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pmc-6507145-1 | A 6-year old domestic huacaya alpaca non-pregnant mare with a body mass of 53 kg, was referred to the Clinic for Ruminants, Vetsuisse-Faculty, University of Bern, Switzerland for further diagnostic investigation. For 6 weeks, the mare had shown respiratory symptoms such as forced breathing but with no fever. Although the mare always had a good appetite, significant weight loss occurred during this time. A treatment by the referring veterinarian did not improve the symptoms.
At clinical examination, the alpaca was alert, nervous and in poor general condition. Body condition was moderate (bodyweight 53 kg: reference range55-90 kg) with a body condition score of 1 out of 5 (body condition score of the Australian Alpaca Association []). Its rectal temperature was 38.3 °C (reference range: 37.5–38.9 °C), heart rate 72 beats per minute (reference range: 60-80beats per minute) and respiratory rate 60 breaths per minute (reference range: 10–30 breaths per minute). The mare showed dyspnea with an abdominally reinforced breathing and bilateral dilated nostrils. There was no spontaneous nor provoked cough or evidence of nasal discharge. Auscultation of its lungs revealed bilateral ventrally scratching and crackling sounds, on the left dorsal aspect respiration sounds were focally absent. Further clinical examination showed no abnormalities, the alpaca had a good appetite once back in the stable, although dyspnea was still present.
Clinicopathological tests included a complete blood cell count and a blood chemistry panel. A neutrophilia (9.37 × 109/l, reference interval: 3.4–9.1 × 109/l) with left shift was present. Blood chemistry showed low potassium (3.64 mmol/l, reference interval: 4–5.2 mmol/l) and magnesium (0.71 mmol/l, reference interval: 0.8–1.1 mmol/l) values. Parasitological examination of a fecal sample revealed no endoparasite eggs or lungworm larvae.
Ultrasonographic examination (Esaote piemedical®, 10mHz linear probe) of the lungs revealed on the day of admission several comet-tail-artefacts over the whole lung field. The left dorsal lung surface was focally retracted and a moderate amount of gas was visualized in the pleural space. An area of increased density of the lung parenchyma was visible. There was no evidence of pleural effusion.
Laterolateral radiographs of the lungs were taken on the day of admission (Fig. ) and four days after admission (Fig. ). On both occasions, a bilateral pneumothorax with retracted lung lobes, mild on one and moderate on the other side, was diagnosed. Due to a mild retraction of lung lobes in the cardiophrenic angle in combination with focal effacement of the contours of the diaphragm and the cardiac silhouette, a very mild amount of pleural effusion was suspected. A focal lesion (17x22mm) located dorsally in the less retracted caudal lung lobe was observed with ill-defined borders, associated with a focal indentation of the lung surface. In the dorsal part of the lung field, several small gas lucent (6-15 mm diameter) lesions without a wall were suspected. In the central parts of the lung field, the opacity was diffusely increased with an ill-defined mixed reticular and bronchial pattern. However, it remained unclear, if this represented a pathology or was a consequence of partially collapsed lungs due to the pneumothorax. In the follow-up radiographs four days after admission, stripy increased opacities were observed in the cardiophrenic angle (Fig. ).
Differential diagnosis included neoplasia, fungal granulomas or tuberculosis. To obtain a better characterization of the lung pathology, a computed tomography scan(CT-scan) of the lungs in general anesthesia was performed.
The CT-scan revealed multiple focal bronchocentric part-solid spiculate nodules with internal air bronchograms in all lung lobes. The pneumothorax was confirmed. [Figures. and ].
Based on history, physical examination, available laboratory results and diagnostic imaging the main differential diagnosis was a pulmonary neoplasia. To distinguish between neoplasia and the other differentials as fungal granulomas or infectious agents (i.e. tuberculosis), the option of performing a fine needle aspiration or a biopsy was discussed.
Over 4 days of hospitalization, the general state and respiratory distress of the animal worsened progressively. Therapeutic options like undertaking pleural drainage to restore the pneumothorax were also discussed, but due to the poor prognosis, the owners elected for euthanasia of the alpaca. The animal was euthanized with an intravenous injection of pentobarbital (Esconarkon ad us.vet., Streuli Pharma AG, Uznach, Switzerland) in a dosage of 150 mg/kg bodyweight.
The postmortem examination, revealed the following findings: Affecting the cranial and caudal lung lobes there were multiple, approximately 1 cm × 0.5 cm × 0.5 cm, nodular, poorly demarcated, non-encapsulated, whitish to yellowish masses. No extra-pulmonary involvement was observed. There were multifocal areas of bullous emphysema within dorsally parts of the right and left cranial lobes. Other remarkable gross findings were multifocal, 1 cm in diameter nodular, centrally mineralized parenchymal masses in the liver and multifocal mucosal ulceration within compartment 3 of the forestomaches. The other examined organs were macroscopically unremarkable and no pathological alteration could be detected.
Samples of the lung and liver were fixed in 10% neutral buffered formalin for histological examination. The samples were routinely processed; paraffin wax embedded, stained with hematoxylin and eosin (HE), sections of 3 μm were cut and observed by standard light microscopy for histological examination.
Microscopically, the pulmonary nodules consisted of unencapsulated, poorly demarcated and infiltrative growing neoplastic masses. The epithelial neoplastic masses showed lepidic growth along pre-existing alveolar walls, which was cuboidal to low columnar with mostly distinguished cell borders, scant to moderate amounts of eosinophilic cytoplasm, mostly basally placed, round nuclei with coarse-stippled chromatin pattern and up to 1 nucleoli. The stroma separating the neoplastic acinar structures was abundant and poorly cellular consisting of plump spindle cells and extracellular deposition of collagen. Occasionally, there were areas of squamous epithelial cells differentiation with central keratin pearls and no apparent acinar formation [Fig. ]. Mitoses were very rare. Multifocal small areas of necrosis were present.The adjacent lung parenchyma showed a diffuse thickening of the alveolar septum characterized by infiltration with small numbers of lymphocytes, plasma cells and scattered macrophages. Alveolar spaces were emphysematous or multifocal filled with small numbers of foamy macrophages and few neutrophils.
The liver lesions were histologically compatible with a chronic, granulomatous and eosinophilic cholangiohepatitis. No trematodes could be observed within the examined sections and Ziehl-Neelsen-stain was negative. There was no evidence of fungal organism in the histopathological examination of liver and lung samples. |
pmc-6507154-1 | A 24-year-old African American woman presented to the emergency department (ED) with several months of recurrent nausea, flank pain, and hematuria. She previously sought care 2 weeks prior and was told she had a diagnosis of urinary tract infection (UTI) and was empirically treated with oral ciprofloxacin. However, a urine culture did not grow any organisms, and symptoms persisted despite completing a course of antibiotics. Her past medical history included UTIs for which she received empiric antibiotic treatment, although all urine cultures in her medical chart repeatedly showed no growth of organisms. She was not taking medications. Family history was not significant for any chronic medical illnesses. She smoked cigarettes but denied consuming alcohol or using illicit drugs. On presentation, vital signs were stable and physical examination was significant for bilateral flank pain.
Urinalysis showed red urine, 3 + protein, 3 + blood, 656 red blood cells (RBCs) per high-power field (HPF), 41 white blood cells (WBCs) per HPF, negative nitrites, negative leukocyte esterase, and red cell casts. Blood urea nitrogen (BUN) and creatinine (Cr) were 41 mg/dL and 5.6 mg/dL, respectively, and the glomerular filtration rate (GFR) was 11 mL/min/1.73 m2. Urinary protein was 116 mg/24 hours with a fractional excretion of sodium (FENa) of 2.8%. A renal ultrasound demonstrated increased echogenicity of both kidneys suggestive of renal disease. By day 3, creatinine was 6.45 mg/dL and GFR was 10 mL/min/1.73 m2. Our patient started hemodialysis.
Laboratory studies included an antinuclear antibody (ANA), rheumatoid factor (RF), complement C3 and C4, human immunodeficiency virus 1 and 2 (HIV 1 and 2) antibody, antineutrophil cytoplasmic antibodies (ANCA), anti-double-stranded DNA (anti-dsDNA) antibodies, and anti-GBM antibodies. All were negative except for anti-GBM antibodies which reported positive 1 week later with a titer level of 1.7 (<1.0 is normal). In the interim, a renal biopsy was performed due to rapidly declining renal function. Light microscopy showed necrotizing and crescentic glomerulonephritis with fibrocellular crescent formation (). Electron microscopy demonstrated widespread podocyte foot process effacement and linear deposits along the glomerular basement membrane () confirmed as IgG by immunofluorescence (). A diagnosis of anti-GBM crescentic glomerulonephritis was made.
Before the confirmatory diagnosis was made by renal biopsy, on day 1 of hospital admission, our patient was treated with methylprednisolone 1 g IV daily for 3 days and then prednisone 40 mg by mouth daily for 1 month. Once the renal biopsy confirmed anti-GBM crescentic glomerulonephritis along with serologies positive for anti-GBM antibody, intravenous cyclophosphamide and plasma exchange were both initiated on day 9 of hospitalization. She received cyclophosphamide 1 g IV every 2 weeks for 3 occurrences. She received plasma exchange daily for 7 days then every other day for 3 occurrences. Plasma exchange was stopped once repeat testing for anti-GBM antibodies was undetectable on day 23. Due to rapidly progressing renal failure, she required initiation of hemodialysis on day 4 of admission and continued to require hemodialysis daily throughout her hospital stay. Our patient was discharged to home on day 28 with outpatient nephrology follow-up and hemodialysis three times a week. Anti-GBM antibody levels remained undetectable. Kidney transplant evaluation was initiated.
Eight months later, our patient presented to the ED with dyspnea and hemoptysis. She last received dialysis the day before admission and had not missed any dialysis sessions. Social history was notable for marijuana use daily for a week prior to admission. She appeared diaphoretic and drowsy with decreasing oxygen saturation despite supplemental oxygen. Physical examination was significant for tachycardia, tachypnea, reduced air movement in all lung fields, and dullness to percussion of the thorax. A portable chest radiograph showed diffuse bilateral patchy opacities (). A computerized tomography angiography (CTA) of the chest with IV contrast confirmed diffuse bilateral consolidations (). Our patient required tracheal intubation and mechanical ventilation with admission to the intensive care unit (ICU).
Our patient had a hemoglobin level of 6.4 g/dL, hematocrit of 21%, and MCV of 95 fL which dropped to as low as a hemoglobin and hematocrit of 5.6 g/dL and 18%, respectively. Three weeks prior to admission, hemoglobin was 8.7 g/dL and hematocrit was 26%. Flexible fiberoptic bronchoscopy was performed, and the bronchoalveolar lavage (BAL) fluid was consistent with alveolar hemorrhage (). The lavage fluid was negative for malignant cells, bacterial, viral, or fungal organisms.
Repeat ANA, ANCA, and anti-dsDNA remained negative. Unlike on initial presentation, repeat anti-GBM antibody levels were undetectable, which did not result until day 7 of the hospital stay. Given our patient's history, a relapse of Goodpasture syndrome with pulmonary hemorrhage was diagnosed. While the most likely diagnosis was a relapse of Goodpasture syndrome, acute heart failure was also included in the differential diagnosis. However, a transthoracic echocardiogram demonstrated a normal ejection fraction of 55–60%, normal left ventricular diastolic function, normal right ventricular systolic and diastolic function, mild tricuspid valve regurgitation with no other valvular abnormalities, an elevated pulmonary artery pressure of 50 mmHg, and a normal central venous pressure. These findings were not suggestive of heart failure; rather, functioning of both left and right ventricles was intact, while the elevated pulmonary artery pressure was suggestive of a primary intrapulmonary process (pulmonary hemorrhage). Starting on day 1, she was treated with methylprednisolone 1 g daily for 5 days, and then changed to prednisone 40 mg daily. Plasma exchange was also initiated on day 1 which she received daily for 5 occurrences. She was treated with mycophenolate 500 mg IV every 12 hours for 5 days. She progressively improved clinically with resolved alveolar hemorrhage and was extubated on day 5. She was transferred to the medical ward in stable condition. On day 10, she was discharged to home with outpatient nephrology follow-up and continued dialysis.
Our patient was stable following her admission for Goodpasture syndrome relapse. She continued to have outpatient hemodialysis 3 times a week. She did not require outpatient oral steroids or immunosuppression. However, 9 months later, we received the news that she had passed away from a drug overdose. |
pmc-6507177-1 | A previously healthy 35-year-old Sri Lankan man presented with high-grade intermittent fever for 3 days with constitutional symptoms. He had spontaneous gum bleeding with no other overt bleeding manifestations. He had associated intermittent frontal headache of moderate severity at presentation and subsequently developed gradually worsening drowsiness. Rest of the history including his past medical and family histories were unremarkable. In particular, he did not have diabetes mellitus, history of recurrent infections, unprotected sexual contact or recreational drug abuse.
On examination, his body temperature was 100 °F and his Glasgow coma scale score was 10/15. He was mildly pale. There was no neck stiffness. Fundoscopic examination was normal. There were no focal neurological signs. His pulse rate was 100 bpm and blood pressure was 130/80 mmHg, Rest of the cardiovascular, respiratory and abdominal examinations were normal.
His full blood count showed a white cell count of 9.2 × 103/dl (Normal Range (NR) 4–11 × 103) with neutrophil predominance (77%). Haemoglobin was 6.9 g/dl (NR 11–15) and platelet count on admission was 7 × 103 (NR150–400 × 103). His coagulation profile was normal with prothrombin time of 12.8 s (NR 10–13) and APTT 30 s (NR 26–40). Serum creatinine was slightly elevated at 137 mmol/l (NR 60–120 umol/L) and the electrolytes were normal. There was indirect hyperbilirubinaemia with total bilirubin of 44.7 μmol/l (NR 1.7–20.5) and direct bilirubin of 7.3 μmol/l (NR 1.7–5.1). The serum lactate dehydrogenase level was 3115 U/l (NR 160–450). Direct coombs test and dengue serology were negative. Non-contrast CT scan of the brain was normal. Blood picture showed evidence of severe thrombocytopenia with microangiopathic haemolytic anaemia (MAHA).
A diagnosis of TTP was made and he was promptly commenced on therapeutic plasma exchange and 1 mg/kg of oral prednisolone. However, he developed two episodes of generalized tonic-clonic convulsions which progressed in to a non-convulsive status epilepticus on the fifth day of illness, which required elective ventilation in the intensive care unit. On day 18, he developed flaccid paraparesis with sphincter dysfunction. Magnetic resonance imaging showed haemorrhage at multiple spinal levels including cervical spine sub-arachnoid space, anterior epidural space and intra-thecal space from T10 to L3 vertebral levels, and in the the region of the conus medullaris.
Surgical drainage of the spinal haematoma was considered hazardous. At this point his platelet count was 85 × 103 and remained < 100 × 103 with MAHA persisting despite 16 plasma exchanges and high dose steroids.
Initially, the refractoriness of the TTP was attributed to ventilator associated pneumonia. However, since successful treatment of sepsis did not improve the MAHA, an alternative aetiology was investigated. Polymerase chain reaction of serum revealed 2100 IU/ml copies of CMV (Reference laboratory cutoff value more than 640 IU/ml was considered clinically significant). CMV DNA was quantitatively determined by RealStar® CMV PCR Kit 1.0 (Altona diagnostics). Screening tests for autoimmune diseases, other chronic infections and immunodeficiency, which included ANA, serum complement levels, serum immunoglobulin levels, HIV 1, HIV 2, VDRL, hepatitis screen and HbA1c were negative.
The patient was commenced on oral valganciclovir 450 mg/daily and continued for 21 days. After about 6 days of valganciclovir treatment his platelet count increased to 198 × 103/cumm and the MAHA resolved. After resolution of the TTP, the patient was transferred to a rehabilitation facility for further care. At three months’ review, he had normal haematological and biochemical parameters and a negative PCR quantification of CMV. He had regained ability to walk with support and had normal sphincter function. |
pmc-6507206-1 | The 9 year-old boy from Southeast China came to Airforce Medical Center of PLA early this year, who was diagnosed of PJS in a local hospital. Multiple MPs on the lips and cheeks were observed by his families shortly after his birth, and this phenomenon did not draw their attention since this family did not have a history of PJS. At the age of seven, the boy got paroxysmal abdominal cramps after meal and fresh blood came out with stool. He was soon sent to the local hospital, and colonoscopy revealed multiple colon polyps. Then endoscopic polypectomy was performed, and pathological exam confirmed them hamartomas. The symptoms relieved largely after colonoscopy. Taking pigmentation and GI hamartomas together, the diagnosis of PJS was confirmed.
One year later, the similar symptoms appeared again, and this time doctors in the local hospital used capsule endoscopy, during which a large polyp with the diameter of 5 cm in the ileum was detected. After expectant treatment, the patient was referred to our department for further treatment. Physical examination confirmed the MPs and revealed no other PJS related findings (testicular tumors included). We arranged enteroscopy for him after his admission, and the large polyp whose diameter was actually 2.5 cm together with another smaller one within the ileum was resected successfully (Fig. a and c). Postoperational pathology reported the PJS-related hamartomas which showed the classical arborizing smooth muscle, consistently with previous results (Fig. b).
During the boy’s remedy, we collected the blood samples of his and his parents after their signing informed consents forms (ICF). In the laboratory, the genomic DNA was extracted from their peripheral blood leucocytes using animal genomic DNA kit (TSP201, TsingKe Biotech). Polymerase chain reactions (PCR) were performed by using modified DNA polymerase mix (TSE004, TsingKe Biotech), and the coding sequence and boundaries of STK11 gene were analyzed by DNA sequencing essentially as previously described [, ].
Fifty unrelated control individuals who came to our department for gastric polyps treatment were also screened for the presence of the mutation to rule out polymorphisms. All biological samples were collected after written ICF being acquired.
Sanger sequencing of STK11 gene revealed a heterozygous germline deletion c.243delG in exon 1 in the patient’s genomic DNA (Fig. d and f), while it was not detected in the parents’ or the control samples’. This mutation has not been documented in literatures or mutation databases such as dbSNP, OMIM, ClinVar, HGMD or ExAC (). It generates a translational frameshift and a premature stop (p.K81Kfs*15), resulting in a large-scale loss of kinase domain and total loss of the C-terminal domain in comparison to the wild type (Fig. e).
Swiss-Model online software (), a homology modeling program [], showed the simulated mutant protein turns into an aberrant shape with significantly smaller size (Fig. h). Evolutionary conservation was analyzed by comparing across different species (), and the results showed these missing amino acid residues were most conserved (Fig. g).
Generally, this mutation is believed to be a novel pathogenic one in STK11 leading to PJS in line with American College of Medical Genetics and Genomics (ACMG) classification system (Table ). |
pmc-6507247-1 | A 78-year-old, Caucasian female patient with metastatic breast cancer under chemotherapy was presented to the emergency department reporting fever, groin pain, vomiting (over 10 times per day) and being unable to get up of the bed for the past four days after the last chemotherapy. The initial laboratory workup revealed increased serum creatinine level of 3.20 mg/dl (GFR=14.89 mL/min/1.73m2, baseline= 0.57-1.11). Moreover, the urine analysis showed increased pyocytes (>100), while the urine cultivation highlighted E. coli with sensitivity to piperacillin/tazobactam.
She was started on intravenous piperacillin/tazobactam (4.5g x 4, due to the impairment of renal function serum creatinine= 1.44 mg/dl) for urinary tract infection and at the same time she was kept hydrated. She had no known drug allergies and no history of psoriasis. She was clinically improved after two days of antibiotic therapy. On the fourth day of the intravenous piperacillin/tazobactam administration protocol, she abruptly developed extensive erythema and pustules that were located predominantly on the folds and anterior proximal thighs (Figures and ). The Nikolsky sign was negative and there was no mucosal involvement. The rash was accompanied by fever (38.5°C) and mild pruritus. The blood tests showed significant leukocytosis neutrophilia. Impressively, the white blood cells increased from 5.26 K/μl (with neutrophils 3.5 K/μl) to 39.6 x 103 K/μl (with neutrophils 27.7 K/μl) within two days of developing the rash. The patient refused to undergo a skin biopsy. However, Tzanck smear showed mainly neutrophils accompanied by the presence of eosinophils and lymphocytes, without any bacterial cocci. Moreover, bacterial culture from pustule content was negative.
Based on the clinical and laboratory results, it was concluded that the patient presented AGEP. Moreover, based on the AGEP validation score of the EuroSCAR study group [], our patient's score was 9, which is compatible with a definite diagnosis of AGEP. Piperacillin/tazobactam administration was interrupted (Day 4) and the patient was given 30 mg of prednisolone intravenously once a day, which was tapered and stopped within 10 days. Both the skin rash resolved, followed by postpustular desquamation, and the white blood cells returned to their normal levels two weeks after the discontinuation of the drug. Based on the WHO-UMC causality categories, association of the described side effect to the culprit drug could be characterized as “probable/likely.” The limitation of this work was the fact that there was no systemic rechallenge. |
pmc-6507249-1 | A 70-year-old male presented with hematuria and bruising of arms and legs for the last three days. He complained of unintentional weight loss of 40 pounds over the last four months. He also noted to have urinary frequency and hesitancy for four months. He denied nocturia, urinary dribbling, dysuria, or sensation of incomplete emptying of the bladder. He denied fever, chills, nausea, vomiting, abdominal pain, bowel complaints, or prior history of bleeding. He denied use of any blood thinners or nonsteroidal anti-inflammatory medications (NSAIDS).
He had past medical history of diabetes mellitus type 2 complicated by erectile dysfunction and hyperlipidemia. He had past surgical history of abdominal hernia repair. He denied smoking, alcohol, or recreational drug use. His medications included glipizide, metformin, tadafil, and atorvastatin. He denied family history of bleeding disorders or cancer.
Physical examination revealed an obese male patient in no acute distress. His vitals were within normal limits. Oral mucosa was moist. No lymphadenopathy was noted on examination. Lungs were clear to auscultation bilaterally. Heart sounds, rate, and rhythm were regular. The abdomen was soft, nontender, and nondistended with no hepatosplenomegaly. Cranial nerves 2-12 were grossly intact. Large ecchymoses measuring 3 × 3 cm on the anterior aspect of the right arm and 7 × 5 cm on the posterior aspect of the right lower leg were present. No rash or joint swelling was noted.
On admission, complete blood count (CBC) revealed a hemoglobin (Hb) level of 8.4 g/dl, white blood cell (WBC) count of 8,170/nl, and platelet count of 88 × 103/μl. The peripheral smear showed moderate red cell anisocytosis with few teardrop cells and rare schistocytes. Few giant platelets were noted. WBC were morphologically normal. Further workup showed PT of 25.1 seconds, INR of 2.5, APTT of 43.9 seconds, fibrinogen of 60 mg/dl, and FDP of more than 20 μg/ml (). The impression was that the patient was in DIC. On admission, he was transfused FFP and cryoglobulin. On day 2 after initial presentation, his INR improved to 1.60 and fibrinogen to 114 mg/dl which remained stabilized on subsequent days without further transfusions ().
A search was initiated to determine an underlying etiology precipitating DIC. Based on the history of urinary complaints and unintentional weight loss, PSA was ordered. His PSA was elevated at 942 μg/dl. CT of the abdomen and pelvis without contrast showed a significantly enlarged prostate with mass effect on the bladder base, mild left side hydroureteronephrosis to the level of the left ureterovesical junction, and numerous enlarged pelvic lymph nodes. A bone scan of the whole body showed increased sclerosis of the L3 vertebral body suggesting osseous lesions. Based on the above findings, there was a concern for metastatic prostate cancer driving DIC.
On day 5 after the initial presentation, the patient was started on ADT consisting of bicalutamide 50 mg daily and leuprolide 7.5 mg subcutaneously on monthly basis as an inpatient. At this time, the tissue biopsy was pending. He refused a prostate biopsy. Upon further discussion, he agreed for a bone marrow biopsy. It showed a hypercellular marrow due to metastatic infiltrate comprising 40% of marrow cellularity (Figures –). Residual hematopoiesis was present but decreased. Islands of erythroid precursors appeared adequate. Megakaryocytes showed some clustering with increased atypical forms. Immunohistochemical stains showed neoplastic cells dimly positive for PSA and prostate-specific membrane antigen (PSMA). The findings were consistent with metastatic prostate adenocarcinoma. By day 6 of the initial presentation, hematuria was resolved. He had no other signs of active bleeding. His hemoglobin was stable at around 8 g/dl. DIC labs were stable (). He was thus discharged from the hospital on ADT. The plan was to closely follow-up with his oncologist and to start chemotherapy as an outpatient.
On day 11 after the initial presentation, the patient was seen in the oncologist's office. He denied any bleeding episodes or new bruising. Lab work showed PT of 15 seconds, INR of 1.1, APTT of 26.6 seconds, fibrinogen of 151 mg/dl, and platelets of 161 × 103/μl. The oncologist had an extensive discussion with the patient about the risks and benefits to start chemotherapy with docetaxel along with ADT. But the patient was hesitant to undergo chemotherapy. So, he was continued on ADT only. He was instructed to follow-up in one month with a plan to recheck PSA and DIC labs at that time. Subsequently, he was then lost to follow-up with the oncologist.
Six months after the initial presentation, he was readmitted to our hospital with hematuria for one week. He denied bruising or bleeding from any other sites. On readmission, blood work showed PT of 22.5 seconds, INR of 2.0, APTT of 52.6 seconds, fibrinogen of 98 mg/dl, and FDP of more than 20 μg/ml (). CBC showed Hb of 8.0 g/dl and platelet count of 63 × 103/μl. PSA was repeated which was 1,970 μg/dl. His testosterone level was 118 ng/dl. At this time, he was off ADT for 5 months. The impression was reactivation of DIC secondary to metastatic prostate cancer with medication noncompliance.
During this time, the oncologist had repeat discussion with the patient about the necessity to start chemotherapy, but he refused. So, he was restarted on ADT. However, his hematuria was worsening. During the hospital course, his Hb was gradually trending down with the lowest value of 6.3 g/dl. Also, on day 10 after second readmission, his DIC labs started worsening (). He was supported with multiple packed red cells, platelets, FFP, and cryoprecipitate transfusions. Due to concern of worsening DIC with ADT, the plan was to start inpatient chemotherapy with docetaxel for more cytoreduction of prostate cancer (patient agreed after rediscussion). On day 12 after second readmission, he was started on docetaxel 60 mg/m2 every 3 weeks (typical dose of docetaxel is 75 mg/m2 which was reduced by 20% secondary to thrombocytopenia). By day 15 after second readmission, his DIC panel showed consistent improvement except persistent thrombocytopenia. Hematuria was resolved. Thrombocytopenia was attributed to chemotherapy-related side effect. Accordingly, the dose of docetaxel was adjusted. He was deemed stable for discharge with close outpatient follow-up with oncology.
As of the writing of this case, the patient has completed 2 cycles of docetaxel with ADT. After 2 cycles of chemotherapy, repeat PSA trended down to 1,070 μg/dl. The testosterone level was 12.5 ng/dl. DIC panel was within normal limits. |
pmc-6507253-1 | An 11-year-old, 6.6 kg, female spayed Maltese, presented comatose to the University of Queensland Veterinary Teaching Hospital within 30 minutes of blunt force trauma after being hit by a car. The dog was previously well with no current medications. Initial physical examination revealed the dog to be laterally recumbent and comatose, with bilateral pin-point pupils and an absent menace response. The oral mucous membranes were cyanotic and the dog rapidly progressed to respiratory arrest. The heart rate was initially 60 beats per minute (bpm). Unfortunately, a blood pressure reading was not recorded at this time. There was haemorrhage from the mouth with trauma evident to the oral mucosa.
A venous blood gas performed at presentation showed a mixed acidosis (pH 6.97 [reference interval: 7.35-7.44], lactate 12.4 mmol/L [reference: <2 mmol/L], pCO2 44 mmHg [reference interval: 33.6-41.2 mmHg]), and hyperglycaemia of 24.8 mmol/L [reference interval: 3.3-6.8 mmnol/L]. The sNa was in the normal range at 138 mmol/L [reference interval: 135-153 mmol/L].
An intravenous (IV) catheter was placed and 3 mg alphaxalone was given IV to permit intubation and manual intermittent positive pressure ventilation with 100% oxygen. A 20 ml/kg IV bolus of lactated Ringer's solution (LRS) was administered, followed by a 4 ml/kg bolus of 7% hypertonic saline (HTS) and an infusion of 0.5g/kg of mannitol over 20 minutes. LRS was subsequently continued at approximately 10 ml/kg/hr for one hour and then reduced to 5 ml/kg/hr. Analgesia was provided with fentanyl at 2 ug/kg IV bolus for three sequential boluses, followed by a constant rate infusion (CRI) at 4 ug/kg/hr.
Once spontaneous ventilation was noted, extubation was achieved and treatment continued in an oxygen cage. Neurological status at this time was improving, however, the dog was still obtunded with ongoing bilaterally miotic pupils and an absent menace response. The heart rate had increased to 116 bpm with strong femoral pulses and a systolic blood pressure of 180 mm Hg as measured by doppler. The respiratory rate was 60 breaths per minute with bilaterally harsh lung sounds in all fields. Chest radiographs were performed which demonstrated changes consistent with pulmonary contusions in the right cranial and left caudal lung fields. The dog was assessed as having a TBI with pulmonary contusions.
Approximately two hours after presentation and following initial treatment, a repeat venous blood gas showed the lactate had decreased to near normal at 2.8 mmol/L, with a pH of 7.34, and the hyperglycaemia had resolved (glucose 6.4 mmol/L). The dog's neurological status improved slightly, though some agitation was subsequently noted. A 0.01 mg/kg IV bolus of acepromazine was administered to address this. Five and a half hours after presentation, a venous gas showed a markedly increased sNa of 159 mmol/L (). Other blood work remained unremarkable.
The following morning, fourteen hours following presentation the sNa was measured to be 162 mmol/L. A free water deficit of 480 ml was calculated, and her IV fluids were changed to five percent dextrose in water (D5W) at 40 ml/hr, concurrently with LRS at 25 ml/hr. The goal was to replace the free water deficit over 12 to 24 hours with a decrease of sNa of 0.5 mmol/L/hour. Despite this, 24 hours after presentation and 12 hours following free water commencement, the sNa had only decreased by 4 mmol/L to 158 mmol/L. The urine specific gravity (USG) was 1.012 concomitantly ().
The dog was noted to have improved consciousness, but was intermittently vocalising. A head tilt to the right and rolling to the left when handled was also noted. The menace response remained bilaterally absent and there was absent conscious proprioception in both forelimbs and delayed in the hindlimbs. Withdrawal was present in all limbs. The dog was noted to be urinating large volumes frequently and refused to eat or drink. Harsh lung sounds were evident bilaterally, with an SpO2 of 94% on room air and 98% whilst receiving cage oxygen. Due to ongoing vocalisation, phenobarbitone at 2 mg/kg slow IV was commenced every 12 hours for sedation.
