, and iron stains being unrevealing.
On hospital day 10, the Leptospira IgM returned positive, consistent with the diagnosis of icteric leptospirosis. He was continued on doxycycline 100 mg twice daily with subsequent normalization of leukocyte and platelet counts (Figure 1(b)). In view of the positive Leptospira IgM antibody results, the liver tissue obtained from biopsy was reevaluated for spirochete organisms with special staining; however no organisms were found.
The patient completed a 10-day course of doxycycline but unfortunately suffered from bile cast nephropathy from severe hyperbilirubinemia with continued rise in Cr (Figure 1(a)), for which he was treated with cholestyramine and ursodiol. His liver function tests and kidney laboratory results began to improve thereafter, and he was discharged on hospital day 26. At six-week follow-up, his renal function improved (Cr of 1.4 mg/dL) and his LFTs had normalized. It was later discovered that the restaurant where the patient had been working had a rodent infestation, which were the most likely source of his infection.
| null | Not supported with pagination yet
| null |
Subsets and Splits
No community queries yet
The top public SQL queries from the community will appear here once available.