Approximately forty hours after presentation, the dog's mentation had markedly improved as evidenced by reduced anxiety and vocalisation. The dog was responsive to sound and was able to ambulate with assistance. Oxygen supplementation was discontinued as pulmonary function had normalised, and the fentanyl CRI was reduced to 2 ug/kg/hr. Nutritional support was initiated at this time in the form of Hills a/d slurry via syringe with a total volume of 10 ml given on this day.
Despite ongoing parenteral free water supplementation, the sNa remained elevated at 157 mmol/L, with a USG of 1.005. This poor response to free water supplementation prompted desmopressin (DDAVP) administration, one drop of 4 μg/ml solution into the conjunctival sac. Free water administration continued unaltered. Measurement of sNa 12 hours later revealed no change, remaining steady at 157 mmol/L. Therefore, a second dose of one drop DDAVP was administered conjunctivally. Almost no effect was noted as six hours later; the sNa was 156 mmol/L. At this time the desmopressin dose was increased to two drops every 12 hours. Three hours later, sNa was 151 mmol/L with concurrent D5W at 40 ml/hr and LRS at 25 ml/hr.
The dog's neurological status continued to improve. By the third day the menace response had returned in the left eye; there was reduced rolling and circling, and return of conscious proprioception in both forelimbs was noted. Despite the improvement in neurological status the dog developed intermittent head pressing, displayed ongoing subjective polyuria, and was also noted to be lame in the left foreleg, which continued throughout her hospital stay. A transient increase in rectal temperature was also noted (40.2°C); however, this normalised following active cooling with a fan and was thought most likely secondary to anxiety. Maropitant was administered at 1 mg/kg subcutaneously every 24 hours for suspected nausea (ptyalism) and ongoing inappetence. The dog subsequently began to eat 20 mls of Hills a/d slurry every four to six hours. Water was offered every four hours. LRS and fentanyl were discontinued, and D5W was increased to 50 ml/hr. She was continued on two drops of DDAVP in the conjunctival sac every 12 hours.
Despite this increase in D5W and DDAVP, during the fourth day the dog's sNa had increased again to 156 mmol/L and DDAVP was subsequently increased to three drops every 12 hours. Urine specific gravity was recorded to be 1.010 at the commencement of day 4. A mild hypokalaemia of 3.3 mmol/L (reference range: 3.4–4.9 mmol/L) was noted and LRS with 40 mmol/L KCl was recommenced at 10 ml/hr and the D5W was reduced to 40mls/hr. Four hours after the increase in DDAVP the sNa dropped to 151 mmol/L.
By the fifth day the sNa was between 151 and 153 mmol/L and frequent urination continued. The DDAVP was decreased to two drops every 12 hours, and the fluids remained unchanged.
On the sixth day sNa was 148 mmol/L. The dog became more alert and aggressive, which was consistent with her temperament prior to the TBI. She was starting to lick slurry from a bowl in addition to the syringe feeding commenced earlier. Over the following two days, her sNa remained between 148 and 150 mmol/L with a K of 4.4-4.8 mmol/L and a USG of 1.008. The clinical status remained unchanged and treatment was continued with 2 drops of DDAVP every 12 hours, and the D5W was reduced to 10 ml/hr. The USG increased slightly to 1.012 by day 8.
In the morning of day 9, the dog was ambulatory, although bumping into objects. The sNa was 144 mmol/L, and the DDAVP was therefore discontinued. The following day, the sNa was 140 mmol/L with a K of 6.2 mmol/L. The blood pH was 7.21, bicarbonate 11.2 mmol/L, pCO2 28 mm Hg, and base excess -15.2 mmol/L. The urea was 12.40 (reference interval: 3.40–10.80 mmol/L). She was given sodium bicarbonate (8.4%), 10 mmol, diluted in 50 ml of saline IV over three hours. A urine sample collected by cystocentesis showed cocci on sediment exam and amoxicillin clavulanic acid (12.5 mg/kg) was initiated subcutaneously once daily. The sNa for that day was 146 mmol/L and the K dropped to 5.1 mmol/L.
The dog had improved mentation with no head pressing or circling. Although she was ambulatory without ataxia, she would follow walls when walking. Her vision appeared reduced. The USG was 1.022 with a sNa of 148 mmol/L. She was discharged home on day 13 with oral amoxicillin clavulanic acid.
The dog returned two days later for a recheck, at which time her owner reported almost normal mentation. A repeat sNa was 152 mmol/L with a K of 4.8 mmol/L. She had an ongoing lameness of 4/5 in her left foreleg with no distinct foci of pain on palpation of the limb. Her aggressive behaviour limited the musculoskeletal examination.
During a final recheck 10 days later, the owner reported that her personality and behaviour had returned to what they had been before the accident. Given her ongoing left forelimb lameness, she was anaesthetised the following day after a preanaesthetic blood screen, in which her sNa was 143 mmol/L. Radiographs were taken of both forelimbs and showed bilateral severe osteoarthritis of her elbows, with the left being more severe than the right. The persistent lameness was likely from osteoarthritis exacerbated by trauma. |
pmc-6507256-1 | A 6-year-old female patient reported to the Department of Pedodontics with a chief complaint of pain in the upper right back tooth region. The right maxillary deciduous first molar was carious with the resorption of more than 2/3rd of its roots and hence had to be extracted. Model analysis was done followed by the placement of a fixed functional band and loop space maintainer (). |
pmc-6507256-2 | A 6-year-old boy had grossly decayed teeth 74, 75, 84, and 85. Radiograph of 74 showed poor prognosis and was extracted. Pulpectomy was done for the remaining teeth, and teeth were restored with stainless steel crowns. A functional band and loop space maintainer was cemented in relation to 74. Patient was recalled after three months, and it was found that there was no soft tissue irritation or dislodgement of the appliance (). |
pmc-6507256-3 | A 7-year-old female patient reported with the chief complaint of multiple decayed teeth and also with a history of extraction of the decayed right upper back tooth. Clinical examination revealed grossly decayed 53 and 64 and clinically missing 54. Radiograph of 64 revealed a poor prognosis and hence was extracted. Following model analysis, a conventional band and loop space maintainer in the 54 region and a functional band and loop space maintainer in the 64 region were cemented. Tooth 53 was endodontically treated and esthetically restored with composite resin (). Patient was reviewed every three months, and she reported that the fixed functional band and loop space maintainer helped her to chew comfortably. There was no mucosal irritation in relation to the appliance. |
pmc-6507256-4 | A 6-year-old female patient reported with clinically missing 74 and 84. Past dental records revealed the extraction of teeth 74 and 84 due to caries four months and two months earlier, respectively. History of difficulty in mastication was also reported. Model analysis was done. There was no space loss; hence, it was planned to maintain space with the functional band and loop space maintainer in relation to 84 and conventional one in relation to 74. Patient was recalled every three months for review, and she felt comfortable to chew on the right side (). |
pmc-6507256-5 | A 13-year-old girl reported to the department with complaint of several broken teeth and one lost permanent tooth. Parents gave history of trauma to the chin region due to accidental fall from a tractor ten days before reporting. Her medical history was not remarkable. On intraoral examination, tooth numbers 15, 16, 25, 26, 35, 36, and 45 had sustained uncomplicated crown fractures. Tooth number 34 was clinically missing, and tooth number 46 showed a complicated crown fracture. Panoramic radiograph confirmed the avulsion of 34 and showed no evidence of fracture involving maxilla or mandible. It was planned to restore tooth numbers 15, 25, 35, and 45 with composite restorations, perform RCT in 46, and restore 16, 26, 36, and 46 with stainless steel crowns as they had extensive tooth structure loss due to the impact of trauma. A functional space maintainer (band and loop with acrylic pontic of tooth number 34) was planned as an interim prosthesis and a space maintainer in the tooth 34 region. As the tooth 35 had extensive tooth loss on lingual aspect, the band would also help in the retention of the composite restoration till future definitive restorative management (). |
pmc-6507261-1 | In June 2018, a woman aged 40 years was admitted to the Anwer Khan Modern Medical College Hospital (AKMMCH), Dhaka, with fever for 4 weeks, dry cough for 2 weeks, and yellow discoloration of urine and sclera for 3 days. She was given intravenous (IV) meropenem (1 g, 8 hourly), moxifloxacin (400 mg, OD), and steroid (hydrocortisone, 100 mg, 8 hourly) for 10 days by a primary caregiver before admission to AKMMCH. The patient was a homemaker and was residing in an urban area. She was hypertensive, but had no history of trauma, diabetes, or other immunodeficiency disorders. On admission, she had a blood pressure of 120/80 mmHg, pulse rate of 92 beats per minute, and respiratory rate of 22 breaths per minute. The fever with the highest recorded temperature of 103°F (39.4°C) was often associated with chills and rigor. Cough was associated with respiratory distress unrelated to exertion or posture. The initial diagnosis was pyrexia of unknown origin, and the patient was transferred to the intensive care unit (ICU). The patient was intubated and kept on mechanical ventilation as she developed pulmonary hemorrhage, hematuria, and septic shock (CRP: 98 mg/L, provided inotropic support). The patient was found to have pulmonary consolidation in chest X-ray, ascites and hepatoslpenomegaly in ultrasonogram, concentric left ventricular hypertrophy in echocardiogram, and type-2 respiratory failure in arterial blood gases. The patient was negative for HBsAg, anti-HBcAb, and anti-HCVAb. Hemoglobin level, lymphocyte count, and platelet count were much lower, while neutrophil count was higher than the reference ranges. Random blood sugar was in the normal level. However, other blood chemistry parameters, e.g., bilirubin, alanine transaminase (ALT), aspartate transaminase (AST), albumin, prothrombin time, C-reactive protein (CRP), lactate, procalcitonin, and lactate dehydrogenase (LDH) were remarkably higher (). Based on these observations, the final diagnosis was pneumonia with acute respiratory distress syndrome along with disseminated intravascular coagulation. The antimicrobial regimen of IV cefepime (1 g, BD) and meropenem (1 g, 8 hourly) was empirically initiated. Blood, urine, and two samples of swab from the HME filter, one from the patient end and one from the machine end, were inoculated on sheep blood agar and MacConkey agar plates. After 24 hours of aerobic incubation at 37°C, there was no growth in blood, urine, and swab from the machine end of the HME filter. However, small colonies with dark violet metallic pigmentation were observed on both blood agar and MacConkey agar plates () from the swab of the patient end of the HME filter. The violet pigmentation led us to assume that these were C. violaceum colonies. Identification of C. violaceum was confirmed by biochemical tests, e.g., triple sugar iron (TSI) agar reaction and catalase and oxidase tests []. The antibiotic susceptibility test of the isolates was performed for amikacin, gentamicin, ceftriaxone, ciprofloxacin, cotrimoxazole, piperacillin-tazobactam, and meropenem by Kirby–Bauer disk diffusion method on Mueller–Hinton agar, and the result was interpreted according to the Clinical and Laboratory Standards Institute (CLSI) guidelines for non-Enterobacteriaceae Gram-negative bacteria []. The antibiotic sensitivity report revealed that the isolate was susceptible to all antimicrobials, and the antimicrobial regimen was switched to IV ceftriaxone (1 g, 12 hourly), pipercillin (4.5 g, 6 hourly), and cotrimoxazole (960 mg, 12 hourly). However, there was no clinical improvement; the patient progressively went into multiorgan failure and died. |
pmc-6507636-1 | An 80-year-old woman, who had undergone aortic valve replacement with a 23-mm Björk-Shiley valve and open mitral commissurotomy at our hospital for multivalvular rheumatic heart disease at age 51, had chest tightness during exertion after many years of uneventful, asymptomatic clinical development. Follow-up transthoracic echocardiographic examinations over the past several years showed a normal left ventricular volume and ejection fraction, and moderate mitral stenosis (mitral valve area: 1.5 cm2) and an elevated peak prosthetic aortic jet velocity of 300-350 cm/s had been maintained within the boundary area without worsening. Cardiac computed tomography (CT) angiography revealed no significant obstructive coronary disease, but an unexpected saccular ascending aortic aneurysm (maximum transaortic diameter: 51 mm) arising from the posterior ascending aortic wall was observed (Fig. A). Transesophageal echocardiography (TEE) revealed eccentric systolic flow jets through the tilting disc valve prosthesis directly impinging on the saccular aneurysm (Fig. A and B, ). In addition, cardiac CT angiography (systolic images) and TEE confirmed that the Björk-Shiley valve with a normal leaflet opening angle (Fig. C–E) was implanted with its major opening directed toward the non-coronary sinus, and was unpredictably tilted by the elevation of the prosthesis in the right-coronary sinus (Fig. B). The patient underwent reoperation for her ascending aortic aneurysm and possible stenosis of the prosthetic aortic valve. The ascending aorta was opened after aortic cross-clamping, and the prosthetic aortic valve and aortic aneurysm were inspected from the inside. Since the orifice of the aneurysm was located on the edge of the aortotomy suture line in the posterior wall of the ascending aorta, the lesion was probably considered a postsurgical false aneurysm. Although the Björk-Shiley valve prosthesis had no restricted leaflet motion without any obvious structural deterioration, thrombus, or abnormal pannus, it was causing an elevated transprosthetic velocity. Therefore, we decided to replace this old mechanical prosthesis with a 21-mm bovine pericardial bioprosthesis (Carpentier-Edwards Magna Ease Aortic Valve: Edwards Lifesciences, Irvine, CA, USA). The ascending aorta was replaced with a 26-mm Dacron prosthetic graft (J-Graft: Japan Lifeline Co., Ltd., Tokyo, Japan). The operative course was uneventful, and the patient recovered from surgery and has been free of complaints for almost three years. |
pmc-6509196-1 | Ms. S was a 63-year-old female with no previous medical or psychiatric history. In July 2017, she presented with dizziness, weakness, chronic shoulder pain, and high blood pressure. She informed her family that she felt helpless and sick. The preliminary examination revealed nothing but multiple lacunar infarcts in brain magnetic resonance imaging (MRI) scans. On September 17, 2017, she exhibited anhedonia, fear, anxiety, impatience, and a propensity to cry after being annoyed with others. She was examined in the psychiatric unit of the local hospital. Her value on the Self-rating Depression Scale (SDS) was 53.75, which pointed to mild depression, whereas on the Hastgawa Dementia Scale (HDS), she scored 13.0, which suggested probable dementia (education: primary school). The memory quotient (MQ) of Wechsler Memory Scale (WMS) was 59. Her sleep was normal. She was diagnosed with depression, and sertraline 50 mg/day was prescribed. Her symptoms nonetheless worsened with insomnia, garrulity, irritability, and gait imbalance. Her memory function deteriorated, and she became disoriented. The psychiatrist changed the antidepressant drug to venlafaxine 75 mg/day on October 8, 2017. However, instead of improving, the condition rapidly worsened. Her speech became hypophonic and monotonous with a paucity of content. She was sleepy during the day and sometimes burst into tears. Her arms curled up, indicating panic. She developed psychomotor retardation, responded poorly to questions, experienced visual hallucination, and suffered from a rigid posture with paroxysmal myoclonus and an inability to walk. The changes in her symptoms were initially considered to be side effects of venlafaxine. Two weeks later, she had deteriorated further and was unable to talk, exhibiting dysphagia and suffering from urinary incontinence. The symptoms did not improve after the withdrawal of the antidepressant. An assessment of her electroencephalogram (EEG) revealed generalized slow activity (). She was then transferred to the neurologic ward of our hospital where the following neurological findings were detected: akinetic mutism (AM), normal muscle strength, increased muscle tension, brisk tendon reflexes, and unresponsive pathologic reflexes. We performed a hematology screen for endocrine, metabolic, autoimmune, neoplastic, and infectious diseases, which were all negative. Cerebrospinal fluid (CSF) studies, including a paraneoplastic, an autoimmune antibody panel, and a tubercular, fungal antibody survey were also negative. Fluid-attenuated inversion recovery (FLAIR) of the brain MRI showed hyperintensities in the bilateral frontal lobe, corona radiate, and near the anterior and posterior horns of the lateral ventricle (). Diffusion-weighted imaging (DWI) showed hyperintensities in the bilateral caudate nucleus and putamen (). A diagnosis of CJD was considered. One week after admission, the second EEG was performed, revealing partially periodic sharp wave complexes (PSWC; ). No gene mutations associated with genetic CJD were found, but methionine homozygotes were detected at codon 129 of the prion protein gene. The final diagnosis was probable sCJD according to the diagnostic criteria for sCJD (, ). Antibiotics, antiviral, and corticosteroid therapies had been tried since admission, but none of them worked. Ultimately, she was discharged from the hospital. |
pmc-6509293-1 | A 50-year-old Japanese man presented to the Department of Gastroenterological Center, Shunjukai Shiroyama Hospital with acute right lower quadrant abdominal pain of a few hours’ duration. Physical examination revealed that he was 172 cm tall, weighed 65 kg, and had a body mass index (BMI) of 21.8 kg/m2. The abdominal pain was localized without rebound tenderness or guarding while bowel sounds were normal. The patient had no associated nausea, vomiting, or diarrhea. His medical history was unremarkable with no previous abdominal operations or problems. Routine blood tests showed that his white blood cell count was 8900/mm3 and the C-reactive protein (CRP) level was 8.13 mg/dl (Table ). Contrast-enhanced CT scan of the abdomen revealed twisting of the omentum with a local mass of fat density and fluid distributed in a whirling oval-shaped mass pattern at the right flank and iliac fossa (Fig. ). The fatty mass was situated between the transverse colon and the gallbladder and contained hyperattenuating streaks.
Therefore, the patient was admitted to our hospital, based on a diagnosis of the omental torsion. At first, he was treated with conservative treatment because his vital signs were stable and his symptoms were non-specific and mild. Although his symptoms were ameliorated by oral analgesics, anti-inflammatory drugs, and prophylactic antibiotics, laboratory tests 5 days after admission showed a decreased leukocytosis (white blood cell count 5500/mm3) but increased CRP (18.49 mg/dl). A second CT scan showed that the twisting of the omentum with a local mass of fat density and fluid had been retained; moreover, the fat density and fluid were worsened. Therefore, laparoscopic omentectomy was performed 6 days after admission.
The patient was placed in the supine position under general anesthesia. Initially, the abdominal cavity was reached using a 12-mm trocar from the left of the umbilicus. Four accessory trocars (5 mm each) were placed in the right upper quadrant, left upper quadrant, left lower quadrant, and above the pubis symphysis. During abdominal exploration, a solid ischemic and hemorrhagic portion of the right greater omentum was found adhered to the right side of the abdominal wall and transverse colon (Fig. ). Although this portion was adhered to the transverse colon, the appendix and gallbladder were normal, and Meckel diverticulum was not present. Then we entered the omental bursa, and the adhesion between the transverse colon and omentum was resected. Therefore, this portion was completely free of adhesions due to the resection (Fig. ). The specimen was retrieved in a bag through a small abdominal incision 5.0 cm in length above the umbilical trocar. The total operating time was 200 min, and the intraoperative blood loss was 100 ml.
Macroscopically, this specimen was 24 cm × 22 cm in size and was twisted and dark red in color, suggesting infarction (Fig. a, b). Histological analysis proved the specimen to be ischemic and hemorrhagic omentum, accompanied by inflammatory infiltration, confirming the diagnosis of omental infarction due to the torsion (Fig. a, b).
The patient’s postoperative course was uneventful, and he was discharged 9 days later. |
pmc-6509294-1 | A 49-year-old male with a history of cholelithiasis presenting with right hypochondoralgia with a positive Murphy’s sign was referred to our department for surgical treatment. Computed tomography (CT) without contrast media revealed a gallstone in the thickened gallbladder wall with a slight increase in the CT value due to surrounding panniculitis (Fig. ). MRCP revealed that the cystic duct branched from the common bile duct and an aberrant bile duct connected to the cystic duct (Fig. , yellow arrow). The link between the aberrant bile duct and major intrahepatic biliary system was not visually apparent by MRCP. The patient was diagnosed with chronic calculous cholecystitis with aberrant bile duct flow into the cystic duct. During laparoscopic cholecystectomy, four ports were placed: a 12-mm camera port in the umbilical area by open method, 12-mm port in the epigastric area, 5-mm port in the right subcostal area, and a 5-mm port at the right lateral abdomen. Due to inflammatory fibrotic adhesion in Calot’s triangle, the ARHD was excessively exposed. A fundus-first technique was performed to confirm the ARHD after exposure of the inner layer of the subserosal layer at the dorsal and ventral side of Calot’s triangle. ARHD drainage into the cystic duct was confirmed. Preoperative MRCP suggested it was not necessary to preserve the ARHD with the extreme narrow drainage area as it seemed to be closed at the hepatic side without communicating with the major right branch of the intrahepatic bile duct.
Intraoperative cholangiography from the cystic duct in the periphery (Fig. ) revealed that the ARHD was not confluent with the major right branch of the intrahepatic bile duct and drained a narrow area, so we removed the excessively exposed ARHD. Removal and ligation of the ARHD on the hepatic side and cystic duct was performed by clipping (AESCULAP DS Titanium Ligation Clips, B Braun brand, Tokyo, Japan). An absence of bile leakage precluded any placement of drainage. The surgical movie is available online only (Additional file 1: Video). The postoperative course was uneventful, and the patient was discharged on the third postoperative day. Follow-up MRCP showed no dilated bile duct in the liver 1 month after surgery (Fig. ). The resected specimen was diagnosed as chronic cholecystitis. Laboratory analysis showed no abnormal increase in AST, ALT, ALP, or bilirubin 1 month after surgery (data not shown), and no abnormal symptoms 3 months after surgery. Then, his family doctor is currently following up him. |
pmc-6509665-1 | A 60-year-old female presented with 3-month history of intermittent fever, cough, dyspnea, fatigability, and personality changes. Prior to onset of symptoms, the patient reported exposure to decayed wood in the summer as her husband was remodeling their house. Her history was notable for systemic lupus erythematosus (SLE), diagnosed 15 years earlier. Although the patient denied follow-up for SLE, she stated she was well controlled with hydroxychloroquine. A complete blood count showed cytopenia with hemoglobin 8.9 g/dL, white blood cells count of 1900 cells/mm3, and platelets count of 22 000 cells/mcl. Additional laboratory studies showed lactate dehydrogenase (535 unit per liter; normal range [140-280]), serum triglycerides (259 milligrams per deciliter; normal range [<150]), ferritin (21 900 nanograms per milliliter; normal range [20-500]), and fibrinogen (57 mg per deciliter; normal range [150-400 mg per deciliter]), elevated hepatic transaminases, positive histoplasma antigen in the cerebrospinal fluid analysis and a negative HIV test. Computed tomography showed bilateral lung ground glass opacity with enlarged mediastinal lymph node and massive splenomegaly. Histologic examination of the bone marrow showed activated macrophages, including some with hemophagocytosis (Figure ), intracellular numerous yeast form, and spiked spherical conidia of Histoplasma capsulatum (Figure ). The patient received a diagnosis of HLH and disseminated histoplasmosis. Treatment with IV liposomal amphotericin B 5 mg/kg once daily was initiated for 4 weeks followed by oral itraconazole 100 mg a day for one week and then 200 mg daily to complete one year of therapy. Although she received a 10-day course of dexamethasone 10 mg twice a day with improvement of the fever, they were stopped for agitation status. Her fever resolved 2 weeks after treatment along with all other symptoms. At follow-up 5 months later, no new symptoms were reported, and the laboratory studies were normal. |
pmc-6509666-1 | A 43-year-old woman was diagnosed with IDA based on her blood test results. She had presented with weakness, fatigue, and palpitation. Because she exhibited the desire to smell exhaust and gasoline and to smoke, she was assessed for pica. The patient reported that she had previously been treated for iron deficiency, following which she had quit smoking and had no desire to smell exhaust and gasoline. The patient was treated with oral ferro fumarate 200 mg/d for 42 days. After the treatment period, her IDA was treated, and she quit smoking as well as lost the desire to smell exhaust and gasoline. |
pmc-6509666-2 | A 37-year-old woman was diagnosed with IDA based on her blood test results. She had presented with symptoms of anemia. On being assessed for pica, she reported that she had an excessive desire to smell menthol. The patient was treated with oral ferro fumarate 200 mg/d for 60 days. During her check-up after the treatment period, her IDA was treated, and the desire to smell menthol was no longer present. |
pmc-6509666-3 | A 47-year-old woman was diagnosed with IDA based on her blood test results. She had presented with symptoms of anemia. On being assessed for pica, she reported that she had a desire to smell exhaust and gasoline. She was treated with intravenous ferric hydroxide sucrose 100 mg/d for 5 days. After 35 days of treatment, her IDA was treated based on her blood test results, and the desire to smell exhaust and gasoline was no longer present.
The clinical characteristics of the cases have been summarized in Table . |
pmc-6509667-1 | A 77-year-old man with smoldering type ATLL had been treated for specific skin lesions. He had been also treated for diabetes mellitus with oral hypoglycemic agents. Erythema progressed to plaques and tumors in spite of treatment with topical corticosteroids, narrow-band ultraviolet B, and oral etretinate. He received electron radiation therapy followed by oral prednisolone and low-dose etoposide. We stopped prednisolone and etoposide because of severe stomatitis. Although there were increasing multiple plaques and tumors on his trunk and extremities (Figure A-D), progression from smoldering to acute subtype did not occur. Histopathologically, a dense infiltration of small-to-medium-sized pleomorphic lymphoid cells was observed in the dermis with prominent epidermotropism (Figure E-G). Infiltrating cells were CD3+, CD4+, CD8−, CD79a−, and CCR4+ (Figure H-K). Foxp3+ cells were observed among atypical cells (Figure L). Although he was elderly, he had no problem with hematological parameters and liver function test: hemoglobin 13.3 g/L, White blood cell count (WBC) 4.6 × 109/L, neutrophils 3.8 × 109/L, lymphocytes 0.33 × 109/L, monocytes 0.38 × 109/L, eosinophils 0.04 × 109/L, basophils 0.02 × 109/L, platelet 221 × 109/L, aspartate aminotransferase (AST) 21 IU/L, and alanine aminotransferase (ALT) 21 IU/L. Blood examination showed mild renal dysfunction: blood urea nitrogen (BUN) 22.7 mg/dL, creatinine 1.06 mg/dL, and estimated glomerular filtration rate (eGFR) 52.3 mL/min/1.73 m2. We intended to inject mogamulizumab 1.0 mg/kg, once weekly for 8 weeks.
Two days later from the first mogamulizumab administration, plaques and tumors became flattening and dark reddish-brown (Figure A-D). Skin lesions continued to be improved during treatment period (Figure E-H). Modified Severity-Weighted Assessment Tool (mSWAT) score was improved 70 (before the first infusion) to 34 (after the second infusion). As more than 50% of skin lesions were improved, we considered partial response (PR) was achieved. Blood examination revealed normal hematological parameters and liver function during and after the mogamulizumab treatment: hemoglobin 13.5 g/L, WBC 6.5 × 109/L, platelet 300 × 109/L, AST 16 IU/L, ALT 15 IU/L after the first infusion, and hemoglobin 12.3 g/L, WBC 5.7 × 109/L, platelet 237 × 109/L, AST 19 IU/L, ALT 13 IU/L after the final infusion. Renal function was not exacerbated: BUN 15.1 mg/dL, creatinine 1.11 mg/dL, eGFR 49.8 mL/min/1.73 m2 after the first infusion, and BUN 22.2 mg/dL, creatinine 1.01 mg/dL, eGFR 55.2 mL/min/1.73 m2 after the final infusion.
Erythema and cracks on his hands, and multiple erythema, papules, and purpuras on his lower legs appeared 19 weeks later from the first mogamulizumab treatment (Figure A-C). Histopathologically, spongiosis in the epidermis, liquefactive degeneration of basal cells, and lymphocytes, eosinophils, and erythrocytes in the upper dermis was observed (Figure D). Lymphocytes were CD3+, CD4−, CD8+, CD79a−, granzyme B+ (partially), perforin−, TIA-1−, and Foxp3+ (slightly) (Figure E-I). We considered his skin lesions as spongiotic dermatitis, not specific skin lesions of ATLL. Spongiotic dermatitis was improved by oral prednisolone 10 mg/d. Only erythema on his legs remained (Figure J-M). The mSWAT score was 24, and PR was maintained. |
pmc-6509668-1 | A 22-year-old man woke up with severe left shoulder pain which later became a severe arm pain. He was diagnosed with shoulder sprain (as noted by his referring physician) initially and was put in a sling. There was no evidence of trauma. Despite these measures, the pain persisted, and he was prescribed multiple medications including paracetamol, gabapentin 600 mg three times a day and hydrocodone 5 mg three times a day PRN. Two weeks later, he started developing hand and arm weakness at which time he was evaluated by us. On examination, he had 2/5 wrist extension, 3/5 finger flection, 2/5 abduction of fifth digit, and 4/5 shoulder abduction but no scapular winging. Pin prick testing did not show sensory loss along the dermatomes. Electrodiagnostic study performed a month from onset showed denervation in multiple muscles innervated by median, ulnar, radial, and axillary nerves. MRI of the brachial plexus with and without contrast showed patchy T2 hyperintense signal involving all the trunks of the left brachial plexus (Fig. ). There was no enhancement with contrast. He was diagnosed with idiopathic brachial plexopathy (neuralgic amyotrophy) and managed conservatively with physical therapy and gabapentin 300 mg three times a day for neuropathic pain. Two months later, he complained of dry eyes and dry mouth. Hence, further workup was performed which showed antinuclear antibodies at 1:1200 (Mayo Clinic, Normal <1:40) and a positive SSA antibody 3.5U (Mayo Clinic, normal- <1.0U). See Table . Lip biopsy showed focal lymphocytic sialadenitis of the minor salivary glands confirming the diagnosis of Sjögren syndrome (based on American College of Rheumatology criteria). He was treated with in hydroxychloroquine in addition to intravenous immunoglobulin (induction dose-2 g/kg actual body weight split over 5 days followed by 1 g/kg actual body weight split over 2 days every 6 weeks for a total of eight doses) for brachial plexopathy. |
pmc-6509669-1 | A 64-year-old man was admitted to our hospital for further examination regarding ischemic findings on myocardial perfusion scintigraphy. His cardiovascular risk factors were type 2 diabetes mellitus and dyslipidemia. His blood pressure was 127/71 mm Hg, and pulse was 89/min and regular. His body mass index was 18.1. No particular finding was noted on physical examination, and laboratory data showed no abnormality without hyperglycemia (blood sugar: 251 mg/dL). No significant sign of cardiac ischemia was noted on twelve-lead electrocardiography. Chest X-ray showed a normal cardiothoracic rate (42.8%) and no abnormal pulmonary lesion. The left ventricular wall motion was within normal limits and no apparent valvular disorder was seen on ultrasound echocardiography.
Coronary angiography showed moderate in-stent restenosis (ISR) at the ostium of the left anterior descending (LAD) artery (Figure A,B Arrows). A Cypher stent (Cordis, CA, USA) had been implanted there 12 years previously. We first checked the lesion with OptiCross (Boston Scientific) intravascular ultrasound (IVUS). The implanted Cypher stent segmentally exhibited less expansion than expected (Figure C). A diffuse longitudinal distribution of high-echoic plaque partially with ambiguous stent struts and typical echo attenuation of the whole circumference were observed in the stenosed segments (Figure A,C). Just after the IVUS scan, the patient suddenly complained of severe chest pain, and ST-segment elevation with an increased voltage of T waves in V2-4 leads appeared on electrocardiography (Figure A). Coronary angiography showed stasis of blood flow at the distal end of the LAD artery (Figure B, Arrowhead). We suspected distal embolism, and the injection of a vascular relaxant agent after aspirating the blood in the LAD artery favorably recovered blood flow. Then, using a Filtrap catheter (NIPRO), a filter device to prevent distal embolisms, we performed balloon angioplasty with a scoring balloon catheter several times and finally inflated a drug-coated Sequent Please balloon catheter (NIPRO) for one minute. After successful retrieval of the Filtrap catheter, we noted favorable vascular expansion with no flow disturbance in the LAD artery (Figure ). Postoperatively, no significant myocardial damage, which would be indicated by an increased level of creatinine phosphokinase (CPK), was observed. |
pmc-6509670-1 | A 49-year-old female patient was admitted for desensitization to lidocaine. The patient was to undergo dental treatment for a tooth extraction and implant; however, the patient was allergic to lidocaine. In 2006, the ligament of the patient's right second finger was ruptured, and local anesthesia was administered for the local surgery. The patient exhibited chest tightness, dyspnoea, and resultant respiratory difficulty immediately after a local injection of lidocaine of just several ml. In the same year, the patient received local anesthesia with lidocaine for the removal of a thorn in her left third fingertip. She felt dizziness and exhibited syncope for a short duration.
Thereafter, she received dental treatment with local anesthesia with lidocaine several times without any inconveniences. However, 3 years ago she visited a dental clinic for a tooth implant and again experienced anaphylactic symptoms of dizziness, a short syncope and respiratory difficulty just after receiving a local injection of lidocaine in the gums.
Recently, this patient found out that her teeth should be extracted due to dental caries, and she consulted the Allergy Center, Cheju Halla General Hospital (Jeju-si, Korea) for the diagnosis and proper treatment of lidocaine allergy. She was admitted for the provocation test to confirm the allergy to lidocaine and to undergo the desensitization to lidocaine.
Blood and skin prick tests were performed for a general allergy laboratory analysis. In the complete blood count with differential count, the eosinophil fractions were 1.0% (normal range, 0%-5%) at the initial evaluation and 1.6% just after the desensitization. The initial serum eosinophil cationic protein level was 5.1 μg/L (normal range, 0.0-14.9 μg/L). The total serum IgE levels were 8.7 KU/L (normal range, 350 KU/L>) at the initial test and 9.2 KU/L just after the desensitization. Specific IgE levels were tested for 40 allergens by MAST (Green Cross®, Seoul Korea). Dermatophagoides pteryonyssinus (100 IU/mL <(normal range, 0-0.34 IU/mL), Grade 6), Dermatophagoides farinae (100 IU/mL <, Grade 6), Crab (0.47 IU/mL, Grade 1), and Ox-eye daisy (0.36 IU/mL, Grade 1) were positive and other allergens (Cat, Dog, Egg white, Milk, Soybean, Shrimp, Peach, Mackerel, Rye, Cockroach, Cladosporium, Aspergillus, Alternaria, Birch/Alder, White oak, Short ragweed, Mug wort, Japanese hop, Hazelnut, Sweet Grass, Bermuda Grass, Cocksfoot, Timothy Grass, Reed, Penicillium, Sycamore, Sallow willow, Cottonwood East, Ash mix, Pine, Japanese Cedar, Acacia, Dandelion, Russian thistle, Goldenrod, and Pigweed) were negative. The skin prick test was negative for 53 tested allergens (Alternaria alternate, Aspergillus fumigatus, Aspergillus niger, Candida albicans, Clasdosporium, Penicillium Chrysogenum, German cockroach, Dermatophagoides pteronyssinus, Dermatophagoides farina, Dog, Cat, Grey Alder(Silver Birch), Grass mix, Mug wort, Short Ragweed, Black willow pollen, Orchard, Bermuda grass, Timothy, English plantain, English Rye grass, Holm oak, Japanese cedar, Cotton flock, Milk, Egg, Chicken, Beef, Pork, Cod, Oyster, Salmon, Prawn, Mackerel, Tuna, Almond, Peanut, Bean, Carrot, Cabbage, Walnut, Maize, Peach, Tomato, Black pepper, Spinach, Wheat, Rabbit, Kapok, Hop, F acacia, Pine, and Poplar).
The skin prick test for lidocaine was negative. The intradermal test for lidocaine was strongly positive at the initial evaluation and converted to negative just after the desensitization (Figure A).
Emergency preparations were undertaken for the potential of anaphylactic shock during the challenge test and desensitization. The EKG and percutaneous O2 saturation of the patient were monitored continuously during the test. Intravenous steroid, antihistamine, and epinephrine were on hand. Body temperature, blood pressure, and pulse rate were monitored periodically.
Due to the pharmacologic/toxic effects of lidocaine on the heart, consults were requested from a subdivision of cardiology and the department of internal medicine, and the consults cooperated during the entire process of desensitization, including dosage modulation. In addition, for the original purpose of dental care, a consultation was requested from the department of dentistry to determine the maximal use of lidocaine during the dental procedure. Due to the patient's psychologic problems resulting from her experiences of anaphylaxis, a consultation was requested from the department of psychiatry, and the patient was supported psychologically during the entire process of desensitization and final dental care.
The patient was to receive local anesthesia the dental care, and there was no other modality for desensitization to lidocaine. For the new advanced concept in the case of impediment during desensitization at a certain dose, IFN-gamma was expected to have allergen-specific tolerogenic effects similar to those observed in food allergies in situations where desensitization meets an impediment and fails. For the application of IFN-gamma in the desensitization of drug allergy, a consultation was requested from the Clinical Pharmacy Coordinator/Antimicrobial Stewardship Pharmacist, Central Valley Specialty Hospital (Modesto, CA) concerning the immunologic effects of IFN-gamma for drug desensitization.
In contrast to the pre-existing protocol for drug desensitization, the dosage escalation was modulated less steeply because severe anaphylactic shock was expected with steep dosage escalation. Desensitization using IFN-gamma was initiated with the impediment dose. According to the theoretical background of anaphylactic food allergy, the incremental dose range was extremely low and rose steeply in the high-dose range on a log scale (Table , Figure ). Lidocaine, which did not contain any other additives, was dissolved in normal saline.
IFN-gamma was administered for the desensitization of food allergies. However, in this case of intravenous lidocaine desensitization, IFN-gamma was used only once per day. If impediment was faced during the desensitization, there was an attempt to overcome it the next day.
The maximally severe clinical signs and symptoms were determined during the challenge test and in the early stage of desensitization as in the tolerance induction of food allergy. Minimal signs and symptoms which permitted progression to the next dose were determined during the challenge test and in the process of early desensitization.
An impediment is an allergy provocation by a certain dose during the desensitization; in the case of impediment, progression to the next step is impossible. In the previous protocols based upon previous basic concepts, the same dose was used repetitively with the expectation that the allergic reactions would diminish or disappear. However, in this case, the patient exhibited a more severe allergic reaction with the repetition of the same challenge dose; therefore, desensitization was deemed to have failed, or the challenging dose needed to be reduced.
IFN-gamma was used only to overcome the impediment. IFN-gamma was not used if the patient did not show significant allergic signs or symptoms at the previous dose.
The desensitization using IFN-gamma was determined to be a failure when the patient exhibited persistent severe allergic signs and symptoms which the patients could not tolerate, or when more severe reactions by repeated challenge with same impediment dose occurred, even using IFN-gamma. Desensitization was allowed to progress when the symptoms and signs were decreased with same impediment dose using IFN-gamma.
To determine impediment based upon the patient's signs and symptoms, ignorable responses should be defined as baseline symptoms and signs during challenge test and desensitization. In addition, untoward side effects of IFN-gamma and pharmacologic effects of lidocaine, and psychologic effects due to psychologic trauma from a history of anaphylaxis should be differentiated (Figure ). Only when the patient showed significant allergic responses was it deemed an impediment.
Intervals for the challenge were determined by the disappearance of significant symptoms and signs that were provoked by previous challenges, considering the pharmacologic effects of lidocaine and the persistent allergic responses. However, when the signs and symptoms were strong, one more interval was prepared until the signs and symptoms reduced to zero or at least to a baseline.
IFN-gamma (Intermax gamma, LG Chemistry®, Seoul, Korea) was administered subcutaneously at a dose of 150 MU (37.5 µg) on the arm 15 minutes before the challenge of the impediment dose, as with anaphylactic food allergies. However, if the patient tolerated this dose well, the IFN-gamma dosage was increased by 2 MU (50 µg) in the middle of treatment. Acetaminophen 650 mg was prescribed 15 minutes before the IFN-gamma injection to avoid untoward side effects of IFN-gamma, including headache, myalgia, abdominal pain, etc IFN-gamma was administered early in the morning.
An intravenous challenge test was performed according to the protocol at day #1 of admission. A single vial of lidocaine contains 20 mL with a concentration of 2% and contains 36 mg. Lidocaine was diluted using normal saline for the skin prick test, intradermal test, and intravenous challenge. For the use of lidocaine in dental treatment, the target test dose was 36 mg with the assumption that a total vial of 2% lidocaine is used for local anesthesia and is absorbed directly into the circulation.
Lidocaine was challenged intravenously sequentially along the basic dosage protocol for the challenge test and desensitization, as shown in Table . The initial testing dose was 1 ng, and the dose was increased in increments of 1 ng within the range of 1-10 ng and by 10 ng within the range of 10-100 ng. The dose was increased 10-fold in every step.
At a dose of 20 ng, the patient experienced a slight periorbital burning sensation and sneezing, which were suspected as allergic responses. With the injection of 30 ng, the patient again experienced burning sensations which were spread to the periorbital area and posterior neck through face along with sweating. When 30 ng of lidocaine was injected again, the patient exhibited more severe anaphylactic symptoms including immediate abdominal discomfort, vomiting, and respiratory difficulty. The diagnosis of anaphylactic lidocaine allergy was made concretely. The symptoms and signs disappeared after 1 hour, and the interval of challenge was determined to be 1 hour.
Desensitization was tried in a pre-existing concept, and 30 ng of lidocaine was challenged as the third trial for the desensitization 1 hour after the diagnosis was made. However, the patient exhibited additional symptoms, such as laryngeal edematous, voice change, and a choking sensation. Lidocaine 30 ng was challenged three times, but the patient reacted more strongly with more signs and symptoms, and the clinical severity increased with the dose repetition. The patient received 30 ng of lidocaine three times intravenously, and the clinical reactions were aggravated by the repetition. It was predicted that anaphylactic shock would occur upon challenging with an increased dose or by continued repetition with same dose.
Desensitization using the pre-existing concept failed or dosage modulation was needed. At this point, it was decided to introduce IFN-gamma with the expectation that allergen-specific tolerogenic effects would overcome the impediment at the dose of 30 ng (Figure ). The starting dose for desensitization was determined as 30 ng as the minimum allergy-provoking dose, and the target dose was 40 mg for dental care, which was deemed to be a non-toxic dose for cardiovascular effects.
Desensitization using IFN-gamma was restarted in the early morning of admission on day #2 with the dose of 30 ng, and the patient exhibited more severe allergic reactions with the repetition. Acetaminophen was administered orally 30 minutes before the challenge to prevent the untoward effects of IFN-gamma, and IFN-gamma was injected at a dose of 37.5 µg (150 million units) subcutaneously 15 minutes before the lidocaine challenge. Lidocaine 30 ng was challenged for desensitization, and, surprisingly, the signs and symptoms were dramatically decreased and included only a burning sensation of the periorbital area and posterior neck. With the repeated challenge of 30 ng, the patient experienced only the burning sensation of the periorbital area and posterior neck, which were considered baseline and ignorable signs and symptoms of allergic responses. After the IFN-gamma injection, the patient reported feeling myalgia, which was regarded as a baseline sign and symptom resulting from IFN-gamma during the desensitization process.
The patient was challenged with an increased dose of 40 ng and exhibited vomiting and laryngeal edema immediately; thus, the challenge was terminated. On admission day #3 at 6:30 am, the patient was pre-treated with Tylenol 650 mg for the prevention of the untoward side effects of IFN-gamma. IFN-gamma was injected subcutaneously 15 minutes after the Tylenol intake. Lidocaine 40 ng was challenged 15 minutes after the IFN-gamma injection as performed for injection 4. No allergic signs or symptoms appeared, except for myalgia, which was considered an effect of IFN-gamma.
The desensitization proceeded, and the patient met impediment five times at the dose of 30, 40 ng, 4, 300, and 700 μg, which were overcome using IFN-gamma. The target dose of 40 mg was reached on the admission day #8.
After successfully reaching the target dose by desensitization, tolerance acquisition was confirmed by repeating the intravenous injection of lidocaine. One day after finishing the desensitization, 20 mg in the morning and 40 mg in the evening were injected without any symptoms or signs. Two days after finishing desensitization, 40 mg of lidocaine was injected, again without any symptoms and signs. The intradermal test for lidocaine was repeated, and the patient showed a negative result (Figure B).
Finally, 3 days after finishing desensitization, the patient received local anesthesia with lidocaine (27 mg) and safely underwent dental treatment (extraction of two teeth). One day after the dental treatment, patient was discharged. |
pmc-6509671-1 | A 37-year-old P4 + 0 delivered a live female baby at the obstetrics unit of our hospital via normal spontaneous vaginal delivery. Antenatal care examinations during the 1st and 2nd trimesters were unremarkable. A routine obstetric ultrasound scan at 32 weeks of gestation, however, had confirmed the findings of a live intrauterine fetus with a sacrococcygeal mass.
The total weight gained during pregnancy was 15kg, and she was maintained on supplemental iron and folic acid.
At birth, the female newborn had a birth weight of 3.800kg. Apgar score was 9/10 and 10/10 in the first and fifth minutes, respectively. There were no significant findings on systemic examination.
Local examination revealed a solid-cystic, firm mass measuring 10cmx7cm in the sacral region with deviation of the anal orifice posteriorly (Figures and ).
Plain radiographs and abdominal ultrasound scans confirmed a sacrococcygeal tumor stage I Altman classification arising from the coccyx. There was no bladder, genitalia, or bowel involvement. The echocardiogram, electrocardiogram, and brain ultrasound were normal. Abdominal CT and MRI scans were not possible due to financial constraints.
Postnatal examination of the P4 + 0 was unremarkable.
All preoperative investigations on the newborn were essentially normal: Hb level 18g/dl, random blood sugar 105 mg/dl, serum alkaline phosphatase 110 IU/dl, PT 15, INR 1, BUN 30 mg/dl, hematocrit 33%, platelet counts 300000/l, and bilirubin 1.3 mg/dl. Cryptococcal antigen and syphilis serology (Venereal Disease Research Laboratory and Treponema pallidum hemagglutination assay) were negative.
Alpha-fetoprotein (AFP) serological measurements were unable to be done due to financial constraints. Due to the unavailability of AFP titers, a decision of surgical excision was made. Tumor resection and coccygectomy were done 10 days after birth using the posterior sacral approach under general anesthesia (Figure ).
An ulcerated cystic mass measuring 11cmX8cmX4.5cm with an ulcer measuring 4cmX3cm and weighing 175grams was resected. The mass contained cystic fluid and purulent material. Histopathological examination of the excised tissue revealed a multilocular cystic mature sacrococcygeal tumor with no evidence of malignancy. Immunohistochemical stains for AFP were positive.
On postoperative day 2, the stitches at the wound site gave way and the wound became septic (Figure ). Blood cultures and wound site swabs were unremarkable. The baby was diagnosed with a surgical site infection.
Since the baby did not have symptoms of hyperbilirubinemia, a decision was made to manage the baby with postoperative intravenous antibiotics—a first-generation cephalosporin—ceftriaxone 100mg/kg, a broad-spectrum antibiotic—metronidazole 35mg/kg, paracetamol 7.5mg/kg after conducting debridement, irrigation with warm normal saline solution and refashioning in the operating theater.
The baby was managed with daily wound pressure bandage dressings in addition to saline wound cleaning. Baby was discharged on postoperative day 25.
Follow-up at 6 months revealed that the child had a well-healed though poor cosmetic scar (Figure ) with no biochemical or physical evidence of recurrence. The child had achieved the age-appropriate developmental milestones. |
pmc-6509785-1 | We describe a case of a 36-year-old black African woman with two previous live births by cesarean section and two previous miscarriages who was referred in her fifth pregnancy after 6 weeks of amenorrhea. Her serum quantitative β-human chorionic gonadotropin (bHCG) was 16,124 mIU/ml. However, an intrauterine or extrauterine pregnancy could not be located on a transabdominal ultrasound scan.
A copper intrauterine contraceptive device had been removed 2 months prior to her last menstrual period. She reported using one cycle of clomiphene 50 mg with the hope of achieving a twin pregnancy.
She had delivered twice by cesarean section for failure to progress. Her last two pregnancies had been first-trimester miscarriages; one was managed expectantly, and the other was surgically evacuated, though the actual procedure was unknown to the patient. She did not have any chronic medical illness and was not receiving any medication prior to this presentation. She stayed in a city suburb that was well serviced. She was a school principal in her second marriage with no children in the current relationship. She did not smoke or drink alcohol.
On examination, she had a normal blood pressure of 113/70 mmHg and a pulse rate of 98 beats/min. Her body temperature was 37.5 °C. On examination, her cardiorespiratory and neurological systems were normal. Her abdomen was soft and not tender. The result of her pelvic examination was normal. TVUS showed a gestational sac of 13 mm with irregular margins and a visible yolk sac located on the anterior isthmic portion of the uterus, raising suspicion of a cesarean section scar ectopic pregnancy. She declined a Doppler ultrasound evaluation scheduled for the next day. She was scheduled to have serial bHCG evaluations every 48 h.
A repeat serum quantitative bHCG done 48 h after the initial test revealed a level of 21,521 mIU/ml, a 33% rise. She defaulted follow-up until 1 week later, when she presented with pelvic pain of increasing intensity for 5 days. An urgent transvaginal scan was performed. A fetal pole with active cardiac activity (crown-rump length 0.9 cm) in a gestational sac was located in the anterior low myometrium. The sac traversed the full width of the anterior myometrium, with the posterior margin of the sac abutting the anterior margin of endometrium and the anterior margin of the sac extending to a subserosal location in a fairly exophytic fashion. There was evidence of trophoblastic circulation on Doppler examination. There was no endometrial fluid or free pelvic fluid (Fig. ).
She was immediately admitted for a diagnostic laparoscopy/hysteroscopy and possible excision of the scar pregnancy if confirmed. A preoperative complete blood count showed hemoglobin 13.4 g/dl, white blood cells 7.2 × 103/μl, and platelet count 243 × 103/μl. The patient’s kidney function was normal with sodium 135 mmol/L, potassium 4.9 mmol/L, urea 3.5 mmol/L, and creatinine 65 μmol/L. The patient’s liver function test results were also normal. She had a negative result in a blood test for human immunodeficiency virus. Urinalysis did not show abnormalities. The patient’s random blood sugar was 5.6 mmol at admission. At laparoscopy, the bladder was adherent high on the anterior uterine wall, and the ectopic pregnancy was not visualized (Fig. ). At hysteroscopy, there were extensive adhesions within the lower endometrial cavity, which obscured visibility. There was no active intracavitary bleeding ruling out a threatened or inevitable miscarriage. We could not visualize any obvious bulge in the cervical canal suggestive of a cervical ectopic pregnancy. Because of the uncertainty of the location of the pregnancy due to adhesions, excision was postponed. Postoperatively, the patient became unstable with low blood pressure, systolic pressure range of 82 to 95 mmHg and diastolic pressure range of 40 to 55 mmHg, and a pulse rate range of 64 to 73 beats/min, but without active vaginal bleeding or use of medications inducing persistent hypotension. Anesthesia had been induced with etomidate 16 mg and suxamethonium 100 mg, and maintenance was initiated with isoflurane 0.8–1.5%. Intra- and perioperative analgesia was induced with fentanyl 200 mg intravenously (IV), indomethacin 100 mg rectally, and paracetamol 1 g IV. Antibiotic prophylaxis was with ceftriaxone 1 g IV and metronidazole 500 mg IV. This prompted us to order an urgent MRI scan to map the location of the pregnancy in the immediate postoperative period. MRI confirmed the TVUS findings of a cesarean section ectopic scar extending to the serosa (Fig. ).
An emergency laparotomy was then performed on the same day. The abdomen was entered through a Pfannenstiel incision along the old skin scar. A transverse incision was made in the upper uterine segment just above the adherent bladder. The products of conception were removed with forceps, and the gap in the anterior myometrium at the old scar was seen and felt. There was massive bleeding from the implantation site. Twenty milliliters of vasopressin (20 U diluted in 100 ml) in normal saline was administered into the bleeding myometrium edges. The edges were apposed in layers with VICRYL suture (Ethicon, Somerville, NJ, USA) to repair the defect. Estimated blood loss was 2000 ml. The patient was transfused with 1 U of packed cells intraoperatively. She was continued on the same intravenous antibiotics and analgesia that had been commenced after the laparoscopy. Her hemoglobin count on day 1 postoperatively was 8 g/dl, and she declined any further transfusion. Oral iron and folic acid supplementation was commenced.
The patient’s postoperative recovery was uneventful, and she was discharged on day 4 after surgery. Histology confirmed the presence of decidua and chorionic villi. The patient wanted a child because she was in a new relationship, but she was no longer sure of her future fertility plans after the ectopic pregnancy. A levonorgestrel implant was inserted 2 weeks postoperatively. The patient last attended physical review at 6 weeks, and she was well with no problems related to the surgery at a telephone review at 3 months postoperatively. |
pmc-6509787-1 | The subject was a 75-year-old man with chronic obstructive pulmonary disease for more than 20 years. The patient was a nonsmoker and had no history of other remarkable illnesses. The patient consulted due to 2 weeks of continuous dyspnea without fever, cough, hemoptysis, or chest pain. Lung auscultation revealed no wheezing sounds. Laboratory examinations, including complete blood count, electrolytes, renal function, liver function, and urinalysis, were negative. Chest computed tomography (CT) confirmed the presence of a large mass lesion of 5.8 cm × 4.4 cm that involves the left lower lobe (Fig. a and b). No lymphadenopathy or pleural effusion was observed. No mucosal lesions were identified through flexible fiberoptic bronchoscopy. CT-guided percutaneous transthoracic biopsy was conducted. Histological examination showed numerous compactly clustered small malignant cells with pleomorphism. A high mitotic rate was observed (Fig. c). Several rhabdomyoblasts were observed in the partial area. Immunohistochemistry showed that the cells were positive for desmin and MyoD1 and negative for c-KIT and S-100 protein, which were consistent with the diagnosis of pleomorphic RMS. Extension study was conducted through gastroscopy, colonoscopy, abdominal CT, and bone scan. The results were negative. The patient was referred for entire body fluorodeoxyglucose positron emission tomography (PET)/CT to exclude metastatic tumors. The result demonstrated a fluorodeoxyglucose-reactive large lesion in the left lower lobe with a maximum standardized uptake value of 12.8 without metastatic lesions. The patient could not bear surgical resection because of poor lung function. The patient received two cycles of vincristine/cyclophosphamide/actinomycin D chemotherapy combined with 40 Gy of intensity-modulated radiation therapy (IMRT). The patient opted to stop chemotherapy because of general weakness. After 6 months, abdominal ultrasound revealed large mass lesions in bilateral adrenal glands, which are suspected for metastasis, and the patient discontinued the therapy.
After 9 months, the patient was referred again to our hospital because of intermittent upper abdominal pain with nausea and vomiting. Physical examination showed that a large mass was palpable in the left abdomen. Bowel sounds were active. Laboratory data showed 2.1 mmol/L serum potassium. Abdominal CT showed small bowel dilatation secondary to entero-enteric intussusception (Fig. ). The patient was given gastrointestinal decompression, potassium supplementation, and nutritional support treatment. Subsequently, laparotomy was performed, and a 15 cm segment of non-gangrenous intussusception was found in the jejunum with a 3 cm tumor forming the lead point of intussusception (Fig. a and b). No enlarged lymph nodes were observed in the mesentery of the affected small bowel. No other lesions were detected in the small bowel and colon. The affected segment of the small bowel was resected with end-to-end small bowel stapled anastomosis. Postoperative period was generally predictable. The histological examination of the surgical specimen revealed a pleomorphism malignant cell tumor that involved the mucosa, submucosa, and muscular tissues (Fig. c and d). Immunohistochemical studies were positive for desmin and MyoD1. These findings demonstrated that RMS originated in the lung. To date, the patient survived for 1 year after he was initially diagnosed and is currently under a good general condition. |
pmc-6509847-1 | Case 1 is a 58-year-old Caucasian male with a history of HIV infection (2002), T2DM (2008), and obesity. His comorbidities included hypertension, dyslipidemia, and obstructive sleep apnea. (Table ). Preoperatively, he was prescribed metformin 500 mg twice a day and glycated hemoglobin (HbA1c) was 40 mmol/mol. His baseline body mass index (BMI) was 47 kg/m2, with a weight of 162.9 kg. Multiple attempts at weight loss, including commercial diets and orlistat, had been unsuccessful. HIV prescriptions included one tablet daily of Atripla (efavirenz/emtricitabine/tenofovir). His preoperative CD4 count was 800 cells/μL and viral load was undetectable. Following assessment by the bariatric MDT, he was found to meet criteria for surgery.
In 2012 he underwent laparoscopic AGB surgery and had an uncomplicated postoperative course. Preoperative and postoperative clinical parameters are presented in Tables , , and and Fig. with sustained weight loss reported. As per local guidelines, this patient continued to receive metformin 500 mg twice a day postoperatively to optimize insulin sensitivity. Six months postoperatively, HbA1c was 35 mmol/mol, and there was no evidence of diabetes-related complications. His HIV infection status was not affected by surgery, and he continued to receive Atripla (efavirenz/emtricitabine/tenofovir). His CD4 count was unchanged at each postoperative visit, with undetectable viral load throughout. He continues to be on antiretroviral and antidiabetic medications as well (metformin 500 mg twice a day) and reports sustained weight loss. |
pmc-6509847-2 | Case 2 is a 33-year-old Caucasian male who was positive for HIV (2011) with a background of T2DM, obesity, depression, and fatty liver disease (Table ). His baseline BMI was 50.7 kg/m2 with a weight of 149.8 kg. Following 2 years of orlistat therapy and lifestyle intervention, his BMI decreased modestly to 48.1 kg/m2. Preoperatively, T2DM was controlled with metformin 500 mg once a day and his HbA1c was 35 mmol/mol. Following 2 years of HAART for which he received Atripla (efavirenz/emtricitabine/tenofovir) 1 tablet once a day, his CD4 count increased to 929 cells/μL from 552 cells/μL at diagnosis. Viral load was undetectable. Further preoperative and postoperative parameters are presented in Tables , , and and Fig. .
A laparoscopic SG was performed in 2013. He reported no complications at postoperative follow-up. T2DM was diet controlled following surgery and his HbA1c remained stable (33 mmol/mol mean). Therefore, complete diabetes remission was achieved according to American Diabetes Association (ADA) criteria []. Postoperatively, his viral load remained undetectable with a mean CD4 count of 735 cells/μL. Following clinical trial recruitment, antiretroviral medication was adjusted in an attempt to better stabilize mood. Depressive symptoms improved and HIV status remained stable. |
pmc-6509847-3 | Case 3 is a 48-year-old Caucasian female with a history of obesity, HIV disease (2003), and poorly controlled T2DM with peripheral neuropathy (2003) (Table ). Her baseline BMI was 47.8 kg/m2 and multiple attempts at weight loss had been unsuccessful. Her preoperative HIV status was well controlled (CD4 count 440 cells/μL, undetectable viral load) with Truvada (emtricitabine/tenofovir), darunavir, and ritonavir. Unfortunately, despite various treatments of sodium-glucose co-transporter-2 (SGLT-2) inhibitor, high-dose insulin sensitizer, glucagon-like peptide-1 (GLP-1) agonist, and high-dose basal insulin, her HbA1c remained elevated at 128 mmol/mol. Extensive discussions were undertaken with the patient and the MDT. Despite lack of glycemic optimization, benefits were deemed to outweigh risks and so SG was scheduled.
Preoperative and postoperative clinical parameters are presented in Tables , , and and Fig. . Her T2DM status improved following surgery: HbA1c dropped to 90 mmol/mol 2 years postoperatively (accompanying fasting glucose of 12 mmol/L). Unsurprisingly, given T2DM duration, preceding control, and preoperative insulin requirements, diabetes remission was not achieved in this case. Following surgery, however, she benefits from a reduced pill burden and markedly reduced daily insulin requirements (38 versus 140 units preoperatively). Anti-retroviral medications were switched to Truvada (emtricitabine/tenofovir) and Rezolsta (darunavir/cobicistat) and her HIV status remained stable (CD4 count 400 cells/μL, undetectable viral load). An esophageal stricture which developed 2 years postoperatively responded to a dilatation procedure. No further complications have occurred. |
pmc-6509853-1 | We describe the case of an otherwise healthy 9-y-old boy with a bladder neoplasm, whose clinical history started a year before years with macroscopic haematuria.
The cystoscopy showed the presence at the right ureteral meatus of papillomatous structure (of about 2 cm of diameters), which was entirely removed through transurethral resection (TUR). The histology revealed “urothelial papillary neoplasia with a low degree of malignancy, without infiltration of the sub-epithelial connective tissue”, according to the 2004 WHO/ISUP (World Health Organization/International Society of Urological Pathology classification.
Then, the patient underwent a six-monthly follow-up, with regular clinical and radiologic screening.
However, the ultrasonography of bladder performed one year later revealed a dendriform intravesical tumour of the lateral walls and of the bladder bottom. The cystoscopy confirmed the presence of a multifocal relapse of the disease (Figs. a and b). The lesions appeared superficial and not infiltrating, sited at the lateral walls and the bladder bottom, with a maximum diameter of 3.5 cm. The histological analysis confirmed the prior diagnosis of PUNLMP.
The computerized tomography with urographic scans (uro-CT) excluded any infiltration of the bladder detrusor muscle and the presence of metastatic disease.
Owing to the clinical history, the histology and the stage of the disease, intra-bladder chemotherapy was adopted. The treatment consisted of a first induction phase comprising mono-weekly intra-bladder instillations of Mitomycin-c (MMC) at a dose of 20 mg for a total of 8 weeks. The cystoscopy performed at the end of the induction phase showed the complete regression of the lesions.
Therefore, maintenance therapy was performed with monthly instillations of MMC at the dose of 20 mg for a total of 6 months. The treatment was well tolerated, without significant complications.
After a month, we performed a close follow-up with renal function, renal and urinary ultrasound, urodynamic evaluation, which was found normal.
Two months later, chemical and cytological urinary tests and cystoscopy were achieved and the random biopsies of the primarily affected areas resulted regular. A TUR was performed during the cystoscopy performed at one year.
Afterward, the child was examined with chemical and cytological urinary tests every three months, besides the renal and urinary ultrasound, the urodynamic evaluation and the cystoscopy were performed every six months in the next two years.
Currently, at five years from the end of the chemotherapy, clinical and instrumental follow-up checks detect the absence of the disease and normal urinary function.
PUNLMP is a histopathological entity introduced firstly in the 1998 WHO/ISUP classification.
These neoplasms have a high propensity to local recurrence mostly in the adult population (with a risk of relapse between 40 and 70%), with lower recurrence risk (about 13%) in the young population [].
The treatment varies widely according to the group of risk. The European Organization for Researches and Treatment of Cancer (EORTC) classifies adult patients based on six different prognostic factors: number of lesions and size of the tumour, previous relapse rate, invasiveness of the lamina propria, concomitant presence of carcinoma in situ and histological grading []. Another important prognostic factor is the result of the cystoscopy performed at three months after the TUR [].
Despite the smallness of the cases, the articles published in the last twenty years suggest overlooking intra-bladder chemotherapy after TUR for the paediatric population with PUNLMP. Although this approach, after one year our patient presented a multifocal recurrence with low-grade and superficial lesions, not infiltrating the lamina propria. Because of the features of the recurrence, the patient belonged to the high-risk group and for the multifocality of the lesions, he would have been subjected to radical cystectomy. Although the lack of standardized recommendations for the treatment of multifocal relapsed PUNLMP of the young age, intra-bladder chemotherapy was suggested for saving organ function [].
The therapeutic options currently available for adult patients include immunotherapy with the Calmette-Guerin bacillus (BCG) and intra-bladder chemotherapy with MMC, Doxorubicin and Epirubicin [, ]. Considering the patient’s age and systemic toxicity related to BCG therapy, we decided to perform treatment with endoscopic intra-bladder MMC instillations. The therapy was well tolerated, with complete remission of the disease at the end of the induction phase.
Then, we made a close follow-up, because of PUNLMP of young age, even if less rarely than in adults, may present an aggressive behavior in terms of recurrence and invasiveness [, , ].
Concerning the follow-up, although the absence of shared recommendations, the key-examination remains the ultrasound of the urinary tract, which may reduce the frequency of execution of cystoscopy among the paediatric patients []. Conversely, cystoscopy remains imperative and should be associated with urinary cytology in the cases of recurrent neoplasms [, , ].
Intra-bladder MMC instillation seems a safe and effective therapeutic option for paediatric patients with a high-risk relapsed PUNLMP, allowing to achieve the complete remission of disease and the bladder sparing.
Given the paucity of cases and the lack of treatment and follow-up guidelines, it would be necessary to prospectively validate treatment recommendations and share follow-up programs for pediatric patients with PUNLMP. |
pmc-6509882-1 | An 80-year-old female patient was referred to our hospital for a relapse of PCDLBCL-LT. She was previously treated by systemic immunochemotherapy with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). The first cutaneous lesions of her left leg developed on March 2009. Initial screening by total body computed tomography (CT) showed, other than the multiple nodules of the left leg (Figure A), a mediastinal mass of 70 × 37 mm associated with pleural effusion. The initial histological investigations confirmed the diagnosis of PCDLBCL-LT in both skin lesions and mediastinal mass, with the positivity of CD20, Ki67, and bcl2 (Figure ). After the completion of 5 cycles of treatment, the clinical examination, and total body CT scan showed complete control of PCDLBCL-LT on June 2010 with clearance of the cutaneous and mediastinal lesions. However, the patient developed neutropenia, and the sixth cycle was not performed.
The patient then developed a relapse of both cutaneous and mediastinal lesions in September 2010 (Figure B). The second course of R- CHOP did not control the disease and was responsible for hematological toxicity, including severe neutropenia. Lenalidomide (10 mg/day for 21 days of every 28-day cycle) was proposed on November 2010, associated with a dexamethasone 20 mg on days 1-7-14 monthly.
This treatment was well tolerated and permitted a good control with a complete disappearance of the cutaneous nodules with control of 5 months of her lymphoma. The lymphoma then progressed by the 6th month (on May 2011) with a rapid progression of the mediastinal mass. She deceased on June 2011. |
pmc-6509886-1 | A 70-year-old man with a medical history of hypertension and diabetes was hospitalized to receive intravenous antibiotic due a surgical site infection. The infection started about 3 weeks after a surgical correction of an ascending thoracic aortic dissection. After 10 days of administration of vancomycin, the man presented with multiple tense blisters on oral and genital mucosal. |
pmc-6509887-1 | A 26-year-old Hispanic male presented to the emergency department of our hospital with acute onset of numbness and weakness in his left arm. Shortly after his arrival, he began to experience a pressure like pain in his left upper abdominal quadrant (LUQ) which was nonradiating and moderate in severity. His neurological symptoms resolved within one hour of presentation; however, his LUQ abdominal pain worsened and was associated with repeated vomiting episodes. The patient had history of smoking (2-3 cigarettes per day), drinking hard liquor daily, and taking illicit drugs (cocaine and marijuana).
The initial evaluation included a CT of the head with and without contrast, a CTA of the cervical carotids and intracranial vessels, and MRI of the brain without contrast; all these imaging tests were negative for any acute intracerebral hemorrhage, infarct, or other abnormalities (aneurysm or obstruction) in the vessels of the head or the neck.
Given the nature of the abdominal pain, both noncontrast- and contrast-enhanced helical abdominal CT images were obtained. CTA of renal arteries revealed a left renal infarct with a filling defect.
Transthoracic echocardiogram showed mild to moderate dilated left ventricle with ejection fraction of 55%-60%. The right ventricle was also mildly dilated with grossly normal systolic function. Two oval, mobile, and pedunculated hyperechoic masses, a 2.4 × 1.5 cm in LV and a 2.3 × 1.1 cm in RV, were identified (Figures and ).
A thrombus is identified as a discrete echo-dense mass with well-defined borders which is seen throughout a cardiac cycle.
Coagulation studies including screening, mixing, and confirmatory studies were performed, and the results revealed the presence of lupus anticoagulant (LA) (Table ).
Patient was treated with proper anticoagulants; his symptoms were resolved; unfortunately, patient was lost to follow up. |
pmc-6509891-1 | A 64-year-old Japanese woman was transferred to the hospital due to loss of consciousness. During the anamnesis, she referred a taste disorder whenever she ate that started 10 days before the hospitalization. She had a history of high blood pressure and dizziness but she did not take any medicine for that. She lived with her son and did not visit any hospital for 10 years. Her favorite foods were fruits, pickled vegetables, and coffee. She had an irregular eating pattern.
On the admission day, the patient's temperature was 31.4°C, her blood pressure was 94/60 mm Hg, and her pulse was 73 beats/min. She was 151 cm tall in height, 76.8 kg in weight, and the BMI at that time was 33.7 kg/m2. Her blood test results showed high regarding fasting blood sugar (1348 mg/dL), HbA1c (15.8%), urea nitrogen (100.5 mg/dL), creatinine (3.94 mg/dL), and low levels of serum iron (21.0 μg/dL). We diagnosed her as type 2 diabetes with hyperosmolar hyperglycemic syndrome and dysgeusia. She received treatment with rehydration and insulin injection. Due to anorexia and dysgeusia, she was referred to the department of dentistry in the hospital 4 days after hospitalization.
There were only four teeth left in the right lower mandible, and she was using upper and lower well-fitting dentures (Figure A,B). Her oral mucosa was dry and red. Her tongue was red and smooth in the middle, and there were white lesions on both sides (Figure C). We detected Candida albicans from the white lesions by bacterial examination. Salt-impregnated test results showed lower sensitivity to salty taste. An additional blood test results showed low serum zinc (52.0 μg/dL; lower limit: 57-65 μg/dL). We diagnosed her as candidiasis, median rhomboid glossitis, as the main reasons for her dysgeusia.
By rehydration and insulin injection, her blood sugar and renal function were improved 5 days after hospitalization (Figure A). Nurses taught her as therapeutic exercise to take at least 2000 steps in a day (Figure C). Nutritionist taught her as dietary counseling to take well-balanced diet, such as seafood contained zinc, and avoid overeating (Figure C). In dentistry, dentist and dental hygienists performed oral care, especially tongue cleaning, and bacteria examination routinely (Figure C). The oral conditions and bacterial examination results were shared with the multidisciplinary team. Thirteen days after hospitalization, C. albicansfell below measurable limits, and her median rhomboid glossitis and dysgeusia were improved (Figure ). Twenty-seven days after hospitalization, she was discharged from hospital since her general health condition had been recovered. Ninety-seven days after hospitalization, she visited the hospital due to the check-up of her condition. Her weight was 62.7 kg (−14.1 kg), and BMI decreased 27.7 kg/m2 (−6.2 kg/m2). Her blood test results also showed improvement regarding fasting blood sugar (121 mg/dL), HbA1c (5.8%), urea nitrogen (16.5 mg/dL), creatinine (0.77 mg/dL), serum iron (54.0 μg/dL), and serum zinc (80.0 μg/dL) (Figure A,B). She got used to have regular eating patterns, to eat seafood, and avoid to eat a big quantity of fruits. Now she takes 5000-7000 steps in a day. |
pmc-6509892-1 | A 20-year-old woman was run over by a car, lost consciousness, which was regained after a few seconds, and presented with GCS = 12. She was admitted in the emergency room of our institution and presented with a Glasgow Coma Scale score of 9 out of 15. The CT scan showed a Marshall CT classification of diffuse injury IV because of a right frontotemporal epidural hematoma that was 2.5 cm in diameter and a small fronto-parietotemporal subdural hematoma in addition to a left traumatic subarachnoid hemorrhage, left small brain contusions and fractures of the skull base, right temporal bone and parietal bones (Figure ). She had a Rotterdam classification score of 3. A ventriculostomy was placed in the ICU while the emergency operating room was being prepared for a neurosurgical procedure and the neurosurgery personnel were arriving at the hospital. Her intracranial pressure was 22 mm Hg. An early bilateral fronto-temporoparietal decompressive craniectomy was performed, and the extracranial hematomas were evacuated. The patient was kept under sedation and paralysis, she had her cerebrospinal fluid drainage monitored, and she received osmotic diuresis to achieve an intracranial pressure of approximately 10 mm Hg. The patient presented a right pneumothorax (Figure ), which was resolved with the insertion of a chest tube for drainage. She was extubated after 2 weeks so that her respiratory process and psychomotor dimension could be agitated when the sedation was suppressed for neurological evaluation. After 3 weeks in the intensive care unit, the patient was admitted to the neurosurgical floor, where she received physical therapy. The patient underwent an early cranioplasty repair with an autologous graft 4 weeks after the accident. Six months later, the patient had a score of 8 on the extended Glasgow Outcome Scale, indicating good upper recovery, and a Disability Rating Scale of 0. |
pmc-6509892-2 | A 24-year-old man was injured in a motorcycle accident in which he did not wear a helmet. He arrived at the emergency room of our institution. He was intubated and presented with a GCS score of 8 out 15 and anisocoria (left mydriasis). The CT scan showed a Marshall classification score of IV and a Rotterdam classification of 2. The CT scan showed an open comminute fronto-parietotemporal left fracture and a fronto-parietotemporal subdural hematoma that was 2 cm in diameter; in addition, the CT scan showed a fronto-basal right contusion, a temporal contusion, and a small right temporal epidural hematoma. He also presented with multiple facial fractures that were addressed after the neurological situation was resolved. He underwent a bilateral fronto-temporoparietal decompressive craniectomy 3 hours after the accident (Figure ). We did not place a ventricular drain in this case. We started on the left side to resolve the transtentorial herniation. The mydriasis was reversed after the decompressive craniectomy was performed on the left side. We also evacuated the extracerebral hematomas on both sides. The patient was kept under sedation and paralysis and achieved an intracranial pressure of 8 mm Hg. He also presented with pneumonia (Figure ) that required ventilatory assistance. The patient underwent a tracheostomy. After 4 weeks in the intensive care unit, the patient was admitted to the neurosurgical floor, where he received physical therapy. A cranioplasty repair was performed 9 weeks after the accident. An autologous graft was used on the right side. On the left side, a PEEK customized cranial implant was used. Six months later, the patient presented with an extended Glasgow Outcome Scale score of 8, indicating good upper recovery, and a Disability Rating Scale of 0. |
pmc-6509896-1 | A 90-year-old woman was diagnosed with type 2 diabetes mellitus more than 10 years ago. Her body weight was 41.7 kg (BMI: 20.1 kg/m2). There was no problem with liver function, AST: 24 (10-40) IU/mL, and renal function, creatinine: 0.68 (0.45-0.82) mg/dL. Diabetic complications were not observed. Initially, hemoglobin A1c (HbA1c) level was controlled at 6% by glimepiride (3 mg) and metformin (250 mg); however, she visited our clinic before the scheduled date claiming that “the medicine is gone.” Due to cognitive decline, an overdose of glimepiride and metformin was suspected, and the drugs were discontinued.
The patient was prescribed teneligliptin (20 mg), a dipeptidyl peptidase 4 (DPP-4) inhibitor with low hypoglycemia risk, but HbA1c levels increased to 10.2% within 6 months. Although resuming glimepiride treatment was considered, we opted to administer 2 mg exenatide-LAR once weekly to minimize the risk of hypoglycemia caused by overdosing. Despite declining cognitive function, she visited our clinic weekly for exenatide-LAR injections. HbA1c level rapidly decreased; after 4 months, it reached 7.1% (Figure ). Moreover, good blood glucose control was achieved.
To reduce the dose frequency, we extended the dose interval to 2 weeks and subsequently to 1 month. HbA1c level was in the 6% range when exenatide-LAR was administered every 2 weeks. It was in the 7% range for more than 2 years when administered once monthly. Fasting glucose just before the next injection did not rise. Once-a-month administration allowed the patient to easily receive effective outpatient treatment despite cognitive loss. However, outpatient treatment became difficult after the patient sustained a spinal compression fracture; hence, the drug was administered by a home care provider without issues. During the period of observation, liver and renal functions were not changed. The patient's body weight did not appreciably change, and no adverse events, such as loss of appetite or hypoglycemia, were observed with exenatide-LAR. |
pmc-6509896-2 | An 85-year-old woman was diagnosed with type 2 diabetes mellitus several years ago. Her body weight was 61.0 kg (BMI: 31.1 kg/m2). There was no problem with liver function, AST: 24 (10-40) IU/mL, and renal function, creatinine: 0.54 (0.45-0.82) mg/dL. Diabetic complications were not observed. She was treated with teneligliptin (20 mg), but blood glucose control gradually worsened and HbA1c level increased to 9.7%. Her family informed us that she often forgot to take her multiple medications, which we assumed accounted for the poor blood glucose control.
As the patient did not wish to receive at-home injections, weekly injections of 2 mg exenatide-LAR were administered at our clinic. After switching from teneligliptin to exenatide-LAR, HbA1c level rapidly decreased and was maintained in the 6% range (Figure ). The administration interval was extended to every 2 weeks and subsequently to 1 month. The blood glucose level was well controlled after both extensions. It remained steady for more than 1 year after shifting to monthly administration even though the patient underwent chemotherapy for breast cancer during this time. Only exenatide-LAR was required for blood glucose control. During the period of observation, liver and renal functions were not changed. The patient's body weight did not appreciably change, and no adverse events, such as loss of appetite or hypoglycemia, were observed. |
pmc-6509899-1 | A 58-year-old postmenopausal woman visited our hospital with a palpable lump in the left breast. A movable lump with a clear border and no tenderness was revealed in the outer quadrant of the left breast. The mammogram revealed only round macrocalcification and no mass lesion (BI-RADS: Breast Imaging Reporting and Data System category 1; this mammographic image had not been preserved). Ultrasonography revealed the lump to be a flat and well-defined hypoechoic lesion measuring 0.8 cm in diameter (BI-RADS category 2; Figure A). At the initial visit, following a core needle biopsy (CNB), the lump was diagnosed as a benign epithelial neoplasm. Ultrasonography 2 years later revealed no increase in tumor size. An annual observation during health examination was recommended to monitor the lump. Eight years later, the lump had increased in size, and she visited our hospital again. Ultrasonography revealed a homogenous hypoechoic lesion measuring 1.5 cm with posterior acoustic shadow, slightly taller than wide ratio and lobulation (BI-RADS category 5; Figure B). However, the cytological diagnosis of the CNB did not change. Since malignancy could not be ruled out by the ultrasonographic findings, we recommended the excisional biopsy, but she refused it. In the ninth year, the tumor was 1.7 cm and more lobulated in shape (BI-RADS category 5; Figure C). CNB was performed again, but the diagnosis did not change. She had no symptoms except for the palpable left breast lump. Despite our recommendation, she refused to have an excisional biopsy.
At 10 years, a firm mass measuring 2.5 cm in diameter was observed in the middle outer quadrant of the left breast, with no palpable axillary or subclavicular lymph nodes. Ultrasonography showed that the tumor was a solid irregular lobulated heterogeneous hypoechoic lesion, with a well-defined border, though a part of it was indistinct (BI-RADS category 5; Figure D). While mammography findings delineated a round and circumscribed lesion with round macrocalcification (BI-RADS category 3; Figure ), magnetic resonance imaging (MRI) revealed a lobulated heterogeneous high-intensity mass with a well-defined border localized in the left breast measuring 2.6 cm. We recommended an excisional biopsy again, to which the patient agreed.
Intra-operative findings revealed a firm, movable mass with a clear border. There was no gross surgical injury.
Macroscopically, the tumor measured 2.5 cm in diameter and was a firm, solid mass with areas of cystic and hemorrhagic lesions (Figure A). Microscopically, the tumor consisted of two continuous components (Figure B). One component showed biphasic proliferation of both ductal and myoepithelial cells (Figure C) and was identified as AME. However, the other component showed monophasic proliferation of cells with pale cytoplasm and was similar to the myoepithelium (Figure D). This latter component had several mitoses and foci of necrosis (Figure D). Immunohistochemistry revealed myoepithelial cells in both the components that were positive for HHF35 (Figure A), α-SMA (Figure B), calponin (Figure C), S-100 (Figure D), CD10 (Figure E), and p63 (Figure F), while negative for desmin. Based on these findings, this tumor was identified as AME with myoepithelial carcinoma. Additionally, the myoepithelial carcinoma was found to be negative for estrogen receptor (ER), progesterone receptor (PgR), and human epidermal growth factor receptor (HER) 2. While the Ki-67 index of the myoepithelial carcinoma was 20%, that of the AME was <5%. Following excision, while the AME component had a positive surgical margin, the myoepithelial carcinoma had a clear margin.
Additional excision of the breast with sentinel lymph node biopsy was performed. Pathological findings showed an absence of cancer cells in the excised specimen, and no metastasis was observed in the sentinel lymph node. After surgery, radiation therapy was delivered with a total dose of 50 Gy in 25 fractions. The tumor has neither recurred locally nor metastasized for up to 2 years after surgery. |
pmc-6509901-1 | A 53-year-old man was referred to our institution after a peripheral PAA had been discovered by contrast-enhanced computed tomography (CT) that demonstrated an 8.6 mm diameter aneurysm in the periphery of the right pulmonary artery A10 (Figure A,B). He chose follow-up observation. A CT one year later showed the diameter of the PAA had increased to 9.9 mm (Figure C-E). No abnormality was found on cardiac ultrasound examination, and his tricuspid valve pressure disparity was normal (14 mm Hg). He had the comorbidity of diabetes, which was well controlled. He had a smoking history (Brinkman index 3450); however, his respiratory function was normal. He had no history of infectious diseases such as syphilis or tuberculosis and no history of Behcet disease or Marfan syndrome.
Transcatheter pulmonary artery embolization was performed to prevent rupture of the peripheral PAA. After placement of a 4 Fr introducer sheath in the right femoral vein under local anesthesia, a right pulmonary artery angiogram confirmed the PAA at the branches of A10b and A10c (Figure A). To embolize the draining artery, an AMPLATZER™ Vascular Plug 4 (AVP 4; St. Jude Medical) was used. A10b, one of the draining arteries, was plugged with a 6-mm AVP 4, and A10c, the other draining artery, was plugged with a 7-mm AVP 4 (Figure B). The PAA was embolized with four hydrogel-coated metallic coils, AZUR® CX35 (Azur peripheral hydrocoil; Terumo Medical Corporation) (Figure C). A10b+c, the feeding artery, was plugged with an 8-mm AVP 4. Occlusion of the PAA was confirmed by repeat angiography after embolization (Figure D). |
pmc-6509902-1 | An 18-year-old girl is admitted to cardiology unit for precordial pain and dyspnoea.
The patient appeared on a manual wheelchair for related neonatal suffering, with a scoliotic kyphosis, contraction of the elbows, and hypoactive left upper limb. She presented a hypotrophy of the bicuspid, tricuspid and gastrocnemius muscles and no signs of hemodynamic decompensation. She had negative family history of sudden death, cardiovascular, and neurological diseases. Her electrocardiogram presents a sinus rhythm with alterations of the ventricular repolarization as inverted T wave in V2-V3, poor progression of the r wave in the precordial leads, maybe as a result of kyphosis, and high voltages in the peripheral leads (Figure ). The echocardiogram performed at the time of admission shows asymmetric hypertrophic cardiomyopathy, particularly in the mid-apical anterior wall (24 mm), in the absence of signs of left ventricular outflow obstruction, tricuspid aortic valve, and slight pericardial effusion, especially at anterior level, associated with epicardial fibrin deposits. Mitral, pulmonary, and tricuspid deficiency was mild.
The young patient was born prematurely at 31 weeks of gestation by cesarean section, and immediately after one hour, she developed respiratory distress and hypoglycemia and was admitted to neonatal intensive care and assisted by intermittent positive pressure ventilation. During the hospitalization, she was subjected to five blood transfusions for anemia, neonatal jaundice phototherapy and antibiotic and antifungal therapies for candida infection. The perinatal encephalic ultrasound allowed to identify small hemorrhage at the level of the caudate nucleus, bilateral peritrigonal hyperechogenicity, and an inhomogeneous area in the right thalamo-striatal region of probable hypoxic-ischemic nature. She was discharged from the neonatal hospital after 68 days with diagnose of prematurity and moderate asphyxiation.
At two years old, the child begins to present bilateral sensorineural hearing loss that suddenly gets worse after three years, for which she is performed to right cochlear implant and left retroauricular prosthesis.
The many brain MR performed before the implant showed a stationary neuroradiological image of alterations in bilateral periventricular and peritrigonal cerebral white matter by perinatal suffering.
Her clinical history and her lower limbs spasticity have led doctors to diagnose spastic diplegia and to start physiotherapy and orthosis treatments associated with focal therapy with botulinum toxin to reduce hypertonicity. During the years, physicians have witnessed a progressive worsening of walking, from a double support to the use of walker associated with orthopedic and orthotic shoes, and disturbed by equinovarism of the feet and global internal rotation of the lower limbs. At ten years, Achilles tendon extension surgery was also necessary to improve the gait of the small patient.
During cardiology admission, her laboratory tests were all within normal limits, including the serum CK level (69 U/l, normal range 26-192). The Holter electrocardiogram showed rare single supraventricular extrasystoles and some monomorphic ventricular extrasystoles, with superior axis and right bundle branch block morphology, as arising from the left ventricle. Spirometry found a moderate degree of restrictive ventilatory deficit.
Given the complex clinical conditions, a genetic counseling was also recommended for the patient. She and her parents were recruited for a genetic screening by next-generation sequencing (NGS) approach. |
pmc-6509919-1 | A 59-year-old woman presented with severe long-term RLS after treatment with pramipexole and pregabalin, which had to be stopped due to side effects. At presentation, she was treated with rotigotine, a dopamine agonist in the form of a patch, with the additional possibility of taking L-DOPA in acute phases of the disease and magnesium if needed. Baseline PSG showed low sleep efficiency (67%) with many arousals (22/h) and many short phases of wakefulness in which she frequently moved because of restlessness. During sleep, there were only few periodic leg movements (PLMS score 6/h) and light obstructive sleep apnea (apnea-hypopnea index 7.5/h, oral desaturation index 8/h). She started add-on treatment with BP 50% tablets at four tablets per day (0-0-2-2) without changing other medication. As shown in Figure , PSG performed approximately 2.5 months thereafter and still under treatment revealed clear improvements in sleep architecture; sleep efficiency markedly improved (from 67% to 90%), PLMS index was very low (1/h). In her subjective feedback about this recorded night, the patient assessed her sleep as very good. In general, the patient reported a marked improvement of RLS symptoms and of the ability to sleep, but with severe phases of restlessness and sleepless nights still experienced from time to time. |
pmc-6509919-2 | A 65-year-old woman presented with severe insomnia and panic attacks, treated with trimipramine, a tricyclic antidepressant. Additionally, she was suffering from prediabetes and cardiovascular disease. Baseline PSG revealed disturbed sleep architecture with a high prevalence of periodic leg movements during sleep (PLMS index 76/h). An exact sleep history revealed that she suffered from restless legs in the evening and at night. The symptoms had started in late childhood and had slowly worsened over time. At the time of baseline PSG, RLS symptoms were quite bothering (IRLS 28/40). After the baseline PSG, the patient stopped trimipramine treatment spontaneously, which resulted in a reduction of RLS symptoms (IRLS decreased from 28/40 to 20/40). Then she started treatment with BP (50% chewable tablets, 0-0-3-3). After 2 months of BP treatment, the patient described a significant improvement in sleep, less suffering from restless legs during the early part of the night, less nocturnal awakening, and greater restorative value in the morning. The patient seemed to benefit markedly from treatment with BP. The RLS improvement perceived by the patient was apparent in the change of her IRLS score (from 20/40 before treatment down to 9/40) and in the reduction of the PLMS index as calculated from the nocturnal PSG (from 76/h down to 41/h). |
pmc-6509919-3 | A 53-year-old woman presented with insomnia, likely related to psychophysiological factors for which she had been regularly taking the benzodiazepine medication lorazepam at a low dose. She had had sleep problems for 10 years and now complained of insomnia symptoms and was suffering from fatigue. She also suffered from RLS (IRLS 18/40), which had worsened under treatment with mirtazapine and improved with pramipexole in the past, but was currently not being treated. Once a week, she would stumble and suddenly fall down, events most probably caused by side effects of lorazepam. In her opinion, RLS complaints were not the main cause of her sleep disorder. During the first consultation, the patient was told to stop taking lorazepam, which she did. Four weeks later, the baseline PSG was performed, revealing markedly fragmented sleep architecture. Chewable tablets with 50% BP (six tablets per day; 0-0-3-3) were recommended to the patient. After well-tolerated treatment with BP for 2 months, no major changes neither in the objective PSG-outcomes (see Table ), nor in perceived insomnia, or restless leg discomfort were observed. The patient reported, however, that the course of symptoms had varied markedly: During BP treatment, the falling episodes became less frequent and she was able to continue without taking any benzodiazepine medication, which she viewed positively. |
pmc-6509919-4 | A 59-year-old man presented with severe sleep problems, low sleep quality, and daytime sleepiness. He did not have any medical history and was on no medication. PSG showed severely fragmented sleep architecture due to frequent periodic leg movements (PLMS score 88/h). A detailed sleep history revealed that he suffered from typical RLS (IRLS 20/40). He complained about waking up frequently during the night and having difficulties falling asleep again because of a disturbing restlessness in his legs. He was not taking any medication. Treatment with BP (50% tablets at four tablets per day; 0-0-2-2 tablets) was initiated. Approximately two months later, the patient reported significant improvements in night sleep, no further RLS symptoms (IRLS 2/40) and better restedness in the morning. Comparison of the PSG outputs at before and after BP treatment revealed that sleep efficiency had improved (from 67% up to 88%), arousals and waking phases during the night had decreased (arousal index 21/h compared to 78/h before treatment), and the PLMS index slightly decreased (from 88/h to 73/h). |
pmc-6509919-5 | A 41-year-old woman presented with chronic insomnia that had lasted for years and had become worse in the last 1.5 years. In addition, she had had multiple sclerosis for approximately 9 years. About one year before, she was found to have RLS and her husband reported nocturnal leg movements, which could be PLMS. At presentation, the patient was being treated with the selective serotonin re-uptake inhibitor (SSRI) citalopram for previous depression. According to the patient, RLS symptoms appeared about five times per week, mostly before falling asleep, sometimes also during awakening (IRLS 20/40). Her subjective sleep quality was low, which translated into a pathologically elevated PSQI; it was 12, whereas people without sleep disorders exhibit values up to 5. A PSG revealed disturbed sleep architecture, associated with a high PLMS index (30/h). After the PSG, BP treatment was suggested. Since the patient had a lactose intolerance, she did not want to take the 50% chewable tablets and was offered the possibility of taking a BP 33% tincture, which she accepted (at 0-0-20-20 drops per day). After 2 months' treatment, the patient reported that she felt better during the day and that the RLS symptoms at night occurred only rarely (IRLS reduced from 20/40 to 10/40). This is in line with the lower PLMS index as measured during PSG (from 30/h to 20/h, see also Figure ) and with the lower value of the PLMS scale from the Douglass questionnaire. Furthermore, her sleep quality had improved markedly (the PSQI went down to 4). When the patient forgot to take the BP medication (about one day every second week), she woke up between 2 and 3 o'clock and had difficulty in falling asleep again, even though she was in bed as usual between 11:00 pm and 6:00 am The fatigue typical for multiple sclerosis was still there, but the perceived tendency to fall asleep was less pronounced, she did not always need a daily nap and had more energy. After two months' treatment, the patient decided to continue to take BP 33% tincture. Fourteen months later, the patient was asked if she was still regularly taking BP 33% tincture; she was, and was still very positive about the effect. |
pmc-6509920-1 | A 74-year-old man who presented with symptomatic adenosine-sensitive supraventricular tachycardia underwent electrophysiology study and ablation.
The procedure was initially conducted using three-dimension electroanatomic mapping system (Ensite Precision™ Cardiac Mapping System, St Jude Medical Inc, St Paul, MN, USA) without the use of fluoroscopy.
Three catheters were used for the electrophysiology study via right femoral vein [Livewire 6 French (F) decapolar catheter (St Jude Medical Inc, St Paul, MN, USA) was placed at coronary sinus, Avail Josephson 6F quadripolar catheter (Johnson & Johnson Medical Inc, New Brunswick, NJ, USA) was placed at right ventricular apex and CRD-2 6F quadripolar catheter (St Jude Medical Inc, St Paul, MN, USA) was placed at His].
After completing electrophysiology study, we were unable to withdraw the quadripolar catheter. On fluoroscopy, the quadripolar catheter was found to be knotted. The knot measured 6.7 mm by 4.7 mm (Figure ).
We considered different options to remove the knotted catheter. One option was to remove the knotted catheter by right femoral vein cut down at the puncture site. Another option was to snare the knotted catheter by gaining assess on the contralateral femoral vein using a larger sheath (at least 21F internal diameter). Both methods may potentially cause vascular damage and preclude subsequent ablation procedure. We decided to unravel the knot using a percutaneous approach. To achieve this, we needed to get through the center of the knot and exert forces in opposite direction to unravel the knot.
A long sheath (SRO, 8.5F) together with dilator and stiff guide wire (0.025″ in diameter and 180 cm in length) was inserted via the right femoral vein. The guide wire was then maneuvered through the knot followed by the dilator and sheath while maintaining traction on the quadripolar catheter (Figure A) under fluoroscopy guidance. Next, using the retained wire technique (after retracting both the long sheath and dilator below the knot), another SRO long sheath and wire was used to cross the knot. The advancement of both long sheaths with dilator across the knot further “opened it up” (Figure B). Next, a deflectable ablation catheter was inserted through one of the long sheaths to exert traction on one side of the knot by fully flexing the ablation catheter. For the other long sheath, the tip of the sheath was caught against the other side of the knot (dilator was removed but stiff wire was retained across the knot) providing counter-traction. Using traction and counter-traction method, we attempted to unravel the knot. However, it was unsuccessful as the deflected ablation catheter alone did not provide sufficient traction to unravel the knot.
In order to improve traction, the SRO sheath was replaced with an Agilis™ (St Jude Medical Inc, St Paul, MN, USA) steerable sheath (8.5F/91 cm). Using an ablation catheter through Agilis™ sheath followed by full deflection of Agilis™ sheath, we were able to exert enough traction while maintaining counter-traction with the other SRO sheath tip to unravel the knot (Figure ).
We were then able to proceed to perform a successful ablation of the concealed right anterolateral accessory pathway. Eventually, the additional fluoroscopy time and radiation dose required to guide the unraveling of the knotted catheter was estimated to be about 30 minutes and 80 000 mGy/cm2, respectively. |
pmc-6509921-1 | A 54-year-old man came to the private dental clinic with complaint of difficulty in mastication and esthetical concern for his upper anterior teeth. He was a nonsmoker and was diagnosed with IgG-kappa type MM in November 2011. In the physical examination, he was diagnosed with MM. Bony metastasis was present at the time of diagnosis of the disease. A full radiographic skeletal survey showed multiple bony lesions at the ribs, femurs, and hip (Figures and ).
Panoramic view revealed bony lytic and punch out lesions at the right side of the mandible. This patient had no history of surgery. His weight had decreased by 7 kg, following 22 months of acute intravenous injection (IV) BP treatment after the last chemotherapy treatment session. His blood pressure was 130/80, and he had a normal breathing and pulse rate. Preoperative examination of his oral mucosa revealed no evidence of pathological lesions, and overall oral hygiene was good. The patient was felt healthy and was well nourished, alert, and cooperative. After thorough clinical examination, maxillary right first premolar was found missing.
After meticulous consulting sessions with the patient and discussing the advantages and disadvantages of all treatment options, he accepted to receive dental implant.
According to the patient's physician, the appropriate time for the surgery relied upon the patient's regular blood cell counts. This patient did not undergo any radiotherapy phases in the entire duration of his active IV BP treatment. He underwent chemotherapy for two separate sessions. After the last session of chemotherapy, the patient received monthly infusion of 3.5 mg of the IV BP drug zoledronate (Zometa; Novartis Pharmaceuticals Corporation) for a period of 22 months (from May 2014 to March 2016). As per the physician's recommendation, C-terminal cross-linking telopeptide (CTX) examination was carried out 6 months after stopping IV BP therapy. The CTX above of more than 150 was considered to be safe, in that the CTX was 289 pg/mL.
Before surgery, the patient was premeditated with 2 g of amoxicillin/clavulanic acid and 50 mg of diclofenac. A root form titanium dental implant (Superline; Dentium) of 3.6 mm in diameter and 10 mm in length was inserted under local anesthesia (Figure ). The patient well-tolerated the procedure and his vital signs were regularly monitored. Postoperative medications including antibiotics (1000 mg amoxicillin/clavulanic acid twice daily for 7 days, starting on the day of surgery), an analgesic (600 mg ibuprofen as required every 6 hours), and mouthwash (0.2% chlorhexidine twice daily for 2 weeks, starting on the day after surgery) were prescribed to the patient. Postoperative course and healing were unremarkable and typical. He was instructed to resume normal oral hygiene and chewing by week six. Postsurgical cleaning protocols, including oral hygiene instructions, were implemented at weeks 1, 2, 6, and 12.
Four months after the implant insertion, the patient returned for punch removal of the gingiva overlying the implants. After 1 week, the appropriate impression copings were connected to the fixture. Polyether (Permadyne light and regular body; ESPE, Plymouth Meeting) was injected around the transfer copings and placed inside the custom tray using the dispenser. After laboratory procedure, abutment were positioned and torqued according to the manufacture's guidelines at 30 Ncm. After the surgical and prosthetic treatments were completed on February 2017, the patient was placed on a regular follow-up for peri-implant maintenance. The patient resumed IV BP therapy on May 2017. The oral hygiene regimen was implemented for this patient in a 6-month recall. The last follow-up (12 months after prosthetic delivery) showed minimum bone loss, as compared with the X-rays taken immediately after the prosthetic delivery and the implant, and its restoration was successful. The patient was satisfied with the treatment (Figure ). |
pmc-6509922-1 | In April 2012, a previously healthy 12-year-old female presented with a malar rash (Figure A). Menarche had started at 11 years of age, and the patient had been vaccinated according to the national Portuguese vaccination program including the first dose of the human papilloma virus vaccine, administered 1 month before symptom onset. The clinical characteristics, histological reports, treatments, and outcome are presented in chronological order in Tables and . A skin biopsy (Figure A) was reported as compatible with a diagnosis of lupus. More specifically, there was a thin epidermis, the basement membrane was not thickened, and a mild perivascular lymphocytic infiltrate and focal vacuolization were found at the dermoepidermal junction. Edema, vessel ectasia, a mild perivascular lymphocytic infiltrate, and mucin deposits were found in the reticular dermis and a lymphocytic infiltrate surrounded hair follicles. At that time anti-SSA antibodies were present, but there were no other abnormalities in the full blood count, renal function, or urinary sediment. There was improvement with topical hydrocortisone, tacrolimus, and photoprotection. One month later, the patient developed fever and lost 1.5 kg in weight, and 3 months later, the rash on the cheeks returned (Figure B). Repeat biopsies in the malar region were performed in July 2012 but a tissue orientation error prevented interpretation. At that time, a lupus band test from unaffected skin revealed the presence of IgM and IgG granular deposits in the basement membrane. Hydroxychloroquine (HCQ) 400 mg/d was started and the rash improved (Figure C). Despite HCQ, in December 2012, symmetrical painful violaceous lesions appeared on the tip of the fingers and toes. These resolved with deflazacort 30 mg/d for 1 week, progressively discontinued in the following 3 months. In June 2013, still on HCQ, worsening of the malar rash was documented. In April 2014, the patient reported the onset of pruritic well-defined hyperkeratotic papules initially in the lower limbs, rapidly spreading to the buttocks, upper torso, arms, palms of hands and scalp, resulting in severe alopecia (Figure D). The complete full blood count, hepatic and renal function tests were within normal ranges. A more extensive profile revealed ANA positivity (1/1280), with an elevated anti-dsDNA, a low C4 and C3. The patient was then treated with daily deflazacort 30 mg, azathioprine (AZA) 50 mg and anti-histaminics, with no improvement. At that time, scabies was suspected and topical treatment with benzyl benzoate was prescribed on two occasions. Several scalp punch biopsies in September 2014 (Figure B) were reported as compatible with lupus, folliculitis being reported in one of the samples (Figure C). No periodic acid-Schiff (PAS) positive microorganisms were identified, and there was no immunoglobulin deposition by direct immunofluorescence. The skin condition progressively deteriorated, and both deflazacort and AZA were discontinued. Several discordant histological diagnosis of perforating dermatosis (Figure D) and psoriasis (Figure E) ensued. The patient was then treated with oral isotretinoin, whole body psoralen, and ultraviolet-A light therapy (PUVA), 3 times a week (oral 8-Methoxsalen administered before each session with initial, final and total doses of 1.5, 9, and 29.5 J/cm2, respectively). These treatments were harmful and stopped after eleven sessions due to the development of generalized, erosive, painful and extremely pruritic disseminated cutaneous lesions with severe alopecia (Figure E), after which the patient was admitted to our unit in July 2015. Laboratory tests showed leucopenia (3100/μL), neutropenia (1680/μL), ANA positivity (1/640), anti-dsDNA antibodies (277 IU/mL; ELISA reference: <25 IU/mL), complement consumption (C3 = 61 mg/dL [normal range: 90-180 mg/dL], C4 = 5 mg/dL [normal range: 10-40 mg/dL]), and sustained proteinuria (highest value: 1006 mg/24 h). ELISA tests for anti-Beta-2 Glycoprotein1 and anti-cardiolipin antibodies as well as the lupus anticoagulant assay were negative. The renal biopsy revealed class V membranous glomerulonephritis with granular deposits of immunoglobulins, complement components, and light chains (Figure ); tissue and serum anti-Phospholipase A2 receptor antibody were negative. In view of her skin condition, off-label intravenous immunoglobulin (IVIG) was administered (20 g/d × 5 days) together with HCQ 400 mg/d, and mycophenolate mofetil (MMF) was started at the dose of 500 mg bd and increased weekly by 250 mg bd to a maximum dose of 1 g bd, together with enalapril 5 mg/d. On the 20th day of hospitalization due to the ongoing severity of the skin lesions, the patient was treated with rituximab (RTX) 1 g preceded by methylprednisolone 500 mg, on days 1 and 15, in addition to the above-mentioned drugs. The skin rash resolved within 2 weeks of the RTX administration, with residual hypopigmentation (Figure C); full hair re-growth was documented at 6 months (Figure D) with well-being and sustained renal remission at 3 years of follow-up, allowing for successful medication taper (Figure ), continuing HCQ and MMF as maintenance treatment. |
pmc-6509923-1 | A 28-week-old premature boy, with a birthweight of 1280 grams, was intubated with a 2.5 mm endotracheal tube via the nose and ventilated for severe hyaline membrane disease (HMD). After receiving two doses of surfactant, the premature neonate was successfully weaned off ventilation and extubated to nasal continuous positive airway pressure (CPAP). On day 9, his clinical course was complicated by a pulmonary hemorrhage, requiring re-intubation. He was given another dose of surfactant and stabilized on high-frequency oscillation ventilation (HFOV). The chest radiograph showed extensive bilateral pulmonary interstitial emphysema, with the left side more extensively involved in comparison to the right. A hemodynamically significant patent ductus arteriosus was treated by intravenous paracetamol. The baby's condition did not improve, and he was selectively intubated into his right main bronchus. The position of the endotracheal tube was radiologically confirmed, allowing the right lung to be oscillated while the left lung was rested. The baby was nursed on his left side for a period of 36 hours, after which the endotracheal tube was retracted into the trachea and secured in that position. The baby's ventilatory status subsequently improved, allowing for extubation on day 7 after the relapse. On day 28 of life, he presented with severe stridor, requiring re-intubation. Difficulty during intubation suggested that subglottic stenosis might be present. One week after this re-intubation a flexible bronchoscopy was performed, which revealed two major findings. The first was a Cotton grade 2 subglottic stenosis, and the other abnormality was near-complete obstruction of the bronchus intermedius. The subglottic stenosis was dilated to 5 mm with the aid of a balloon dilator (Boston scientific Mustang™ balloon dilatation catheter). The bronchus intermedius was extremely narrow, and a 2.2 mm flexible bronchoscope was not able to pass through the area of stenosis.
After 2 weeks, the bronchoscopy was repeated and the subglottic region had improved to near normal in diameter. The bronchus intermedius stenosis, however, remained unchanged. A chest Computed Tomography (CT) scan was performed to determine the length of the bronchial stenosis. The CT scan confirmed that the stenosis involved a short segment and had a web like in configuration (Figure ).
It was decided to balloon dilate the stenosis under fluoroscopy. As the cardiac catheterization suite offered the best quality fluoroscopy, it was decided to perform the dilatation in the suite. The baby was intubated, and a guidewire was inserted into the right main bronchus under fluoroscopy. Water-soluble contrast was injected, and the position of the airway identified. A 3.5 mm coronary artery balloon catheter was inserted into the area of stenosis via the guide wire, and the position was confirmed by fluoroscopy. The balloon was inflated at 16 atmospheric pressure for 20 seconds. This was repeated for another 20 seconds before water-soluble contrast was reinjected, demonstrating significant decrease in the bronchial stenosis (Figure ). Following the procedure, the baby was ventilated for less than 24 hours. At follow-up bronchoscopy 2 weeks later, the stenosis had significantly improved, allowing a 2.8 mm flexible bronchoscope to pass comfortably through the stenotic region. The posterior part of the stenosis had completely resolved, with a small anterior shelf remaining. The baby was discharged with no known respiratory complications, and at follow-up bronchoscopy 6 weeks, after the latter dilatation procedure, the airway remained patent and the baby remained asymptomatic, with a normal chest radiograph. Follow -up bronchoscopy was done due to the risk of restenosis and the fact that the baby was from a rural area, with limited medical services. |
pmc-6509924-1 | A 66-year-old man with extensive lymphadenopathy (chest, abdomen, and inguinal lymph nodes) and splenomegaly was diagnosed with FL, predominantly grades 1-2 with focal areas of grade 3A, Stage IV, with high-risk FL International Prognostic Index 2 (FLIPI2) score. He had a good response to bendamustine and rituximab. Three months into his treatment, he noticed left eye swelling and visual disturbance. Clinical examination revealed erythematous left orbit with restricted extra-ocular movements and elevated Intraocular pressure (IOP). MRI was highly concerning for orbital cellulitis with a secondary anterior displacement of left globe. In the emergency room, ophthalmology performed canthotomy and cantholysis to decrease IOP and broad-spectrum antibiotics were initiated for suspected orbital cellulitis.
On the following day, orbitotomy revealed a firm mass compressing the optic nerve. An excisional biopsy of this mass was obtained. Subsequently, the patient received steroids due to optic nerve compression and antibiotics were discontinued. He later received radiation therapy the following day. Excisional biopsy of the orbital mass revealed a B-lymphoblastic lymphoma (Table ; Figure ), entirely comprising of blastoid B cells, positive for CD19, CD10, TdT, CD38, negative for CD3, CD5, CD11c, and CD20 with Kappa light chain restriction (partial, very dim). Cytogenetics/FISH analysis also demonstrated simultaneous presence of MYC and BCL2 translocation. In light of the patient's history of FL, this B-lymphoblastic leukemia/lymphoma is presumably a transformed lymphoma. Further diagnostic workup showed the cerebrospinal fluid involvement by B-lymphoblastic lymphoma. There was no evidence of lymphoma/leukemia in the bone marrow. Subsequently, he received intrathecal cytarabine and methotrexate for leptomeningeal carcinomatosis. He was also started on systemic chemotherapy with HyperCVAD (cyclophosphamide, Vincristine, Adriamycin, and dexamethasone). Unfortunately, the patient could not tolerate intensive chemotherapy after two cycles of therapy and decided to enroll in hospice care. |
pmc-6509927-1 | A 27-year-old black male was admitted in the medical ward with a 1-month history of dyspnea and 2 days history of hemoptysis. He also reported a one-year history of a painless left testicular mass. He had no history of undescended testes nor a known family history of testicular cancer, no backache. On examination, he was dyspneic, with oxygen saturation of 83% on room air for which a facemask oxygen was placed and had a marked gynecomastia. A firm, irregular, and nontender left testicular mass measuring about 10 cm × 7 cm was noted. No neurological deficit was present on admission. He was transferred the following day to the urology ward where he spent less than 24 hours before his urgent referral to the oncology department away from our institution.
Initial investigations showed the following:
Cannon ball lesions on chest X-ray (Figure ); Hemoglobin (Hb) = 4.9 g/dL (normal range = 13.4-17.5); Beta-human chorionic gonadotropin (βhCG) = 807 593 IU/L(normal range = 0); Alpha-fetoprotein (AFP) = 2.4 μg/L (normal range = 0.0-7.0); Lactate dehydrogenase (LDH) = 1052 U/L(normal range = 48-115); Calcium = 1.69 mmol/L (normal range = 2.15-2.50); Alkaline phosphatase = 57 U/L(normal range = 53-128).
Histopathology assessment post radical orchidectomy revealed on macroscopic examination the presence of an enlarged left testis that measured 100 × 60 × 55 mm. The cut surface had a nodular appearance with areas of hemorrhage, necrosis, and solid gray tumor nodules. The tunica appeared intact, and the spermatic cord appeared to be uninvolved. Microscopic examination confirmed the presence of a mixed germ cell tumor composed of a choriocarcinoma (90%) and classic seminoma (10%). The choriocarcinoma was composed of cytotrophoblast, intermediate trophoblast, and syncytiotrophoblast (Figure ). There was extensive hemorrhage and necrosis. The seminomatous component comprised sheets of clear cells which displayed prominent nucleoli (Figure ). Prominent lymphovascular space invasion was evident. Areas of intratubular germ cell neoplasia were also seen. Immunohistochemical staining performed was CD117 which was in seminoma component of the tumor (Figure ).
He was transfused to an Hb of 8.2 g/dL prior to surgery. Bronchoscopy could not be performed due to patient's breathing difficulties. Radical orchidectomy was done under spinal anesthesia due to lung metastases. Six hours postoperative, he developed an acute weakness and numbness of both lower limbs after he had recovered from spinal anesthesia. On subsequent examination, both lower limbs had no tone; power: 0/5; absent reflexes bilaterally; no sensation; and a sensory level of T4. He also had a decreased anal tone and loss of bladder control.
Urgent MRI spine was done and showed a thoracic epidural macrolobulated and heterogeneous mass with predominant T2W hyperintensity causing spinal cord compression from T3 to T6 (Figure ). MRI findings are also suggestive of a possible intralesional hemorrhage. Postoperative βhCG = 815 815 IU/L. He was sent for urgent radiation and chemotherapy while on corticosteroids. He received eight Gray of emergency radiation therapy to thoracic spine (T2-T8) and completed one cycle of bleomycin, etoposide, and cisplatin. Patient demised a few days later due to progression of disease. |
pmc-6509929-1 | Case 1 was a 35-year-old (height, 156 cm; weight, 56 kg; BMI, 23 kg/m2) pregnant woman. She was 6 months and 26 days pregnant, with no history of smoking or childhood asthma. She lived on an outlying island of Japan close to northwestern Okinawa Main Island. She was diagnosed with bronchial asthma 7 years earlier, at which point treatment with salbutamol and inhaled steroids was initiated. She had recently developed common cold symptoms with yellow sputum. On presentation, she had a 1-day history of wheezing. She presented to a local clinic, where she received oxygen therapy via mask (5 L/min) and hydrocortisone (100 mg). However, the treatment did not alleviate her symptoms. She subsequently developed low oxygen saturation levels and was transported by helicopter to the emergency department. She was fully conscious and cooperative upon admission to the emergency department. Her main symptoms were dyspnea (Borg scale severity level 7) and tightness in the chest. Her wheezing was categorized as Johnson classification degree II. Her respiratory rate was 28 breaths/min. She was not able to lie down and remained in an orthopneic position. Her body temperature was 36.9°C (98.42°F). Serum C-reactive protein (CRP) level was 2.43 mg/dL; white blood cell (WBC) count was 20 400 cells/µL. NPPV (V60 Ventilator; Respironics Inc, California, USA) was initiated due to hypoxia (P/F ratio, 163) using an NPPV mask (5 L/min; Confortgel Blue Nasal Mask, Respironics Inc). A nurse with expertise in respiratory care attached NPPV while assessing for the possibility of air leak. NPPV settings used were as follows: S/T mode; inspiratory positive airway pressure (IPAP), 7 cm H2O; expiratory positive airway pressure (EPAP), 4 cm H2O; inspiratory time (I-time), 1.0 seconds; and inspired oxygen fraction (FiO2), 50%. Hydrocortisone (200 mg) was administered via intravenous drip, and a salbutamol metered-dose inhaler was incorporated in the NPPV circuit using a respiratory gas mixer (Aero Chamber MV, Trudell Medical International, Canada). Figure shows changes in P/F ratio, PCO2, respiratory rate, heart rate, and Borg scale levels at 30 and 60 minutes after the initiation of NPPV therapy. Her P/F ratio dramatically improved during the first 30 minutes along with improvements in PCO2, respiratory rate, heart rate, and Borg scale classification. After the initiation of NPPV therapy, her wheezing was resolved at auscultation. He subsequently shifted from the orthopneic to Fowler's position. Fetal heart rate (FHR), as assessed by the obstetrician using Doppler echocardiography, was 152 beats/min. She was transferred from the intensive care unit (ICU) to the general ward. Oxygenation was stabilized to a level that can be coped with low-flow oxygen therapy, even if NPPV is temporarily discontinued after the P/F ratio is improved. Moreover, re-exacerbation of wheezing, dyspnea, tachypnea, and vital signs was not observed even if the NPPV mask was removed. Finally, NPPV and oxygen therapies were withdrawn. Bronchitis was suspected to have triggered the acute asthma attack, and she was treated with antibiotics. Inhaled steroids were administered. She was discharged on day 4 post admission. After discharge, her asthma was well controlled, and she was followed up by the doctor at the original clinic, under the advice of a respiratory specialist.
Finally, NPPV and oxygen therapy were withdrawn. Bronchitis was suspected to have triggered the acute asthma attack, and the patient was treated with antibiotics. Inhaled steroids were administered. She was discharged on day 4 post admission. After discharge, her asthma was well controlled, and her follow-up was conducted by the doctor at the original clinic, under the advice of a respiratory specialist. |
pmc-6509929-2 | Case 2 was a 29-year-old (height, 149 cm; weight, 53 kg; BMI, 24 kg/m2) pregnant woman. She was 7 months and 2 days pregnant, with a breech presentation. She was a never smoker, with a history of childhood asthma. Pulmonary spirometry revealed the following: forced vital capacity (FVC), 2.5 L; forced expiratory volume % in 1 seconds (FEV1), 1.65 L; and FEV1/FVC ratio, 0.66. She was previously prescribed inhalation therapy with fluticasone; however, she discontinued treatment on her own. She complained of fatigue during childcare over the preceding week. On presentation, she developed symptoms of wheezing and dyspnea at approximately noon. She was subsequently transported to the hospital by ambulance.
On physical examination, she was fully conscious and cooperative. Her main symptoms were dyspnea (Borg scale severity level 9) and wheezing (Johnson classification degree III); her respiratory rate was 36 breaths/min. She was in an anteflexion position. Body temperature was 36.9°C (98.42°F). Serum CRP level was 0.72 mg/dL; WBC count was 122 00 cells/μL. Further deterioration of symptoms compelled the medical team to consider tracheal intubation. NPPV was initiated using a reservoir oxygen mask (10 L/min) to address the patient's hypoxia (P/F ratio, 141). NPPV settings used were as follows: S/T mode; IPAP, 8 cm H2O; EPAP, 4 cm H2O; I-time, 0.8 seconds; and FiO2, 60%. A nurse with expertise in respiratory care attached the NPPV mask and made the necessary adjustments to prevent air leak and patient discomfort. Methylprednisolone (40 mg) was administered via intravenous drip. Magnesium (20 mEq) was injected intravenously. A hypodermic injection of adrenaline (0.3 mg) was also administered.
Figure presents changes in P/F ratio, PCO2, respiratory rate, heart rate, and Borg scale classification at 30 and 60 minutes after the initiation of NPPV therapy. Borg scale classification and respiratory rate dramatically improved over the first 30 minutes along with improvements in P/F ratio, PCO2, and heart rate. Under NPPV therapy, her wheezing improved to Johnson classification degree II at auscultation, and she shifted from the anteflexion to Fowler's position. FHR, as assessed by an obstetrician using Doppler echocardiography, was 146 beats/min. She was subsequently transferred from ICU to the obstetric ward. Oxygenation was stabilized to a level that can be coped with low-flow oxygen therapy, even if NPPV is temporarily discontinued after the P/F ratio is improved. Moreover, re-exacerbation of wheezing, dyspnea, tachypnea, and vital signs was not observed even if the NPPV mask was removed. NPPV therapy was discontinued, and oxygen therapy was initiated. She was treated with sultamicillin tosilate hydrate (1500 mg) for a suspected infection. Ultimately, oxygen therapy was also withdrawn, and she maintained percutaneous oxygen saturation (SpO2) of 97% on room air. She was discharged on day 5 post admission with a prescription for prednisolone (30 mg) and a budesonide dry-powder inhaler. She safely delivered a female child weighing 2490 g (Apgar score 9/9) at gestational age of 9 months and 1 day via cesarean section performed under lumbar anesthesia. After discharge, she was treated as an outpatient by a respiratory specialist. She has continued to adhere to the prescribed regimen, and her asthma remains well controlled. |
pmc-6509929-3 | Case 3 was a 20-year-old (height, 158 cm; weight, 51 kg; BMI, 21 kg/m2) pregnant female. She was 9 months and 2 days pregnant. She was a never smoker, with a history of childhood asthma and allergic rhinitis. Pulmonary spirometry revealed the following: FVC, 2.45 L; FEV1, 0.96 L; and FEV1/FVC ratio, 0.39. She was treated for asthma until 17 years of age; however, the treatment was then interrupted. She had developed influenza 2 weeks before presentation. She complained of nocturnal cough, which eventually resolved. On the day of presentation, she suddenly developed dyspnea around noon and was transported to the hospital by ambulance.
She was fully conscious at the time of admission to the emergency department. Her main symptoms were dyspnea (Borg scale severity level 9) and wheezing (Johnson classification degree III); her respiratory rate was 34 breaths/min. Her neck accessory muscles (scalene and sternocleidomastoid) were prominently contracted. She was in an anteflexion position. Her body temperature was 37°C (98.6°F). Serum CRP level was 0.45 mg/dL; WBC count was 7700 cells/μL. Arterial blood gas analysis during reservoir oxygen mask therapy (10 L/min) revealed acute decompensated respiratory acidosis with hypercapnia (pH, 7.28; PaO2, 109 mm Hg; sat, 97%; PaCO2, 52 mm Hg; and HCO3−, 24.4). Her P/F ratio was 109. NPPV was initiated via face mask to address her low P/F ratio and hypercapnia. The nurse attached the NPPV mask, while evaluating it for air leak. The NPPV settings were as follows: S/T mode; IPAP, 10 cm H2O; EPAP, 4 cm H2O; I-time, 0.8 seconds; and FiO2, 100%. Methylprednisolone (40 mg) was administered via intravenous drip, and adrenaline (0.3 mg) was administered via hypodermic injection. Figure presents changes in P/F ratio, PCO2, respiratory rate, heart rate, and Borg scale level, at 30 and 60 minutes after the initiation of NPPV therapy. P/F ratio (382), PCO2 (39 mm Hg), and Borg scale level (grade 2) dramatically improved within the first 30 minutes along with improvements in respiratory and heart rates over time. Under NPPV therapy, her wheezing improved to Johnson classification degree II. The patient's position shifted from the anteflexion to Fowler's position. Prominent contraction of accessory respiratory muscles resolved. FHR, as estimated by an obstetrician using Doppler echocardiography, was 150 beats/min. The patient was transferred from the ICU to the obstetric ward. Oxygenation was stabilized to a level that can be coped with low-flow oxygen therapy, even if NPPV is temporarily discontinued after the P/F ratio is improved. Moreover, re-exacerbation of wheezing, dyspnea, tachypnea, and vital signs was not observed even if the NPPV mask was removed. Additionally, hypercapnia and respiratory acidosis were not re-exacerbated after NPPV is discontinued temporarily. NPPV therapy was discontinued and replaced with oxygen therapy. Ultimately, oxygen therapy was also withdrawn. The patient was discharged on day 8 post admission and prescribed budesonide and a formoterol fumarate hydrate dry-powder inhaler. The patient safely delivered a female child weighing 3006 g (Apgar score 8/9) at gestational age of 10 months and 5 days via spontaneous cephalic delivery. After discharge, the patient's condition was well maintained under the care of a respiratory specialist. The patient has adhered to the prescribed regimen of asthma medication. |
pmc-6509932-1 | A 64-year-old female patient presented to an emergency department with severe shortness of breath and lethargy that was preceded by 3 days of vomiting and reduced oral intake leading to dehydration. She had a recent history of undergoing a gastric sleeve weight loss surgery 4 weeks prior. Her other significant past medical history included hypertension, hypercholesterolemia, gastroesophageal reflux, osteoarthritis, vitamin B12 deficiency, migraines, obesity for which she was treated with the gastric sleeve surgery, in addition to type 2 diabetes mellitus for which she was treated with insulin, metformin, and dapagliflozin. Since she had the surgery she lost 20 kg with insulin dose reductions, while remaining on metformin and dapagliflozin.
On examination, she was noted to be tachypnoeac and tachycardiac with heart rate of 100 beats per minute. Her other physical examination including cardiovascular, respiratory, abdominal, and neurological systems were unremarkable. Arterial blood gas on presentation showed a pH of 6.93 [7.35-7.45], pO2—151 mm Hg [83-108], pCO2 9 mm Hg [34-45], HCO3 2 mmol/L [22-28], lactate 1.5 mmol/L [<2.2], sodium 142 mmol/L [135-145], potassium 4.3 mmol/L [3.5-5.0], chloride 123 mmol/L [95-110], and glucose of 13.5 mmol/L [4.0-7.8]. Given the modest elevation in glucose, a diagnosis of DKA was not considered at initial presentation, with ketones level not being ordered by the treating physicians. The cause of severe metabolic acidosis was not clear at this stage. She was investigated to exclude ischemic bowel and a computed tomography of her abdomen excluded this.
Her treatment included rapid rehydration with 3 L of normal saline administered over 3 hours, along with 10% dextrose and normal insulin. She was also given 300 mL of 8.4% sodium bicarbonate intravenously to correct severe acidosis, leading to improvement in pH (see Figure ). She was subsequently admitted to the hospital's intensive care unit (ICU) for further electrolyte correction and management of DKA. After 10 hours of hospitalization, in ICU her pathology results had improved with pH of 7.27, blood glucose level (BGL) 9.1 mmol/L, but her ketones remained elevated at 6.9 mmol/L while on an insulin infusion at 2 units per hour with potassium replacement of 60 mmol at the standard rate of 10 mmol/h. After review by an endocrinologist, the diagnosis of euglycemic DKA was established and the rate of insulin and glucose 10% infusion increased to 4 units/h and 80 mL/h, respectively, to resolve ketosis. Twenty-four hours into patient's treatment, she was still ketotic with level of 3.7 mmol/L with large requirement of potassium replacement and drop in phosphate level to <0.3 mmol/L [0.75-1.5]. Concurrently, the pH normalized at 7.39 and the patient was planned to be switched to intermediate and short-acting insulin once oral intake was adequate with cessation of oral hypoglycemic therapy including on discharge. Phosphate was replaced by sodium and potassium phosphate 26.4 mmol infused over 2 hours and regular 1000 mg of oral phosphate tablets administered three times a day. By middle of the second day of admission, patient's ketones fell to 0.4 mmol/L, while still on an insulin infusion at 4 units/h dextrose 10% infused at 80 mL/h. Overnight of the second day, patient BGL dropped to 5.7 mmol/L with insulin infusion being stopped while dextrose 10% continued at 40 mL/h with further 60 mmol of potassium administered to target a level above 4 mmol/L. In the morning of the third day, the ketone level has risen to 2.2 mmol/L and potassium level remained at 3.6 mmol/L. On the fourth day of admission, the patient was transferred to a medical ward with further optimization of her insulin dosing regimen by an endocrinologist with initiation of a combination of intermediate and short-acting insulin (Novomix 30®) at a dose of 6 units twice a day with additional short-acting insulin dose at of 4 units when BGL were above 12 mmol/L. Her BGL was stable at 11.9 mmol/L, and her phosphate levels improved with oral phosphate dosing dropped down to 1000 mg twice a day, and potassium level at 4.5 mmol/L with oral potassium changed from immediate release 14 mmol tablets to slow release 8 mmol tablets. On the eighth day of admission, the patient was discharged home with Novomix 30® insulin dosed three times a day, 14 units in the morning, 24 units at lunch, and 24 units at night, with an outpatient endocrinology review in the following week. |
pmc-6509933-1 | A 77-year-old man was admitted to our hospital with hemoptysis and slight fever of two weeks’ duration. He had been treated and followed for an old myocardial infarction and was taking clopidogrel and aspirin. On admission, his blood pressure was 111/55 mm Hg, pulse rate was 72/min, and body temperature was 37.2°C. His arterial oxygen saturation was 91% on O2 at 2 L/min via nasal canula, and arterial blood gas analysis showed a PaO2 of 61.9 Torr, PaCO2 of 30.3 Torr, and pH of 7.46. Chest X-ray and computed tomography (CT) showed bilateral ground-glass opacities (Figure ). Blood tests revealed a white blood cell count of 10 900/µL (normal, 3900-9800) (neutrophils 82.9%, lymphocytes 9.0%, eosinophils 2.3%, monocytes 5.7%), hemoglobin 10.7 g/dL (normal, 13.5-17.6), platelets 202 000/µL (normal, 130 000-369 000), prothrombin time 13.9 seconds (normal, 10.7-12.9), activated partial thromboplastin time 49.4 seconds (normal, 24.0-39.0), C-reactive protein 14.3 mg/dL (normal, <0.3), LDH 386 IU/L (normal, 119-229), total protein 7.4 g/dL (normal, 6.6-8.4), serum albumin 3.2 g/dL (normal, 3.8-5.2), serum creatinine 0.8 mg/dL (normal, 0.6-1.1), blood urea nitrogen 16 mg/dL (normal, 8-20), calcium 8.2 mg/dL (normal, 8.8-10.5), IgA 946 mg/dL (normal, 110-410), immunoglobulin G (IgG) 964 mg/dL (normal, 870-1700), and immunoglobulin M (IgM) 37 mg/dL (normal, 35-220). Urinalysis showed protein of 223 mg/dL (normal, <19) and was negative for occult blood. We initially suspected bacterial pneumonia, with antiplatelet agents as the cause of the DAH. Although we started antibiotics and stopped the antiplatelet agents, his pulmonary lesions gradually worsened, and hemoptysis continued on the 2nd hospital day. He required the administration of oxygen at an FiO2 of 80% to maintain an O2 saturation above 90%, which precluded the performance of bronchoscopy. We also considered systemic vasculitis and started a 3-day course of intravenous methylprednisolone (1000 mg/d) followed by oral prednisolone at 40 mg/d. However, the corticosteroid therapy was not effective: the inflammatory reaction increased, and his respiratory function continued to deteriorate. Therefore, we began another 3-day course of intravenous methylprednisolone on the 9th hospital day. Tests for antibodies associated with connective tissue diseases, including MPO-ANCA, PR3-ANCA, and anti-GBM antibody, were all negative. Although his IgA value was high, his IgG and IgM values were normal. Serum electrophoresis showed no M protein, but serum immunoelectrophoresis revealed the presence of IgA-κ-type M protein. Serum free light chain (sFLC) assay showed a κ/λ ratio of 21.4 (normal, 0.2-1.8). The patient’s β2-microblobulin was 1.6 mg/L (normal, 0.9-1.9), and he was negative for urine Bence Jones protein. An abdominal CT performed to search for MM revealed a left iliac tumor (Figure ). A CT-guided needle biopsy of this tumor was performed that revealed abnormal cells on hematoxylin and eosin staining. Immunostaining of the biopsied specimen showed the cells to be positive for CD79a and CD138, indicating that the abnormal cells were plasma cells (Figure ). Moreover, immunostaining of these cells was positive for IgA and kappa light chain, and thus we diagnosed the patient as having IgA-κ-type MM.
After the patient’s respiratory condition improved, bronchoalveolar lavage fluid (BALF) was obtained from the left B5 by bronchoscopy. We instilled 150 mL of 0.9% NaCl and aspirated 70 mL of bloody fluid containing 93.2% macrophages, 4.7% lymphocytes, 2.1% neutrophils, with a CD4/8 ratio of 0.5, and 25% hemosiderin-laden macrophages. A transbronchial lung biopsy (TBLB) showed interstitial fibrosis but was negative for plasma cells. We found no amyloid deposition in the lung by direct fast scarlet staining. However, deposition of IgA was revealed on a blood vessel wall by immunofluorescence staining, but no deposition of IgG was present (Figure ). We thus thought that the patient had DAH caused by IgA deposition due to MM of Durie/Salmon stage IIA and International Staging System stage II. We reduced the corticosteroid dose due to the diagnosis of MM, and he was discharged from our hospital on the 55th hospital day. Nine days later, treatment with lenalidomide and corticosteroid was started by the Department of Hematology in another hospital. His serum IgA and κ/λ ratio decreased, and the DAH improved after two courses of the treatment; however, the tumor in the left ilium did not improve. |
pmc-6509948-1 | We report a 48-year-old male patient who was referred to our Neurological out-patient clinic due to a tic disorder resembling GTS.
One year prior, the patient had been involved in a car crash causing a non-commotional cranio-facial trauma. A cerebral computer tomography (CT) scan was unremarkable. However, the emotional impact of the accident on the patient was great, as he had lost his only brother a year before due to the sequalae of severe traumatic brain injury, which he had sustained in a major car accident. His father had also died earlier in a car crash. A month after the patient's accident, he began to develop involuntary stereotyped facial movements, such as forceful eye closure or grimacing with his mouth, as well as phonic tics such as pronouncing deep and prolonged sounds or vocalization. Their frequency of occurrence was high, with numerous attacks per day, but devoid of any particular triggering factor. Because of the socially disabling symptoms, he had to quit his job and isolated himself from his community.
Oral alprazolam (3 mg/d), sertraline (50 mg/d) and risperidone (2 mg/d), administered in sequence, were ineffective in mitigating the motor-vocal symptoms but they could make the patient feel “internally more quiet.”
Neurological examination was totally unremarkable except for intermittent motor tics such as blepharospasm-like forced lid closure, grimacing, forced lip closure, noisy suction movements and phonic tics like grunting, vocalizations (mostly a deep prolonged “ah”), not in a constant combination or sequence, and lasting several seconds. The tics increased with attention in intensity and frequency. At the end of an episode, the patient appeared mortified and apologized for the occurrence of the tics. On two occasions, attempts to distract the patient during a series of tics could interrupt a sequence of vocalizations. The patient, however, was unable to interrupt the tics voluntarily. He reported that the tics would arrive suddenly but denied a preceding internal urge or any warning signs.
The patient consented to videotaping (, ), and interestingly, during the recorded clinical examination, he presented more tics than during other times, e.g., while sitting seemingly unobserved in the waiting room.
The patient's history was unremarkable with respect to pre, peri-, and postnatal development. There was no family history of tics nor any other movement disorders. The family's socio-financial situation was difficult, and his parents could take care of him only marginally. He attended normal school until the age of 18 years, but with overall poor performance.
At the time of his first presentation at our institution, the patient lived with his elderly mother and had a part-time job without public contacts. He declared to be emotionally prostrated by the involuntary movements, and to be highly motivated to find treatment. The patient also complained of poor attention and memory, and that he feared of developing dementia. A two weeks trial with haloperidol (2 mg/d) failed to ameliorate the symptoms. |
pmc-6509954-1 | A 69-year-old man presented to the emergency department, referred by his general practitioner. He complained of tumefaction and right hemi-cervical pain, dysphagia for solids, dysphonia, and loss of weight (10 kg in two months). Blood analysis revealed an inflammatory syndrome without hyperleukocytemia. Neck computed tomography (CT) showed a mass arising from the right lobe of the thyroid focally invading the trachea, associated with esophageal extrinsic compression and bilateral cervical lympadenopathies. Open surgical biopsy led to the diagnosis of unresectable anaplastic thyroid carcinoma. Subsequent positron emission tomography (PET)-CT was performed to evaluate the extension of the neck tumor and revealed a 18-Fluoro-deoxy-glucose (FDG)-avid lesion in the left adductor space (Figure ). Ultrasound-guided biopsy of the hypo-echoic thigh muscular mass (Figure ) confirmed metastasis.
A treatment by combined radio-chemotherapy was initiated. A temporary tracheal prosthesis was positioned; dysphagia was finally handled by gastrostomy. Cervical evolution was excellent under treatment, but muscle metastasis progressed (Figure ) and pulmonary metastasis appeared, leading to second-line chemotherapy. |
pmc-6509999-1 | A 9-month-old full-term unvaccinated Amish female baby with no known significant past medical history presented to the emergency department via EMS with fever, cough, and acute increased work of breathing. The patient was ill-appearing in significant respiratory distress with bilateral wheezing on examination. There was no clinical improvement following a nebulized albuterol treatment, and she quickly required intubation secondary to persistent tachypnea. A chest X-ray revealed bilateral infra-hilar streaky opacities, worse on imaging immediately following intubation (Figure ).
A complete blood count was grossly unremarkable. She received a dose of ceftriaxone and was admitted to the pediatric intensive care unit, where she was continued on piperacillin/tazobactam (Zosyn). Notably, the patient had previously been hospitalized in the intensive care unit 21 days prior for acute hypoxic respiratory failure secondary to a left lower lobe pneumonia following an unwitnessed aspiration while being bottle-fed. During this prior hospitalization, her initial chest X-ray showed a right peri-hilar opacity and tracheal aspirate culture was positive for both Streptococcus pneumoniae and Haemophilus influenzae. Clinical improvement was achieved with both bronchodilator therapy and a 7-day course of ceftriaxone. However, following hospital discharge, parents reported minimal clinical improvement on scheduled albuterol with continued episodes of increased work of breathing, persistent coughing, and wheezing. She developed fever and acutely worsening respiratory symptoms, thus prompting this current presentation (Figure ).
Despite aggressive bronchodilator therapy and airway clearance, she continued to show clinical obstructive airway disease. Her tracheal aspirate culture was positive for H influenzae, while her respiratory viral panel detected both rhinovirus and enterovirus. Piperacillin/tazobactam (Zosyn) was discontinued 48 hours after a repeat tracheal culture showed no growth. Although her initial chest X-ray showed acute left-sided lung collapse, parents denied any choking episodes or the possibility of a foreign body aspiration (FBA) prior to symptom onset. Daily chest X-rays were significant for persistent atelectasis, primarily of the left lower lobe (Figure ). computed tomography (CT) of chest showed no anatomic ring or sling, but significant left lung volume loss and complete opacification of the left lower lobe (Figure ). At that time, an irregular filling defect in the left main stem bronchus seen on CT was suspected to be secondary to secretions, debris, and inflammation rather than a foreign body.
A bedside bronchoscopy demonstrated significant mucus obstruction from the proximal left main stem bronchus, requiring robust secretion retrieval from the left distal branching airways. Dornase alfa (Pulmozyme) was started to promote mucus clearance with subsequent chest X-rays showing improved aeration of the left lung. She began to display signs of clinical improvement; however, she became septicemic delaying the plans for a repeat bronchoscopy. She was started on 48 hours of vancomycin and ceftriaxone with no organisms on repeated cultures taken after antibiotic initiation. Throughout her intensive care hospitalization, her respiratory examination was not consistently focal. Fluticasone propionate (Flovent) was started due to acutely worsening bilateral wheeze. A chest X-ray showed return of left lung collapse, and a hospital-acquired pneumonia was suspected. Due to multiple 48-hour antibiotic courses, she was continued on 10 days of vancomycin and cefepime (Figure ).
Once she became more clinically stable, a second bronchoscopy visualized transparent foreign body pieces in the left upper, right lower, and left lower lobes with segments adhering to the lung wall. Rigid bronchoscopy was successful in removing these foreign body pieces, which were suspected to be candy wrapper fragments (Figures , and ).
At this point, parents reported that an older sibling had been feeding the patient candies prior to her initial previous hospitalization 1 month prior. Following the removal of these foreign body pieces, the patient had rapid and complete return to clinical baseline with no further respiratory issues prior to the hospital discharge. |
pmc-6510001-1 | We present the case of a 45-year-old male patient, a 14 pack-years smoker, without significant medical history, a former worker in a coalmine, retired for 5 years. He was admitted in a hospital emergency department after 5 days of incoercible vomiting, epigastric pain, lumbar pain irradiated toward the base of the thorax, followed by muscular pain and gradual decrease in strength in upper and lower limbs.
Physical exam showed skin pallor and no organomegaly, no cutaneous bleeding, cardiovascular, or respiratory pathological changes. The neurological exam established the diagnosis of peripheral proximal tetraparesis, predominantly in the upper limbs with preserved sensitivity. These findings demanded urgent running of biological tests.
Blood tests showed severe microcytic, hypochromic anemia, increased serum iron level, serum ferritin level, and transferrin saturation coefficient and normal corrected reticulocytes count. The white blood cell and thrombocytes counts were normal (Table ).
The peripheral blood smear showed anisocytosis, hypochromic red blood cells, red blood cells with basophilic granules, droplet red blood cells, and rare ovalocytes. Direct and indirect Coombs tests were negative. The bone marrow aspirate showed 46% sideroblasts, 42% ringed sideroblasts, 2+/3+ macrophages, suggesting the diagnostic of erythroid hyperplasia and sideroblastic anemia.
The remainder of the blood chemistry tests that were carried out initially showed hyperbilirubinemia with increased indirect bilirubin, inflammatory syndrome, hepatocytolysis, hepatic cholestasis, and normal renal function (Table ).
The association of the clinical symptoms involving the nervous, hematological, and gastrointestinal systems made this clinical presentation a diagnostic challenge.
We considered a number of differential diagnosis based on the pathological changes:
Peripheral tetraparesis may have multiple causes: trauma, vertebral tumors, vertebral disk hernia, Guillain-Barre syndrome, chronic degenerative neuropathy, multiple sclerosis, or other degenerative neurological diseases, as well as radiation and intoxications.
We performed a thoracolumbar and sacral spine MRI that showed no sign of tumor or trauma and described the diffuse reduced T2-FLAIR (Fluid-attenuated inversion recovery) at a level that is suggestive of a deposit disease (Figure ).
The hypochromic, microcytic anemia may have various causes but we limited ourselves to those, which combine high blood iron and ferritin levels, high saturation coefficient of transferrin and the presence of ringed sideroblasts in the bone marrow aspirate.
The presence of ringed sideroblasts in the bone marrow raises the question of a congenital anemia or of an acquired anemia induced by ethanol, other toxins, and low level of vitamin B6 or infections.
High blood iron and ferritin levels as well as the high transferrin saturation index raised the question of a congenital hemochromatosis; nevertheless, same results may be attributable to other conditions like thalassemia major, sideroblastic anemia, chronic hemolytic anemia, aplastic anemia, chronic liver disease, porphyries, elevated iron intake, or blood transfusion. We excluded hemochromatosis as the genetic test for C282Y mutation of human hemochromatosis protein (HFE) proved negative for our patient. Hemoglobin electrophoresis showed beta thalassemia minor, with a value for Hb-A of 94% and for Hb-A2 of 5.9%.
The gastric and neurological symptoms associated with the complex hematological changes raised the diagnosis of acute intermittent porphyria. We determined the urine level of porphyrins and found an elevation of the coproporphyrins, uroporphyrins, porphobilinogen, and delta amino levulinic acid (Table ).
The high urinary values of different porphyrins raised the suspicion of porphyria. Acute intermittent porphyria was suggested by the elevation of the urinary level of delta amino levulinic acid and porphobilinogen. However, the normal levels of enzymatic hydroxymethylbilane synthase contradicted this diagnosis. The patient had no cutaneous manifestations seen in other forms of porphyria, which could have explained the high urinary level of porphyrins.
Other causes of increased urinary porphyrins were explored: intoxications, infectious diseases, chronic liver disease, and neoplastic disorders.
Biological tests showed high levels of blood and urinary lead of 48 µg/dL (normal, <20 µg/dL) and 290 µg/24 h (normal, <40 µg/24 h), respectively.
Our final diagnosis was nonprofessional lead intoxication complicated by peripheral tetraparesis, severe hypochromic, microcytic anemia, and beta thalassemia minor. |
pmc-6510002-1 | A 72-year-old man was referred to our hospital for evaluation of a huge mass on CT (Figure A, arrows). He presented to the previous hospital with awareness of nontender abdominal mass. He was otherwise asymptomatic. 18F-FDG PET/CT revealed a bulky mesenteric mass (13 cm diameter) with a maximum standardized uptake value of 13.26 (Figure B). He underwent laparotomy for excisional biopsy, which were compatible with follicular lymphoma. He was treated with bendamustine plus rituximab. He has been followed up on outpatient basis and has had no recurrent disease to date.
Primary mesenteric tumors are very rare, with incidence of <1 in 200 000, and follicular lymphoma is the most common histological type. There are various imaging patterns of mesenteric lymphoma at CT; including rounded, enhancing, or homogenous masses. Although the sandwich sign, bulky lymphadenopathy in the mesentery encasing vessels and the bowel, is known to be suggestive of malignant tumors, its diagnostic performance has been unknown. Because mesenteric lymphoma may be indolent, a focus on patient complaints, such as awareness of mass as this case, is essential for early diagnosis. Among solid mesenteric tumors, malignant lymphoma should be considered a priority. |
pmc-6510006-1 | A 26-year-old gentleman with a past medical history of hypertension, presented to our hospital with persistent fevers for 2 weeks. He was in his usual state of health when he began developing daily fevers associated with proximal shoulder and thigh weakness. His fevers were refractory to antipyretics. He denied any other associated symptoms including rashes, arthralgias, myalgias, headache, nuchal rigidity, cough, abdominal pain, nausea, diarrhea, or dysuria. He had not recently travelled outside of the United States and denied any sick contacts. His last sexual encounter was 1 year prior to presentation. He denied genital lesions or discharge. He reported no history of drug abuse. He was born in the Philippines and immigrated to the United States 9 years prior to presentation. At birth, the patient was told he had a hole in his heart that would spontaneously close by adolescence. He recalled “turning blue” while crying as a child, but had since denied cyanotic spells since childhood. As an adult, he could walk several blocks and climb flights of stairs without difficulty. However, he reported dyspnea with jogging and running, which he had attributed to deconditioning. He was unaware of any significant family history.
Upon arrival to the emergency department, he was afebrile and hemodynamically stable. The physical exam was notable for left, anterior, nontender, mobile cervical adenopathy and a III/VI holosystolic murmur heard loudest at the 3rd left intercostal space. Initial labs were notable for a mild leukocytosis and elevated erythrocyte sedimentation rate and c-reactive protein. Electrocardiogram (EKG) showed increased voltage, prominent R waves in the precordial leads, and nonspecific ST segment and T wave changes (Figure ). Chest X-ray showed cardiomegaly and a chest computed tomography showed multiple pulmonary nodules in bilateral lung fields.
A transthoracic echocardiogram (TTE) was obtained and showed moderate-to-severe right ventricular hypertrophy (RVH), RVOT, and a VSD consistent with Tetralogy of Fallot (TOF), although no evidence of an overriding aorta. He subsequently underwent a transesophageal echocardiogram (TEE) that again showed RVH and a VSD, but also noted a muscular band within the RV (Figures and ). Blood cultures grew aggregatibacter aphrophilus. He was diagnosed with endocarditis by Duke's criteria: one major (positive blood culture with HACEK organism), and three minor (fever, predisposing cardiac lesion, pulmonary emboli). He was discharged with a 4-week course of intravenous Ceftriaxone following the last negative blood cultures. After discharge, the patient was further evaluated to differentiate between TOF and DCRV. A cardiac catheterization showed an anomalous band dividing the RV into two chambers, with a peak-to-peak gradient of 115 mm Hg between proximal and distal RV consistent with DCRV (Figures and ). Additionally, the patient underwent a cardiac magnetic resonance imaging (MRI) showing a D-shaped left ventricle and RVOT with subpulmonic stenosis (Figure ). He was referred to cardiothoracic surgery for RV muscle band myomectomy and patch closure of VSD. |
pmc-6510012-1 | A 55-year-old man with history of diabetes mellitus, AF, and family history of sudden death was admitted to the emergency department for worsening HF (NYHA class III). The 12-lead ECG showed AF with a ventricular rate response of 110 bpm. His medications were furosemide, spironolactone, bisoprolol, ramipril, and digoxin. On admission, a transthoracic echocardiogram was performed showing a LVEF of 25% with global hypokinesia. At first, the patient was stabilized with heart rate control and intravenous (iv) furosemide infusion. The coronary angiography revealed normal coronary arteries. Once the transesophageal echocardiography excluded intracardiac thrombi, electrical cardioversion was attempted. However, even if the patient was loaded with amiodarone iv, ECV was unsuccessful. For this reason, the rate control therapy was optimized and the patient was scheduled for a cardiac magnetic resonance (CMR) to better evaluate the underlying substrate. CMR showed global systolic dysfunction resulted in an LVEF of 30% and no late-gadolinium enhancement (LGE) throughout the left ventricular myocardium. Considering that the patient had an otherwise not explained dilated cardiomyopathy, we considered this clinical scenario compatible with tachycardiomyopathy and a rhythm control strategy was planned. Patient underwent radiofrequency (RF) catheter ablation of AF according to the latest consensus recommendations.
Briefly, PVI was performed under conscious sedation. A 7F decapolar catheter was inserted into the coronary sinus to guide the transseptal puncture. Transseptal access to the left atrium (LA) was obtained using a Brockenbrough XS needle (Abbott Medical, MN, USA) and an SL1 8.5F transseptal sheath (Abbott Medical, MN, USA). After transseptal puncture, unfractionated heparin was given as bolus (10 000 U) followed by a continuous infusion (1000 U/h) to maintain an ACT ≥350 seconds. The procedure was guided by a 20-pole circular mapping catheter and electroanatomical mapping system (CARTO 3, Biosense Webster, CA, USA). A 3D shell of the LA and the pulmonary veins (PVs) was created prior to ablation. Pulmonary vein isolation (PVI) was performed using a 3.5 mm bidirectional open irrigated tip catheter with contact force capability (SmartTouch SurroundFlow, Biosense Webster, CA, USA) with an upper temperature limit of 43°C, power of 25-35 W, and an infusion rate of 17 mL/min. A single circumferential lesion was created around the PVs ostia guided by ablation index score. During RF delivery along the ridge between the left superior pulmonary vein and the left atrial appendage, AF interruption with sinus rhythm restoration was documented (Figure ). Exit and entrance block was sequentially confirmed in each of the PVs using the circular catheter. Far-field capture and sensing were ruled out using differential pacing maneuvers. Postprocedural echocardiogram showed no pericardial effusion. Two days after the procedure, the patient was fitted with a WCD (LifeVest, Zoll, Pittsburgh, PA, USA), instructed on how to use the WCD by the providing physician and a device representative, and discharged without any complications. At three-month follow-up, patient symptoms' significantly improved (NYHA class I), his compliance was satisfactory, and no shocks were recorded. The transthoracic echocardiography showed a complete normalization of the LVEF (55%) and normal end-diastolic left ventricular volume. At this point in time, the WCD was discontinued and the patient was kept on amiodarone and beta-blockers and closely followed in the outpatient clinic. At six months, amiodarone was discontinued. The patient had no episodes of atrial and ventricular arrhythmia at 9-month follow-up. |
pmc-6510013-1 | A 73-year-old woman presented with one month of progressive pain and swelling in her right breast. She had a past medical history of right breast cancer sixteen years prior, which had been treated with lumpectomy and chemoradiation in Colombia, followed by bilateral textured silicone breast implant placement.
Breast MRI showed that the right breast implant had been deformed by a complex effusion within the fibrous implant capsule, giving it the appearance of rupture on ultrasound. Extending superiorly from the right implant capsule was a mass, measuring up to 8 cm and invading both the chest wall and pleura. While some simple fluid extended across the midline to the medial aspect of the left breast implant, there was no suspicious enhancement to suggest left breast involvement.
A core needle biopsy of the mass was performed. Sections showed neoplastic infiltrate comprised of large malignant cells with round, oval and irregular nuclei, finely stippled chromatin, conspicuous nucleoli and abundant pale, vacuolated cytoplasm. Tumor cells were associated with a rich mixed inflammatory infiltrate comprised of small T and B lymphocytes, many eosinophils and histiocytes. Tumor cells were noted to infiltrate skeletal muscle and other soft tissues. Immunohistochemistry showed diffuse positivity for CD45, CD30, CD43, CD4, MUM-1, and very weak positivity for CD2. The Ki67 proliferative index in tumor cells was high, approaching 90%. Tumor cells were negative for CD79a, PAX5, CD20, CD8, CD56, CD3, EMA, CD34, CD5, ALK-1, pan-keratin (AE1/AE3/PCK26), CK5/6, CK818, CK903, CD31, Factor VIII, CD15, D2-40, EBER (in situ hybridization), CD163, and CD68.
The patient underwent bilateral explantation of her prostheses, followed by full-body staging CT scans. There was a right supraclavicular lymphadenopathy, with three nodes measuring up to 4.1 cm in diameter. The chest wall and axilla showed extensive inflammation with subcutaneous gas. The tumor mass was associated with a right pleural effusion, which was found to contain only benign inflammatory cells and reactive mesothelial cells by pathologic examination. A bone marrow biopsy was negative for ALCL, but incidentally positive for a kappa clonal plasma cell population comprising 20% of the cellularity.
The patient underwent palliative radiation therapy for persistent breast pain. Her case was presented to a multidisciplinary tumor board, and she subsequently began monotherapy with brentuximab vedotin (BV). Her dosing regimen was initiated at 1.8 mg per kilogram of body weight for 18 cycles at three-week intervals. Her treatment was interrupted after her first cycle of BV due to a 12-day hospital admission for multilobular pneumonia, colitis, and associated septic shock. Upon recovery, she resumed BV treatment and a subsequent PET/CT scan after her 5th cycle showed that the chest wall mass and supraclavicular adenopathy had resolved and a large seroma had formed in the anterior chest wall (Figure ). She developed a peripheral neuropathy in her hands and feet after the 9th cycle, which was successfully treated with duloxetine and no dose reduction was required. At the writing of this case report, the patient is 20 months postcompletion of her 18-cycle regimen and subsequent PET/CT scans continue to demonstrate complete remission. |
pmc-6510461-1 | A 4-month-old Caucasian female with a medical history of mild torticollis presented to the emergency department at Children's Hospital New Orleans on May 30, 2017, with loss of head control milestone and hypotonia of the neck for 2 weeks. She also had experienced decreased range of motion of the neck and poor feeding during this time. On examination, the patient was found to have poor head control and could not hold her head up when placed in either the upright position or the prone position. Range of motion and strength was normal in all extremities. Neck CT (Figure ) and AP/lateral radiographs showed abnormal calcification and edema in the retropharynx and around the C1/C2 vertebrae articulation. MRI was also performed and supported these findings, showing enhancement and calcification in this area. TC was then suspected. Laboratory workup was then performed for secondary causes of TC, where parathyroid hormone, Ca, vitamin D, and P were all found to be within normal limits. Orthopaedic surgery and Ear, Nose, and Throat were consulted for operative management.
After imaging and discussion among the interdisciplinary team, it was agreed upon to take the patient for surgery using a biopsy procedure to confirm an accurate diagnosis of TC. After induction of anesthesia and orotracheal intubation, the patient was positioned in the operating room bed and mouth was opened with a Crowe-Davis mouth gag. A bulge was seen in the left retropharyngeal space, and an incision was made in the retropharyngeal space. A stat was then inserted and opened, and fluid with chalky white deposits was aspirated and sent for microbiology and pathology. A curet was then taken and the anterior portion of the lesion was curettaged, and about 2 mL of chalky white fluid mixed with serosanguinous fluid was aspirated, only taking the portions of the mass that were easily accessible. Afrin-soaked pledgets were packed into the incision to close the gap, and the patient was handed back to anesthesia for recovery.
Pathology (Figure ) demonstrated calcified material with scanty cellular material. Calcification was psammomatous in some areas with deposition of bluish and pinkish calcified material. No lamellar bone or increased inflammation was identified, nor was granulomatous material appreciated.
The patient recovered well postoperatively and saw improvements with apraxia and head control during the hospital stay, as well recovered feeding capabilities, and was discharged on postoperative day 7. The patient was then followed up with serial CT scans and with neurology over the next 5 months. The patient returned to the clinic for follow-up at 5 months postoperatively and was found to have no neurological deficits. Full strength and range of motion was seen in the neck and all four extremities, and no sensory deficits were noted. Repeat CT showed no residual retropharyngeal calcifications, some persistent calcification surrounding dens and extradural space at C1 and C2 levels, and no new calcium deposits. A subsequent follow-up at 11 months postoperatively continued to show no physical complaints, and a normal examination and repeat CT (Figure ) showed near complete resolution of TC. |
pmc-6510563-1 | A 70-year-old Indian male with a history of a Gleason 7 (3+4) prostate cancer who underwent aborted prostatectomy a week prior, presented with weakness, abdominal distention, decreased urine output, increased lower extremity edema, and constipation for five days. On exam, his vital signs were normal. His abdomen was soft but distended, and bilateral lower extremities showed pitting edema to the level of the knees. Initial laboratory studies were notable for sodium of 117 mmol/L, potassium of 6.3 mmol/L, creatinine of 9.92 mg/dL, and white blood cell count of 21 x 108/L. Computed tomography (CT) of the abdomen and pelvis was remarkable for diffuse urinary bladder wall thickening extending to the level of the seminal vesicles, bilateral hydronephrosis, diffuse peritoneal carcinomatosis with moderate ascites, and small low-density lesions in the right lower lobe of the liver too small to characterize (Figure ). The patient subsequently underwent emergent hemodialysis followed by interventional radiology guided nephrostomy tube placement. Nuclear medicine (NM) bone scan did not show definitive evidence of osseous metastases.
Given the rarity of peritoneal involvement of prostate cancer, the pursuit of confirmatory testing was necessary to evaluate for other primary cancers. In addition, the patient’s origin from a tuberculosis endemic region raised concern for possible peritoneal tuberculosis. Ascitic fluid cytology studies confirmed the presence of malignant cells. Immunohistochemistry staining positive for prostate-specific antigen (PSA) and Ber-EP4 (a monoclonal antibody used to distinguish between adenocarcinoma and reactive mesothelium) was compatible with primary prostate adenocarcinoma (Figures -) []. However, the hematoxylin and eosin (H&E) staining also showed focal signet-ring cell features concerning for possible gastrointestinal carcinoma primary (Figure ). Ascitic fluid cultures for acid-fast bacilli, Mycobacterium tuberculosis polymerase chain reaction (PCR) and Quantiferon assays, were negative. The patient declined further tissue analysis via cystoscopy.
The patient was treated with androgen deprivation therapy (ADT), which included leuprolide and bicalutamide. Extensive discussions were held with regard to the addition of docetaxel to ADT according to the chemo-hormonal therapy vs. androgen ablation randomized trial for extensive disease in prostate cancer (CHAARTED) [], but the patient declined as he felt too weak. The patient’s total serum PSA decreased from 38.2 ng/ml to 4.7 ng/ml over four months. Repeat staging CT scan of the abdomen and pelvis that was done five months after the initiation of therapy showed an overall improvement in the peritoneal carcinomatosis. However, there was an increase in the size of some nodules in the omentum as well as a new soft tissue mass extending into the seminal vesicle consistent with progressive disease (Figure ). NM bone scan was negative for osseous metastases and patient’s serum PSA was 192.
The patient was deemed castrate-resistant with plans to start on abiraterone and prednisone therapy. Before the patient could start therapy, he developed hematuria, requiring admission to the hospital for bladder outlet obstruction from compressive effects of the mass. Repeat CT scans showed increased tumor burden including the progression of the peritoneal carcinomatosis. Although the patient was out of the window of benefit per the CHAARTED trial, he was started on docetaxel given his high volume disease while continuing his Lupron. After cycle six of docetaxel, his total serum PSA down-trended to 3.38 ng/ml with repeat CT imaging showing the response in almost all target lesions, except for an increase in the size of a nodule at the vesicoureteral junction. Given the concern for progression, the patient was subsequently started on abiraterone and prednisone. After six months of therapy, the patient had progression with worsening peritoneal carcinomatosis and a noted increase in his PSA to 4.66 ng/ml. After discussion with the patient regarding his goals for treatment, it was decided to start therapy with cabazitaxel. After one cycle of therapy, the patient had improvement of his symptoms with a decrease in his PSA. Upon completion of his fourth cycle, however, the patient developed sepsis, requiring admission to the hospital. Given his worsened functional status, the patient was deemed an unsuitable candidate for further chemotherapy and was later transitioned to home hospice. |
pmc-6510567-1 | A 50-year-old woman presented to our clinic with complaints of worsening mechanical axial lower back pain for the past 10 years that had now become debilitating, and intermittent bilateral radicular components towards the end of the day that were poorly defined. She worked as a nurse, and had a history of a small patch of hair that was removed from her mid lower back when she was four years old, without reported surgical exploration. She carried the diagnosis of tethered cord syndrome. Her clinical examination was benign except for mild diffuse hyperreflexia. A standard 3 Tesla MRI of the lumbar spine was obtained in the supine position and included axial T1 and T2 cuts, as well as T2 sagittal reconstructions. The T2 sagittal reconstructions were used to assess the position and the motion of the conus, and the axial T1 images were used to assess the presence of a fat-infiltrated filum. We then positioned the patient prone and obtained the same sequences. Normal ventral motion of the conus was defined as >10% of the total antero-posterior canal width as previously described by Stamates et al. []. Her supine MRI showed a low-lying conus medullaris at the level of the L3-4 disc space (Figure ).
Her axial images showed a split cord malformation, without the presence of a bony septation (Figure ).
Axial imaging at the level of L5-S1 revealed a small fatty filum terminale (Figure ).
A prone MRI was obtained (Figure ).
It showed significant anterior motion of the spinal cord of more than 10% of the central canal width when comparing prone to supine sagittal T2 images. She was prescribed an intensive physical therapy regimen for 12 weeks, and her symptoms completely resolved but her hyperreflexia persisted. |
pmc-6510569-1 | Mr. X, a 29-year-old male with past psychiatric diagnoses of major depressive disorder, generalized anxiety disorder, borderline personality disorder, and history of multiple suicidal attempts by overdose in context of feeling “void”, “numb”, “bored” and “overwhelmed”. He presented with vague and unclear anxiety, panic attacks, feelings of generalized discomfort, and reports of passive suicidal ideation. Upon evaluation, he was guarded, oddly related, occasionally distracted, and unable to provide relevant or logical answers to most questions due to prominent blocking and disorganization of thoughts. He exhibited ambivalence in basic decision-making processes related to treatment and disposition and reported confusion about his sexual orientation and preference. Throughout his stay in the hospital, he displayed poor interpersonal and coping skills, impaired impulse control, and a gradual decline in functioning that had commenced 11 years prior to the current presentation. Records from previous outpatient visits and inpatient hospitalizations described presentations of numbness, emptiness, poor impulse control and interpersonal skills as well as superficial expression of emotions which led to diagnoses of borderline personality disorder, generalized anxiety disorder, and major depressive disorder. Notably, he had also failed several antidepressant trials. He was prescribed an oral dose of 50 mg of quetiapine which was titrated upwards to 300 mg, at which point there was significant improvement in symptoms of anxiety, depression, and suicidal ideation. His thought process became somewhat linear; he slowly started gaining insight into his condition and was able to communicate. |
pmc-6510569-2 | Ms. Y, a 39-year-old female with a history of major depression and anxiety, diagnosed and managed by her therapist for a few months prior to her hospitalization, was referred to the emergency room when she decompensated, reporting vague symptoms of paranoia, auditory and visual hallucinations. She was noted to be anxious, apprehensive, and perplexed, demanding to be seen repeatedly for reassurance. Vagueness, tangentiality, and circumlocution pervaded her conversations. She narrated events that permitted the team to learn about her poor boundaries with strangers, inability to maintain healthy relationships, and difficulty with keeping steady jobs. Borderline personality traits were initially suspected but collaterals from her aunt described her as an “odd, paranoid person” with poor interpersonal skills and said that she had often seen her talking to herself. However, being distant and not causing nuisance in the community, she had not drawn attention to herself and had never been hospitalized. A diagnosis of schizophrenia was formulated and her symptoms of anxiety, hallucination, and thought disorganization improved on oral risperidone gradually titrated to a daily dose of 6 mg. |
pmc-6510571-1 | A 47-year-old female was diagnosed with Sjogren’s Syndrome six years ago by another rheumatologist, based on her history of eye and mouth dryness. She was found to have a negative rheumatoid factor at that time, but her sedimentation rate by modified Westergren (erythrocyte sedimentation rate, ESR) was recorded as low as 48 and as high as 61 (normal: less than 20 mm/h). Her C-reactive protein (CRP) was 1.74 (normal less than 0.80 mg/dl). Two years ago, she saw a second rheumatologist who agreed with the diagnosis of Sjogren’s Syndrome. At that time, her rheumatoid factor was now elevated at 69 IU/ml (normal: less than 14 IU/m/). Her antinuclear antibody (ANA) and Sjogren antibodies (SS-A and SS-B) were absent. Her anti-CCP antibody and 14.3.3 ETA protein were normal. Her ESR was 48 and her CRP was 1.42. Past medical history included a diagnosis of fibromyalgia. She also had a history of breast cancer that had been in remission for 20 years, a generalized seizure disorder, and elevated liver tests with normal biopsy. Additional medical issues included symptoms of neuropathy, anxiety, and depression. A prior sleep study did not reveal evidence of sleep apnea. She first came to see this author 18 months ago, seeking another opinion, with complaints of fatigue, severe musculoskeletal pain, as well as dryness of her eyes and mouth.
Her daily medications to help with her symptoms of Sjogren’s Syndrome and fibromyalgia included Lexapro, Restasis, meloxicam 15 mg, vitamin D3, magnesium, tramadol 100 mg daily prn, salagen 5 mg tid prn, and hydroxychloroquine 400 mg daily. Her exam demonstrated widespread trigger points affecting both sides of her body, above and below her waist. Her blood work was remarkable for an ESR of 37 and a CRP of 0.77. Her alanine aminotransferase (ALT) was 40 U/L (normal 6-29 U/L).
She elected to try low-dose naltrexone (LDN), which was compounded using a short-acting filler and started at 1.5 mg daily with instructions to increase the medication weekly by 1.5 mg. She came back to see me two weeks after starting the medication and was taking 3 mg daily. She stated that she felt terrific. Her lab was remarkable for a normal ESR of 25 and a CRP of 2.33. Her ALT was normal.
She was seen in follow-up 16 months ago and remained on 3 mg of naltrexone. She felt well but complained of neuropathic pain. Her ESR was now 20. CRP was not ordered. Her ALT was 31 U/L. She was seen in follow-up 14 months ago and remained on 3 mg of naltrexone and continued to feel well without stiffness or pain. She noted that, previously, it would take her all day to feel better. Her ESR remained at 20 and CRP was only minimally increased at 0.87.
She was then seen in follow-up 11 months ago with complaints of increased achiness. She had widespread tender points. She was given a short course of corticosteroids with symptomatic improvement in place of meloxicam. Naltrexone was increased on that visit to 4.5 mg. On the day of that visit, her ESR was 40 and CRP was 25.7 ( current normal value less than 8 mg/L). She was again seen in follow-up nine months ago, doing well on 4.5 mg of naltrexone. Hydroxychloroquine was discontinued a few weeks earlier due to a prolonged QTc interval. Her ESR was back down to 20 and CRP was down to 10.9.
Overall, the patient noted significant clinical benefit with her fatigue and pain within two weeks of starting low-dose naltrexone but no significant change in her dry eyes or mouth. She continues to do well on low-dose naltrexone four months after stopping hydroxychloroquine due to the electrocardiogram (EKG) abnormalities. While her symptoms improved, what is most interesting about this case is that her clinical improvement was associated with an improvement in her inflammatory markers. |
pmc-6510660-1 | The patient is a 52-year-old male with a history of a severe motor vehicle collision, who presented with the chief complaint of persistent right shoulder weakness. Fifteen months prior, he was struck as a pedestrian by a motor vehicle. As a result, he sustained multiple severe facial fractures, fractures of the first, third, fourth, and fifth right ribs, a fracture of the left first rib, fractures of the left T1 and T2 transverse processes, and a comminuted transverse fracture of the right scapular body (Figure , Figure ).
He received multiple facial reconstruction surgeries and was kept in the hospital for over one month. Since then, he had been followed by an orthopedic surgeon and received physical therapy but the right shoulder weakness persisted. Physical examination of the right shoulder in our clinic revealed marked atrophy of the infraspinatus muscle when compared to the left shoulder. In addition, there was a significant loss of range of active and passive motion in all planes of the right shoulder. EMG was performed, which demonstrated a normal insertional activity and interference pattern in the biceps and deltoid muscles, suggesting no denervation at these muscles. He had reduced recruitment, a reduced interference pattern, and a few positive sharp waves in the supraspinatus muscle, suggesting denervation. The infraspinatus muscle had little to no activity and was difficult to measure due to significant atrophy. These findings suggested that there was an injury to the suprascapular nerve with more severe denervation at the branch to the infraspinatus muscle. The suspected cause of the injury was the scapular fracture. The patient was sent for physical therapy and was unwilling to consider further invasive treatment at this time. Although the patient's shoulder pain was stable at the time, it was thought that physical therapy could be useful to prevent further atrophy and weakness. |
pmc-6510746-1 | A 74-year-old Japanese woman noticed a tender lump in her right breast. She immediately went to a breast clinic to get a breast cancer screening. She had no family history of breast and ovarian cancer. After a month, she was referred to our institution with suspicion of metaplastic breast carcinoma with a core needle biopsy at the breast clinic. Physical examination revealed a hard, tender, and 25-mm mass in the upper outer quadrant of her right breast and a palpable lymph node in her right axilla. Mammography indicated an indistinct mass on the mediolateral oblique view and the craniocaudal view. Ultrasound (US) showed an 18 × 16-mm, irregular-shaped, and hypoechoic mass with a suspicion of a spread to the nipple inside the duct (Fig. a) and several swollen lymph nodes in levels I to II (Fig. b). Magnetic resonance imaging (MRI) detected enhancement of a 17 × 17-mm indistinct mass surrounded with a non-mass enhanced segmental lesion toward the nipple side spreading a maximum of 74-mm range, which had no interaction with the chest bone, muscles, and breast skin, in the right breast tissue (Fig. ). Invasive carcinoma with multiple axillary lymph node metastases was strongly suspected on clinical examination and imaging. Histological evaluation of the biopsy for the mass revealed a tumor with the growth of oval and spindle-shaped cells and multinucleated giant cells, the infiltrating lymphocyte into the breast tissue, and hyalinization in the stroma. The multinucleated giant cells stained positively for CD68. A part of the oval and spindle mononuclear cells stained weakly positive for CD68. These tumor cells stained negatively for estrogen receptor (ER), progesterone receptor (PgR), and human epidermal growth factor receptor 2 (HER2). There was a focal hemorrhage without necrosis. Few non-epithelial atypical cells were observed in the breast duct, but no atypical epithelial cells consistent with breast cancer were detected. GCT of the breast, breast cancer with OGCs, and giant cell-rich sarcomas should have to be considered as differential diagnoses, and the pathological findings suggested most GCT. Fine needle aspiration biopsy for the swollen lymph node revealed only normal lymphocyte, even though metastatic lymph node was strongly suspected on US. However, we could not rule out the possibility that the biopsy tissue showed a part of malignant tumor with OGCs and biopsy for the lymph node was false negative, because there was a gap between the clinical presentation, such as a tender mass suggesting rapid growth and multiple lymphadenopathies, and the pathological presentation of biopsy tissue. To obtain further evidence of malignancy, the tumor was sampled using a vacuum-assisted US-guided biopsy again. The result was the same as the prior biopsy. After discussing the treatment plan with the patient, we performed mastectomy and sentinel lymph node biopsy according to a surgical procedure for node-negative breast cancer with a wide ductal spread. The resection tissue histologically revealed similar findings to the biopsy specimen. The tumor was composed mainly of oval and spindle mononuclear histiocyte-like cells and multinucleated giant cells (Fig. ). The mitotic figure of these cells did not stand out. There was no evidence of malignancy, and only intraductal epithelial hyperplasia around the tumor, which did not fill the criteria of ductal carcinoma in situ (DCIS). No sentinel lymph nodes contained malignant cells, and we concluded the lymphadenopathies were a response to the inflammation around the tumor. Immunohistochemically, a high proportion of the multinucleated giant cells stained positively for CD68 (Fig. ). A part of the oval and spindle mononuclear cells stained weakly positive for CD68. These cells were negative for CK OSCAR, GATA-3, and MGB1 (Fig. ). These findings were consistent with the GCT of the breast. The patient received no adjuvant therapy because GCT-ST is usually considered as a benign tumor. She is being followed up with regular clinical examinations without any symptoms of recurrence after 1 year past from surgery. |
pmc-6511127-1 | A 67-year-old man underwent surveillance esophagogastroduodenoscopy once a year at our hospital after endoscopic submucosal dissection (ESD) for an early GC that was a conventional, well-differentiated tubular adenocarcinoma. He received eradication therapy for a week after the first ESD, after which he received no proton pump inhibitor medication. A surveillance endoscopy revealed another GC 3 years after the first ESD. No metastasis or primary tumor was detected in other organs, including the pancreas, by computed tomography. He underwent ESD for the new lesion, and the ESD specimen contained a 12 × 8 mm slightly depressed tumor with irregular margins. Histologically, the tumor was composed of two components, and it showed submucosal and lymphatic invasion (Fig. a). Although one component had a glandular structure and mucin production (Fig. b), the other had nested trabecular or acinar-like structures (Fig. c). The proportions of glandular and solid components were 60 and 40%, respectively. There were no ectopic pancreatic cells or pancreatic metaplasia in the background mucosa.
Because of the submucosal and lymphatic invasion, distal gastrectomy and lymphadenectomy were performed. There was no residual cancer in the surgically resected stomach and no lymph node metastasis. The patient has not received chemotherapy. He has remained alive without recurrence or metastasis for 15 months since ESD was performed. |
pmc-6511133-1 | A 25-year old woman was admitted to a psychiatric hospital due to psychotic mania. During the preceding month she had experienced elevated mood, decreased sleep, increased thought pressure, excessive spending, distractibility and expansive ideas. One week prior to admission she had been exposed to a traumatic event by proxy, which triggered intense anxiety, loss of energy and motor agitation. She subsequently started experiencing referential thoughts, which were felt as increasingly convincing, and she developed grandiose delusions raising concern in her family. She was escorted for medical and psychiatric evaluation, and was admitted into a psychiatric hospital for treatment. By the time of admission she had also experienced frequent referential delusions to surrounding events, a Fregoli-delusion, visual illusions and auditory speech hallucinations. Her behaviour and speech were disorganized, and affects changed from elation to agitated anxiety.
According to a structured clinical interview for DSM-IV Axis I disorders, the patient was initially diagnosed as suffering from a manic episode with mood congruent psychotic symptoms. Peroral treatment with aripiprazole 15 mg/day and lorazepam 3 mg/day was started. Rorschach comprehensive system revealed sensory distractibility and distorted reality testing []. Neuropsychological testing indicated above average performance in psychomotor speed, word fluency, social cognition and working memory and average performance in executive functioning and visual learning performance []. Routine clinical and laboratory assessments at admission were normal. There was no evident pathology in brain MRI taken at 29 days from admission. Due to intermittent bouts of low blood pressure, and tachy- or bradycardia, aripirazole was changed to quetiapine 300 mg/day, which was rapidly switched to olanzapine 20 mg/day. Olanzapine was then combined with lithium 300 mg/day due to increasingly volatile affects, novel paranoid delusions and an apparent lack of therapeutic effect.
Elbow rigidity, gait abnormalities and tremors had been absent at admission. After 1 month of antipsychotic treatments olanzapine was discontinued due to difficulties in focusing vision, joint rigidity and shuffling gait. Extrapyramidal and motor symptoms continued to worsen nevertheless. Joint rigidity increased gradually, and at the same time the patients speech became slower, verbal content poor, and affects blunted. The poor clinical response to antipsychotics prompted a neurology consultation. An EEG examination was normal but oligoclonal bands of IgG were found in cerebrospinal fluid (CSF) when blood and CSF samples for autoimmune encephalitis were drawn 8 weeks after admission to psychiatric hospital. Mood stabilizing medications were discontinued as neurologic symptoms worsened with opisthotonus posturing, retrocollis, swallowing difficulties, increased salivation, nocturnal incontinence, autonomic instability and periodic unresponsiveness. Malignant neuroleptic syndrome was previously ruled out in an emergency consultation. In the context of fluctuating psychotic, manic and disorganized behavior, a clinical picture of catatonia started to emerge including mutism, mannerisms and incoherence. The patient received a series of three sessions of electroconvulsive therapy without clinical response.
At this time, 82 days after admission to psychiatric hospital, the serum and CSF sample results for NMDA receptor antibodies were reported as positive. IgG antibodies were detected using indirect immunofluorescence of 1:10 diluted serum or undiluted CSF incubated on cerebellar tissue (Clinical Immunological Laboratory, Prof. Dr. med. Winfried Stöcker, Lübeck, Germany). The patient was transferred to a neurology ward and intravenous (i.v.) methylprednisolone 1 g/day and i.v. immunoglobulins 20 g/day were started and commenced for 5 days. Peroral lorazepam was continued at 4 mg/day, and prednisone was started perorally after i.v. treatments at 50 mg/day. Ovarian teratomas and tumors were excluded with a pelvic MRI and whole body computer tomography. Positive titers of serum NMDA receptor antibodies were still measured at 166 days and 242 days after admission to psychiatric hospital. One infusion of rituximab 500 mg i.v. was given at 220 days due to persistently elevated antibody titers.
The premorbid level of functioning of our patient had been excellent, with a highest global assessment of functioning (GAF) level of 91 during the past year. At the time of admission to psychiatric hospital GAF was 43, and at the start of neurologic rehabilitation GAF was 21. Prior to illness, the patient had been working to complete a university degree after a lifetime history of academic success at international schooling. Prejuvenile and juvenile social adjustments were good, [] and there were no history of neurological illness, chronic medical illness, perinatal complications, head trauma, nor history of psychiatric disorders or substance use disorders in the family.
After 50 days of treatment in the neurological ward the patient was discharged. She received 15 sessions of neuropsychological rehabilitation, speech therapy and psychological counseling during outpatient treatment and further functional recovery was gained. Disregarding subjective concentration difficulties, objectives for neuropsychological recovery were attained. Medical status was assessed again at 290 days after admission to psychiatric hospital. All medications had been discontinued 3 months prior to this assessment. The patients weight and abdominal circumference changed marginally between assessments at 14 and 290 days from 55 kg to 54 kg, and 78 cm to 76 cm respectively. At this time GAF was rated at 70, which was the highest level of functioning during the past year. She had only mild subjective experiences of difficulties in verbal expression, distractibility, concentration difficulties, slowed-down thinking and lack of thought energy. Auditory working memory performance was solely reduced in repeated neuropsychological testing compared to baseline, but remained within normal limits. Positive symptoms, catatonia, joint rigidity or gait disturbances were no longer present. There was no detectable pathology or longitudinal change in conventional brain MRI. Serum NMDAR antibody titers were still positive at 408 days from admission The patient has subsequently completed her university studies, and has been since employed in full time work. All subjective neuropsychological symptoms have disappeared and no symptomatic relapses have occurred during a 946 day follow-up.
We further wanted to explore for subclinical morphological changes between the first and latest brain T1 weighted MRI using Freesurfer image analysis suite () for cortical reconstruction and volumetric segmentation in a longitudinal design []. The patient, and eight healthy female controls of similar age, underwent the same structural MRI scan with the Philips 3 T Ingenuity PET/MR hybrid scanner at baseline, and after 9 months with same imaging sequences. We compared regional volume changes between time points with changes measured from the reference sample. Values deviating from the lower or upper quartile for more than 1.5 times the interquartile range (IQR) was chosen as a cutoff for outlier status from reference population. See Additional file : Supplementary methods for details.
In the baseline MRI scan of this case conventional neuroradiological assessment revealed no major pathology. A more detailed volumetric analysis revealed lower volumes in multiple brain regions, but these did not clearly deviate from the control subjects when corrected for intracranial volume. However, the patient had more volume reduction between baseline and the follow-up time point than any subject in the reference group in primarily frontal cortical regions (Fig. ). Movement artefact was minimal, but slightly more prominent in the patient baseline MRI scan compared to the follow-up scan. Movement during a functional MRI series from the same scan session was higher in baseline for the patient, but there was no association of change in movement to change in volume in the complete study sample. |
pmc-6511165-1 | A 46 years old, previously healthy female presented with headache, fits and decreased vision for last 5 months. Headache was diffuse, non-throbbing, moderate intensity and occasionally associated with nausea and vomiting. The patient was suffering from headache every day without diurnal fluctuations. She was on daily analgesics for relieving the pain. A few days later she developed fits with eyes deviating to either side along with drooping of the eyelid and pupillary dilatation to the side where the eye would deviate. (Additional file 2: Movie S1) During the episodes, the patient responded partially and become very irritable afterward. The episode used to last for few minutes followed by irritable mood and crying for the next few hours (Additional file 3: Movie S2). The episodes were occurring daily. In most of the episodes forced eye deviation was to right with right eye ptosis. There was no history of fever, rashes and myoclonic jerks except significant loss of weight in the last 3 months.
At admission, she was conscious, alert, higher mental functions were normal. Cranial nerves were normal. Bulk, tone, power and deep tendon reflexes were normal in both upper and lower limbs. There was no evidence of limb or gait ataxia. Bilateral plantar reflex was extensor.
Haemoglobin was 9.2 g% with microcytosis and anisocytosis. Total leucocyte count was 14,200/cu mm with neutrophil dominance. Liver functions were deranged with raised liver enzymes (SGOT-57 IU/L, SGPT-74 IU/L) and alkaline phosphatase (ALP-507 IU/L). Kidney functions, serum electrolytes and thyroid profile were normal. Her Serum ANA and ENA screen were negative. Chest X-ray showed haziness over the right lower zone. MRI brain axial T2 and coronal T1 weighted images revealed encephalomalacia left frontal area (Fig. ). There was no enhancement on gadolinium contrast. Cerebrospinal fluid examination showed 20 cells all mononuclear, low sugar (55.6 mg% with corresponding blood sugar 145 mg %), and normal protein (41 mg %). CSF did not reveal any abnormal/immature cells. CSF PCR for Herpes Simplex Virus, varicella, enterovirus, Cytomegalovirus, and Japanese Encephalitis was negative. CSF gene expert for Mycobacterium tuberculosis was also negative.
The patient was started on intravenous antibiotics and loaded with phenytoin. As seizures were not controlled antiepileptics were gradually stepped up using levetiracetam, lacosamide, and clobazam in appropriate doses. Frequency of seizures reduced to 1–2/day. The patient was also given a course of intravenous methylprednisolone for 5 days.
As the patient had significant weight loss and abnormal chest X-ray, CT chest and abdomen was ordered to rule out malignancy. CT abdomen of the patient showed heterogeneously enhancing gall bladder mass and CT chest suggested multiple metastatic lesions in the lungs Fig. . Biopsy from cervical lymph node confirmed metastatic adenocarcinoma primary being gallbladder carcinoma (Additional file : Figure S1). Chemotherapy was planned but caregivers denied further treatment and took the patient home. |
pmc-6511172-1 | A 62-year-old male patient was admitted to the hospital due to a 50-year history of intermittent pain and limited activity of the right hip that had been aggravated for 1 month. A radiograph of the pelvis showed high dislocation of the right hip (Fig. ), and the patient was diagnosed with right Crowe type IV DDH. The patient’s visual analogue scale (VAS) score was 8, and his Harris score was 21. A physical examination showed that the right lower extremity was shortened by 5.3 cm, and the patient had a limp, local tenderness of the right hip, and aggravated pain upon internal and external rotation of the hip. The degrees of right hip joint motion were as follows: flexion, 90°; outreach, 12°; adduction, 14°; internal rotation 10°; and external rotation, 5°. The patient reported no other disease history.
The patient was treated with right THA and femoral subtrochanteric shortening transverse osteotomy. The osteotomy was located 1.6 cm below the lesser trochanter, the length of the osteotomy was 2.7 cm, and steel wires were attached at both ends of the osteotomy to prevent fracture. The intraoperative characteristics of the Johnson company S-ROM prosthesis are as follows: bio-type, 44 mm acetabular cup, 28 mm polyethylene liner, 28 mm ceramic head, and standard shank. The patient was treated postoperatively with antibiotics, analgesics, anticoagulants, and gastroprotectants. A radiograph of both lower extremities and the right hip showed satisfactory positioning of the prosthesis after the operation (Fig. ). One day after surgery, the patient could walk in the ward with the help of a walker. One week after surgery, the patient could walk freely but limped because of an evidently tilted pelvis. The patient’s VAS score was 3, and his Harris score was 60. At 40 days after surgery, the patient could put on his socks by himself, and the limp and pelvic tilt were significantly corrected after following the training regimen of the rehabilitation plan. The patient was instructed to practice squatting to facilitate using the toilet. The two ends of osteotomy were in good contact with each other (Fig. ). The patient’s VAS score was 2, and his Harris score was 79. Three and a half months after the operation, the patient reported a slight sensation of bone rubbing and mild pain in the operated hip. The patient’s VAS score was 3, and his Harris score was 70. The ends of the osteotomy had rotated and united poorly (Fig. ). We recommended that the patient undergo hospitalization to receive internal fixation, but the patient requested to continue observation. Five months after the operation, the patient had obvious sensations of bone rubbing, and his right hip pain was significantly worse. The patient’s VAS score was 5, and his Harris score was 52. The ends of the osteotomy had rotated and exhibited nonunion (Fig. ). The patient had obvious symptoms of pain, and hospitalization for surgery was recommended.
The patient was diagnosed with nonunion of the osteotomy and was again admitted for plate and screw internal fixation with bone morphogenetic protein (BMP). The patient was treated postoperatively with antibiotics, analgesics, anticoagulants, and gastroprotectants. Three days after surgery, the patient reported a significant reduction in pain, and the sensation of bone rubbing was absent. The patient’s VAS score was 3, and his Harris score was 60. A radiograph of the right hip and both lower extremities showed that the plate and screw was well fixed (Fig. ). One month after internal fixation was applied, the patient reported that his situation was good. The patient’s VAS score was 2, and his Harris score was 78. The ends of the osteotomy had not rotated and had begun to exhibit union (Fig. ). Two and a half months after internal fixation was applied, the patient’s VAS score was 2, and his Harris score was 85. The ends of the osteotomy were firmly fixed, and the union was progressing satisfactorily (Fig. ). Five months after internal fixation was applied, the patient had only occasional slight pain that did not affect his life. The patient’s VAS score was 1, and his Harris score was 87. Bone scabs had formed at the ends of the osteotomy (Fig. ). Eight months after internal fixation was applied, the patient reported no problems with normal activities. His VAS score was 0, and his Harris score was 90. The ends of the osteotomy had united (Fig. ). |
pmc-6511183-1 | A 75 years old man with a past history of gastric cancer was introduced to Oita Prefectural Hospital for a routine colonoscopy examination. An 18 × 12 mm superficial elevated polyp was detected in the rectum and resected endoscopically.
Microscopically, 90% of the tumor cells showed dysplastic columnar epithelium with hyperchromatic short spindle nuclei regularly arranged in the basal portion and eosinophilic cytoplasm (Fig. a and b). We diagnosed it as conventional tubular adenoma with low grade dysplasia. Additionally, 10% of the tumor cells had dysplastic columnar epithelium with randomly arranged pyknotic polygonal nuclei and clear cytoplasm (Fig. a and b).
Periodic acid-Schiff (PAS), PAS diastase (PAS-D), Alcian blue, and mucicarmine staining were all negative for the clear cell component (Fig. c and d). The antibodies used in this study are listed in Table . Immunohistochemically, both tumor components were negative for CK7, focally positive for CK20, and positive for CDX2 (Fig. e). A difference in results was observed following staining for carcinoembryonic antigen (CEA) (Fig. f). Positive CEA staining was found on the luminal side in the conventional area of the tumor; however, diffuse cytoplasmic staining was observed in the clear cell area. MUC2, MUC5AC, MUC6, CD10, AFP, AR, perilipin, and adipophilin were all negative for clear cell components. The Ki67 (Fig. g) labeling index (LI) was 83.7 and 73.8% for conventional and clear cell components, respectively. Electron microscopic examination found multiple lipid-like vacuoles in the clear cell component but not in the conventional component (Fig. h and i). He received regular follow-up and did not have a recurrence for 4 years. |
pmc-6511183-2 | A 58-year-old man was admitted to Oita University Hospital for the medical examination of an abnormality. The contrast CT examination showed a wall thickness of the sigmoid colon and a colonoscopy was performed. There were multiple polyps detected in the sigmoid colon and a 25 mm in size pedunculated polyp was endoscopically resected. Microscopically, 10% of the tumor cells were conventional tubular adenocarcinoma with hyperchromatic oval nuclei regularly arranged in the basal portion and eosinophilic cytoplasm (Fig. a, b, c, and d). The other tumor cells displayed dysplastic columnar epithelium with large epithelioid or polygonal nuclei randomly arranged and clear or vacuolated cytoplasm, showing cribriform or fused tubular structures and desmoplastic reaction was seen in the surrounding stroma (Fig. b). These findings were thought to be invasion. Tumor invaded into submucosa (pT1b).
PAS, PAS-D, Alcian blue, and mucicarmine staining were all negative for the clear cell component (Fig. e and f). Immunohistochemically, both tumor components were negative for CK7, focally positive for CK20, and positive for CDX2 (Fig. g) and MUC2. The differences in results between the two components were staining for CEA (Fig. h) and CD10 (Fig. i). Positive CEA staining was observed for the luminal aspect in the conventional component; however, there was diffuse cytoplasmic staining in the clear cell component. CD10 was only positive for the clear cell part and adipophilin (Fig. j) was only focally positive for clear cell component. MUC5AC, MUC6, AFP, glypican 3, perilipin, and AR were all negative. COX2 and APC were weakly diffuse cytoplasmic staining for both components, but the staining of APC seemed to attenuate or disappear in invasive areas. The Ki67 LI (Fig. k) was 80.0% and almost 100% for conventional and clear cell components, respectively. An immunoelectron microscopic examination was performed according to procedures described in a previous study [] and showed that the nuclei were surrounded by multiple cytoplasmic lipid-like vacuoles similar to case 1 and they were mostly negative for adipophilin (Fig. l). Postoperative follow-up testing such as a laboratory examination, CT imaging, and endoscopic examination didn’t show any sign of recurrence and he was free from the disease for 1 year and a half. |
pmc-6511195-1 | A 52-year-old African woman presented to our department complaining of 8 months of fever with hematuria, weight loss, decreased appetite, generalized weakness, and intermittent right flank pain. She had a history of pulmonary tuberculosis treated for a 6-month period 10 years ago. Her physical examination was unremarkable. Her temperature was 37.7 °C, blood pressure 124/84 mmHg, and pulse rate regular at 86 beats/min. Laboratory investigations revealed hemoglobin of 10 g/dl, total leukocyte count 15,000/mm3, and elevated erythrocyte sedimentation rate of 150 mm/hr. Liver function test and other biological investigation results were normal. Urinalysis demonstrated urinary pH 6.0, leukocytes 1+, protein 4+, erythrocytes 3+, uncountable leukocyte casts, and negative culture of the urine for pyogenic agents. Abdominal color Doppler ultrasound revealed an enlarged right kidney measuring approximately 8 × 6 cm with minimal flow. Contrast-enhanced computed tomography of the abdomen subsequently revealed a large heterogeneously enhancing mass in the right kidney, measuring approximately 8 × 7 cm, giving a radiological impression of renal cell carcinoma (Fig. ). An enhanced computed tomographic scan showed a normal bladder. No hydronephrosis or wall thickening of the ureter was seen. Considering the clinical presentation as well as laboratory and radiological investigations, a provisional diagnosis of renal cell carcinoma was made, and the patient underwent an open right radical nephrectomy using a transperitoneal approach in view of the large size of the lesion. Radical nephrectomy of the specimen was sent for histopathological examination.
The patient’s postoperative course was uneventful. Surprisingly, histopathological examination of the specimen revealed numerous confluent caseating granulomas with areas of dense inflammation extending into the perinephric fat, suggesting renal tuberculosis (Figs. and ). The patient had received bacille Calmette-Guérin vaccination as a child. A cutaneous tuberculin test was performed (12 mm), and ten samples of urine for mycobacterial culture and bronchoscopy with culture for Koch bacilli from the bronchoalveolar lavage were obtained. All mycobacterial culture results were negative. The result of a QuantiFERON-TB Gold test (Quest Diagnostics, Secaucus, NJ, USA) was positive. Treatment with antituberculosis drugs was started and continued for 6 months. She was in good health after 35 months of follow-up. |
pmc-6511215-1 | A 25 years old female patient of African ethnicity was referred to the Department of Oral and Maxillofacial Surgery, Ayder Referral hospital (Ethiopia) with the chief complaint of a mandibular gingival mass of two years duration. On general examination, the patient was apparently healthy. The medical history and family history were insignificant. No notable findings were recorded on extra-oral examination.
Intra – oral examination revealed a solitary, well defined, roughly oval shaped gingival mass arising from the attached and free labial gingival margin covering two thirds portion of the crown of teeth 33 and 34 (Fig. ). It was a slow growing swelling which gradually progressed to its present size of 2 × 2 cm. The overlying mucosa was intact and the color was similar to the adjacent mucosa. Associated signs or symptoms such as pain, bleeding, discharge, numbness or fever were absent; oral hygiene was inadequate. The swelling was non-tender on palpation with firm consistency and smooth surface texture. Intraoral periapical radiograph (IOPA) of right mandibular anterior region was recorded. A minor arc shaped bone loss in relation to teeth 33 and 34 was demonstrated.
Based on the clinico-radiographical findings, clinical diagnosis of pyogenic granulomas was established with differential diagnosis of peripheral ossifying fibroma, peripheral giant cell granuloma and fibrous epulis. The rare differential diagnosis include benign connective tissue tumors and peripheral odontogenic neoplasms.
Following the routine blood examinations, the lesion was removed in toto under local anesthesia. The excised mass was sent for histopathological examination.
Grossly, the specimen was roughly spheroidal in shape measuring 2 × 2 cm approximately and covered by a capsule with soft to firm consistency. Cut section revealed grayish - white appearance with minute hemorrhagic areas.
The haematoxylin and eosin (H&E) stained sections were examined microscopically. The tumor mass was chiefly composed of varied proportions of spindle/polyhedral, cuboidal and columnar cells arranged in multiform patterns with a few areas showing cystic degeneration. Spindle/polyhedral cells were arranged in the whorled pattern; rosette formation was also observed. Cuboidal to tall columnar cells were arranged in the form of microcysts or ducts (Fig. ). Convoluted rows composed of double layer of columnar cells were also present with an eosinophilic rim of varying thickness between the two layers; these structures were surrounded by proliferation of spindle to polyhedral cells with interspersed eosinophilic material droplets in a hemorrhagic background (Figs. and ).
On the basis of these classical features, the final diagnosis of peripheral adenomatoid odontogenic tumor was made. |
pmc-6511216-1 | A fifty-one-year-old man known for active e-cigarette smoking and history of cigarette smoking, type 2 diabetes mellitus and a personality disorder was brought to the Emergency Department 30 min after injecting himself intravenously in his right forearm with 10 ml of e-liquid with 100 mg/ml of nicotine diluted in propylene-glycol in a suicidal attempt.
On arrival, the patient already complained of diffuse abdominal cramps. He confirmed the intravenous injection of 10 ml of e-liquid in the forearm and brought the product with him. Initial vital signs showed a heart rate of 139 beats per minute, a blood pressure of 170/113 mmHg, a temperature of 36 °C (96.8 °F), a respiratory rate of 41 breaths per minute and a saturation of 100% on room air. Physical examination was irrelevant except for psychomotor agitation and mydriatic pupils poorly responsive to light. No local reaction was visualised around the injection site (on the forearm). The ABG showed a mixed acidobasic disorder with metabolic acidosis and respiratory alkalosis (pH 7.56, pCO2 1.31 kPa, pO2 15,8 kPa and bicarbonate 8.9 mmol/l, lactate 11.1 mmol/l). The anion gap was elevated (31.1 mmol/l) as was the osmolar gap, reaching 16 mOsm/kg. Venous blood analysis showed hypokalaemia (3 mmol/l) and hypophosphataemia (0.23 mmol/l). The ECG showed a sinusal tachycardia without repolarisation changes and the troponins were negative. The patient was initially rehydrated; IV potassium and phosphate infusion was initiated and morphine was administrated to control pain. Two hours post-injection the patient became stuporous with bradypnoea and desaturation. The subsequent ABG showed persistent uncompensated lactic acidosis with the appearance of alveolar hypoventilation, (pH 7.22, pCO2 5.25 kPa, bicarbonate 16.6 mmol/l and lactate 5.7 mmol/l). The patient fell into a coma and was quickly transferred to the Intensive Care Unit (ICU) where he was immediately intubated using rapid sequence induction (etomidate, succinylcholine and fentanyl). For the next 3 h, the patient was not sedated but remained in a profound coma, being unarousable (GCS 3/15) and showing insufficient spontaneous respiration needing controlled ventilation. He presented periodic myoclonic movements of both lower limbs with no abnormal movement of the upper body without improvement after 1 mg of IV clonazepam. Seven hours post-injection the patient recovered spontaneous ventilation and woke up progressively. Ten hours post-injection, the patient was alert and answered simple questions by shaking his head, but his pupils were still mydriatic and poorly responsive to light. We noted a right lateral gaze palsy and flaccid tetraparesia 2/5 with hypoactive deep tendon reflexes, thus preventing extubation. A brain CT excluded any cerebral lesion. Eleven hours post-injection the patient showed complete recovery of motor response and normalisation of deep tendon reflexes allowing extubation.
His mean arterial blood pressure stayed in the normal range without vasopressive agents. We observed a sinus tachycardia (110–130 bpm) and short runs of atrial tachycardia. No ventricular arrhythmias were noted during monitoring. The troponins peaked at 1450 ng/l 24 h post-injection. We concluded a type II myocardial infarction due to sympathetic overstimulation. Daily aspirin was introduced and an ambulatory cardiac assessment was organised.
We noted polyuria up to 400 ml/h with a normal urine analysis and a urine osmolality of 620 mOsm/kg. IV hydration was initiated to compensate for the polyuria, which resolved spontaneously after 8 h. Metabolic acidosis with hyperlactataemia persisted on serial ABGs but it normalised after 11 h.
Cardiologic and neurologic monitoring was performed for 24 h. The patient was discharged after a psychiatric evaluation and the organisation of an out-patient follow-up. A summarized view of the patient’s evolution is shown in Fig. .
Knowing the concentration of nicotine and the quantity injected of a labelled product, we did not ask for more investigations. We confirmed the poising by performing nicotine and cotinine dosages, which were 18 h post-injection12 mcg/l and 3210 mcg/l respectively. The toxicology screen for other common drugs was negative. The presence of Propylene glycol was confirmed as an adjuvant on the label of the bottle the patient brought with him, but its dosage was not available in several universitary laboratories. |
pmc-6511659-1 | A 31-year-old male patient presented to our clinic with symptoms of neck pain, back pain (pain in the lower cervical and upper thoracic region), and numbness in both arms for the last 3 months. His physical examination revealed hypoesthesia at the C4 and C5 dermatomes in both arms with no loss of strength. Cervical computed tomography (CT) showed a destructive and compressive lesion in the C4 vertebra corpus (Fig. ). The retropulsion caused by compression had narrowed the canal. The lesion was also seen to be completely wrapped around the vertebral foramen at the right C4 level and to extend to the lateral mass posteriorly in the axial sections on CT (Fig. ). Weinstein, Boriani, Biagini (WBB) classification was used for the classification of the tumor (Fig. ) []. In this case, the tumor was located at the regions 5, 6, 7, 8, and 9 and invaded all the layers except the dura mater. Corpectomy was performed to the C4 vertebra with an anterior approach together with discectomy to the upper and lower disc spaces during surgery. The lesion was seen to extend to the right C4 vertebral foramen in the surgical observation after corpectomy, and the tumor was carefully dissected 360° around the vertebral artery at this level. Once the vertebral artery was revealed, we entered between the mass extending posteriorly to the lateral mass, the spinal cord, and the vertebral artery and performed meticulous intracavitary curettage. In order to ensure stability after tumor excision, the upper and lower corpus endplates were decorticated with the curette. A corpectomy cage was placed into the C4 space, and the system was fixed by placing a plate screw on the upper and lower vertebra from the anterior (Fig. ). There was no additional neurological deficit postoperatively. The patient’s neurological complaints improved during the postoperative period. There was no residual or remaining tumor after resection. The pathological microscopical evaluation revealed a tumor rich in osteoclastic multinuclear giant cells interspersed in a stroma composed of cells with oval-fusiform nuclei. The pathological diagnosis was giant cell tumor of the bone (Fig. a, b). No recurrence was seen during 3 years of follow-up (Fig. ). |
pmc-6512015-1 | An 18-year-old immune-competent male patient with no significant medical history was admitted to our hospital with 1-week history of headache, fever, and change in mental status suggestive of meningitis. The CSF analysis showed a high leucocyte count, lymphocyte predominance (92%), and high protein (0.92 g/L) and relative low glucose levels (4 mmol/L). Rapid TB PCR, HSV PCR, and India ink staining test results were negative. Chest imaging and sputum analysis did not reveal any positive findings. Initially, he was treated empirically for pyogenic meningoencephalitis. Follow-up computer tomography (CT) and MRI performed 2 weeks later showed hydrocephalus and basal meningeal disease with a pattern of cystic nodular enhancement (Figures and ) highly suggestive of TBM. At this time, the patient was also empirically treated with anti-TB medications (rifampicin, isoniazid, ethambutol, and pyrazinamide) supplemented with steroids. An external ventricular drain was placed, followed by conversion into a VPS. MRI of the spine revealed diffuse smooth dural thickening, representing nonspecific meningitis. The patient showed slow improvement and was discharged only on anti-TB medications.
After approximately 3 months, the patient was readmitted with bilateral leg weakness. The MRI of the spine showed progressive diffuse dural thickening and enhancement with intradural thick-walled abscesses causing cord compression and edema (). The patient underwent spinal decompression. Acid-fast bacilli (AFB) stains were negative and mycobacterial culture did not show growth. However, the patient was continued on first-line anti-TB therapy.
Approximately 6 months later, he presented with shunt dysfunction and an enlarging abdominal cystic mass related to the tip of the VPS catheter revealed by CT of the abdomen (). Laparoscopy showed a large pseudocyst with local inflammatory changes. The pseudocyst was drained and resected and the VPS was removed. Finally, mycobacterium complex was isolated in culture from the cyst aspirates and a CSF shunt tap (mycobacterium complex included M. tuberculosis, M. africanum, M. bovis, M. microti, and M. pinnipedii). Multidrug-resistant TB was confirmed by a drug sensitivity test, given resistance to rifampicin and isoniazid and sensitivity to ethambutol and pyrazinamide. This led to modifying the regimen to a 7-drug therapy including moxifloxacin, pyrazinamide, ethambutol, ethionamide, and cycloserine (for at least 12 months), in addition to Amikacin and Linezolid (for 6 months). An external ventricular drain was inserted and then removed after passing the clamp-challenge test. Patient showed slow progressive clinical improvement with no new disease and stable appearance, specifically with respect to the nodular basal meningitis and ventricular caliber. |
pmc-6512016-1 | Our patient was a 32-year-old previously healthy female at the 39th week of gestation who accessed the first aim department of a primary healthcare centre of a peripheral hospital for severe dyspnoea and chest pain. Her past medical history did not present other hospitalizations for the same symptoms. Due to the clinical manifestations, the patient was initially treated as a case of pulmonary embolic disease and subjected to a massive anticoagulant therapy. Considering the clinical diagnosis and the child to term, an emergent caesarean delivery was performed in order to avoid foetal complications. The caesarean section was successfully performed under general anaesthesia using Stark's method due to the urgency related to the patient's clinical condition of increasing dyspnoea. Moreover, although the pAVM was still unknown at time of the caesarean section, the execution of spinal anaesthesia seems to be not indicated because of the risk of pAVM association with other AVMs, such as those located in the spinal cord, especially in case of HHT.
The foetal outcome showed an Apgar index of 3, 6, and 9, respectively, at minutes 1, 3, and 5; these data are in line with the administration of general anaesthesia and the acute maternal condition of severe dyspnoea.
Taking into account the foetal weight at birth, it showed a restriction of the expected value. The child weight was in fact 2590 gr at 39 weeks of gestation. However, ultrasounds performed during the pregnancy reported a reduction of the potential foetal growth from the 33 weeks of gestation without any Doppler alteration. This phenomenon should be the result of the chronical adaptation of the pregnancy to the unknown pAVM.
As far as the macroscopic exam of the placenta is concerned, a percentage of cotyledons infarcts inferior than 10% was reported.
Considering the patient's postoperative course, it showed a subsequently worsening of the clinical conditions, resulting in an acute distress syndrome that required an immediate transfer to the Gynaecology and Obstetrics unit of our structure. Due to the critical care panel and the low clinical conditions, the patient was intubated and housed in the ICU department. Considering the acute distress syndrome, a chest CAT scan was performed highlighting the presence of a left pAVM expanded, associated with a massive hemothorax that compressed the correspondent lung. The vital signs panel showed systolic blood pressure of 70 mmHg, diastolic blood pressure of 35 mmHg, pulse rate of 150/min, pulse oximetry saturation 88% on 100% inspired oxygen, afebrile temperature, and respiratory rate of 40/min. Initial labs revealed normal platelets, normal coagulation panel, and haemoglobin of 7 gm/dL. Critical care panel showed pH of 7.4, pCO2 of 43 mm hg, pAO2 60 mmHg, and saturation of 88%. After the placement of a chest tube, 3 litres of frank blood were removed; this action resulted in a normalization of the blood pressure and improved oxygenation on the monitor. The successive management was the clinical observation of the patient's conditions as well as the vital signs and labs test in order to perform the pAVM embolization when the patient clinical conditions will be stable. After three hours from the drainage, worsening of the patient conditions was observed reporting a severe collapse of the vital signs as well as a decrease of antithrombin III, fibrinogen, and haemoglobin values, with parameters of 33%, 122 mg/dl, and 5.8 gm/dl, respectively. Moreover, considering the postoperative caesarean course, the gynaecologic clinical evaluation showed a low uterine fundus contraction and the presence of conspicuous abnormal lochia. Uterine fundal massage was performed as first approach to solve the uterine low contraction followed by Credè's manoeuvre. Due to the failure of both, a pharmacological treatment was attempted starting with a simultaneous administration of intravenous Oxytocin (10-40 UI per 1 litre saline solution) and intramuscular Methylergometrine (0.2 mg one dose). The latter pharmacological approach involves the use of intravenous Sulprostone (0.5 mg per 1 litre saline solution) that was administered within half an hour from the signs of low uterine contraction and abnormal lochia. None of the previous pharmacological treatments succeeded.
Considering the reproductive age of the patients, procedures as appositions of tamponade-balloon and embolization of the uterine arteries were taken into account but were not applicable in order of the unstable and precipitant parameters of the woman. Due to the patient's life-threating condition, a simultaneous surgical intervention of thoracic surgeons and gynaecologists had been necessary to solve the urgency, with the performance of a contemporaneous surgical reparation of the pAVM and resection of damaged left lower lobe (LLL) as well as a preventive hysterectomy to avoid the risk of disseminated intravascular coagulation (DIC). The surgical interventions were performed successfully but intraoperative blood transfusions and administration of antithrombin III and fibrinogen were necessary. The postoperative treatment showed a normalization of vital signs and labs panel as well as patient's clinical conditions. Due to the stable condition of the woman, the anaesthetist established the patient's autonomous breathing. After one week from the intervention, chest CT with intravenous contrast was performed showing a 4 cm area of active contrast. Pulmonary angiography confirmed the presence of a pAVM with feeding branch of a basilar left pulmonary artery supplying aneurysmal AVM and dilated draining vein. Transcatheter embolotherapy (TCE) of the culprit vessel was performed by placement of a nonadhesive liquid embolic agent (Onyx 34®). Repeated chest X-ray and chest CT after one week from TCE showed expansion of remaining left lung and signs of pAVM embolization and pulmonary resection of LLL, respectively (). The patient course was subsequently uncomplicated and the discharging home happened after 14 days. MRI evaluation was performed in order to detect any head AVM but the result was negative. The genetic testing for HHT was not performed during this hospitalization period but the genetic examination performed a few months afterwards showed no association. |
pmc-6512020-1 | A 48-year-old woman was referred to the emergency department with a one-week history of fever and cytopenia (Hb 8.7 g/dL, normal range 11.8–15.5; platelet count 77 × 109/L, normal range 155–346). Clinical examination found a huge splenomegaly, confirmed by abdominal CT scan but no arthritis. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18FDG PET-CT) showed an enlarged hypermetabolic spleen and perisplenic and hilar hepatic hypermetabolic lymph nodes with hypermetabolism around the cervix (). Biological investigations revealed mild anemia (Hb 8.4 g/dL), severe thrombopenia (platelet count 40 × 109/L), elevated CRP (200 mg/L, normal range <10), acute liver cytolysis (GPT 57 U/L, normal range <34), hyperferritinemia (ferritin 4724 μg/L, normal range 30–350), increased LDH (4482 U/L, normal range <214), increased soluble CD25 or soluble IL-2 receptor (>30000 pg/mL, normal range 632–4883), and biological intravascular disseminated coagulation (PT<50%, normal range >70% and fibrinogen 77 mg/dL, normal range 160–400). H-score, according to those results, was 99.6% [, ]. All this pattern was suggestive of HPS [, ]. Bone marrow aspiration and biopsy initially did not confirm and were not contributive for the etiology of HPS. All the infectious serologies including HTLV 1 and 2, HHV 6 and 8, parvovirus B19, Toxoplasma, and Borrelia were negative. EBV PCR and CMV PCR were negative. Evolution was marked by apparition of jaundice, worsening of liver function (total and conjugated bilirubin, alkaline phosphatase, gamma glutamyl transferase, and glutamic oxaloacetic transaminase were 4–6 times above normal range), and unexplained lactic acidemia (lactacte 8.9 mmol/L, normal range <2 mmol/l) without any clinical manifestation. Liver biopsy was performed, finding cholestasis without any inflammation probably due to toxic etiology. Etoposide (2 courses: first 300 mg and then 200 mg eight days later) and dexamethasone were given with good evolution. The patient was discharged on reduced doses of prednisone, with prednisone 8 mg/day planned to be gradually tapered.
She was readmitted 3 weeks after discharge and 1 week after discontinuation of prednisone, for nausea and vomiting in the setting of inflammatory syndrome (CRP 117 mg/L). Clinical examination was unremarkable. New biological investigations were suggestive of HPS (sCD25 at 15000 pg/mL and ferritin at 1740 μg/L) with evidence of hemophagocytosis at bone marrow aspirates, with acute renal failure and hypopituitarism (), clearly concerning gonadotropic axis and thyrotropic axis. Gonadotropic hormone levels were as follows: estradiol <25 ng/L, progesterone <0.2 mcg/L (corresponding to menopause values), LH 2.5 IU/L (normal range: 7.7–58.5), and FSH 7 IU/L (normal range: 25.8–134.8). FSH and LH levels were inappropriate regarding progesterone and estradiol levels, although the patient was not taking oral contraceptives. Regarding thyrotropic axis, TSH was 0.1 mU/L (normal range: 0.3–4.2); free T4, 0.5 ng/dL (normal range 0.9–1.7); and free T3, 1.8 pmol/L (normal range 3.1–6.8). Growth hormone was 0.68 mcg/L (normal range 0.06–6.88), and IGF-1 dosage was performed. Corticotropic axis seemed to be normal: cortisol was 350 nmol/L in the morning (normal range 166–507) and ACTH was 11.4 ng/L (normal range 7.2–63.3). Finally, there was no diabetes insipidus. Brain MRI presented an enlarged pituitary gland and pituitary stalk with nonspecific lesions (). A new 18FDG PET-CT showed a reduction of size and hypermetabolism of the spleen but no pituitary hypermetabolism. Levothyroxine and hydrocortisone substitution were started, the latter considered given the concern of an increased steroid need due to pyrexia and the diagnostic splenectomy rapidly planned. A rapid onset of paraparesis with urinary and fecal incontinence led us to perform a new brain MRI. It showed a hypersignaling (T2/flair) medial medullary lesion associated with corticosubcortical prerolandic homogeneous lesion (). Analysis of CSF showed an inflammatory liquid with increased protein (0.80 g/L normal < 0.40) but normal cell count (2 cells/mL).
Given the result of histological examination, the patient has been treated by two courses of R-CHOEP with a three-week interval (rituximab 375 mg/m2; cyclophosphamide 750 mg/m2; doxorubicin 50 mg/m2; vincristine 1.4 mg/m2; etoposide 100 mg/m2 on day 1, 2, and 3; and methylprednisolone 80 mg/day), together with weekly intrathecal infusion of methotrexate 15 mg, cytarabine 40 mg, and methylprednisolone 40 mg. Under this treatment, a marked decrease in the size of the pituitary gland and pituitary stalk was noted. Nevertheless, two months after beginning this treatment, the patient died from several infections in the context of persistent medullary toxicity. No autopsy was performed. |
pmc-6512028-1 | A 36-year-old man was diagnosed with synovial sarcoma around the left knee joint. After 4 courses of preoperative chemotherapy, we planned wide excision including the peroneal nerve and fibular head. We planned reconstruction of the resulting soft tissue defect using a free latissimus dorsi muscle flap and reconstruction of the peroneal nerve defect using a sural nerve graft. For the recipient vessels, the peroneal artery and vein were selected after confirming patency by enhanced computed tomography and ultrasonography.
The size of the soft tissue defect after wide excision was 11 × 13 cm (). Thus, an 11 × 15 cm latissimus dorsi muscle flap was elevated. When the recipient vessels were incised after dissection, arterial blood flow was not observed. We diagnosed vasospasm and attempted to warm up the vessels using warm saline, along with topical application of heparin solution and 2% lidocaine. However, no arterial blood flow was achieved after 15 minutes. Subsequently, we sprayed approximately 5 mL of a 15-fold dilution of fasudil hydrochloride (ERILTM, Asahi Kasei Pharma, Japan) with saline around the recipient vessels. Arterial blood flow and pulsation appeared soon after application, and arterial blood spouting was achieved after approximately 1 minute. Thereafter, 1 artery and 2 veins were anastomosed, and the wound was sutured after confirming there was no bleeding point in the operative field (). No complications, such as wound hemorrhage or hematoma formation, were observed after surgery, and the flap completely survived. |
pmc-6512029-1 | A 70-year-old Asian male presents to the emergency room with a 2-week history of productive cough and fever. Temperature usually as high as 102°F occurred mostly at night time with associated chills. Cough was productive of whitish sputum without blood. He had seen his primary care doctor as an outpatient and was prescribed 5 days of amoxicillin/clavulanic acid for presumed community-acquired pneumonia. His history is significant for HIV diagnosed about 8 months ago with CD4 lymphocytes count of 121 cells/mm3 and an HIV viral load of 109,720 copies/mL. At that time, he was started on atovaquone for Pneumocystis jirovecii pneumonia prophylaxis. He has been on dolutegravir/emtricitabine/tenofovir alafenamide with his most recent CD4 lymphocyte counts above 200 and HIV viral load 20. Physical examination was positive for splenomegaly but otherwise unremarkable. His blood tests showed hemoglobin of 6.5 g/dL and platelet 81,000 × 106. Chest X-ray showed no infiltrates. The patient was transfused 2 units of packed red blood cells (pRBCs) with improvement of hemoglobin level; however, the hemoglobin level kept declining, and needing repeated pRBCs transfusion. Platelet levels plummeted to the 20s also requiring platelet transfusions. Stool occult blood was repeatedly negative. Blood cultures were negative, but the patient kept spiking fever intermittently (100–105°F). A CT of the abdomen and pelvis revealed multiple enlarged lymph nodes in the chest, abdomen and pelvis, and hepatosplenomegaly. Further testing revealed a ferritin of 7953 ng/mL, IL-2 receptor alpha interleukin 8592, EBV PCR <100 copies/mL, and CMV PCR neg. Bone marrow biopsy of the posterior iliac crest was consistent with hemophagocytosis (). An excisional biopsy of an axillary lymph node showed HHV-8 associated multicentric Castleman disease with plasmablastic aggregates and Kaposi sarcoma on CD 138 stain (). The patient continues HAART and was started on dexamethasone and etoposide therapy. Unfortunately, patient's condition continued to deteriorate, and family decided to get a palliative consult and place the patient on comfort care until the patient expired. |
pmc-6512030-1 | This is a 61-year-old Caucasian female with significant past medical history of ovarian cancer complaining of shortness of breath for several weeks. Five years prior, the patient was diagnosed with stage IC clear-cell ovarian carcinoma and had undergone robotic-assisted laparoscopic hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic and periaortic lymphadenectomy, and 3 cycles of carboplatin and paclitaxel intravenous and intraperitoneal with no evidence of disease on imaging. Her last cancer antigen 125 (CA 125) level was 8. Unfortunately, she lost a follow-up with her oncologist until this hospitalization. On admission, she stated symptoms started 2 weeks prior, were worse on exertion, and were associated with a dry cough and 10 pounds of unintentional weight loss. She denied fevers, chills, night sweats, chest or abdominal pain, diarrhea, or constipation. Reproductive history was significant for 2 full-term vaginal deliveries with 2 living sons, menarche at 12 years old and menopause at 56 years old. Her family history was significant for her paternal grandmother with breast cancer in her 60s, but no history of gynecologic or colon cancer. She denied ever using tobacco, alcohol, or illicit drugs. Upon further questioning, she stated that over the past 6-8 weeks, she noticed a tender lump in her right breast. On admission, vital signs were significant for oxygen saturation of 92% on 4-liter nasal cannula. On physical exam, she was an ill-appearing thin female in mild distress secondary to shortness of breath. Lung examination yielded decreased breath sounds bilaterally and diminished at the bases. Breast examination yielded a firm right-sided chest mass just right of midline measuring 8 × 4 centimeters. Complete blood count and metabolic panel were unremarkable. Chest radiography showed a large left-sided and small right-sided pleural effusions (). Computed tomography (CT) with angiography revealed a right medial breast mass, mediastinal and axillary lymphadenopathy, and bilateral effusions, greater on the left (). CT abdomen and pelvis showed a small amount of ascites and mesenteric metastases. An ultrasound-guided thoracentesis was performed which aspirated 1.1 liters of clear yellow fluid from the left pleural space. The fluid was sent for cell culture and cytology. The fluid was consistent with metastatic clear-cell carcinoma of the ovary. Ultrasound of the breasts showed a right-sided dominant malignant appearing lesion in medial breast with axillary adenopathy and left-sided multiple malignant appearing breast and pectoral lesions with left axillary adenopathy. She underwent ultrasound-guided biopsy of left and right breast lesions as well as left axillary lymph node. Pathology from both breast tissue samples revealed Mullerian epithelial primary, consistent with metastatic clear-cell carcinoma of the ovary (Figures and ). Repeat CA-125 was strongly positive at 318. She was referred to gynecology oncology service. Upon follow-up, she was started on chemotherapy with docetaxel, carboplatin, and bevacizumab. Next-generation sequencing was ordered. Radiation oncology was consulted. Genetic counseling and testing were recommended. Her cancer was re-staged as stage IV clear-cell carcinoma of the ovary with metastases to the bilateral breasts, axillary lymph nodes, and abdomen. |
pmc-6512031-1 | A 27-month-old previously healthy boy presented to the emergency department with repeated vomiting, sweating, generalized weakness, dizziness, anxiety, and reduced consciousness. He was found to be in shock with a heart rate of 200 beats per minute, respiratory rate of 49 breaths per minute, and blood pressure of 84/43 mmHg. The abdomen was soft but grossly distended with sluggish bowel sounds. Arterial blood gas revealed metabolic acidosis with a pH of 7.12 and base excess of −14. The arterial lactate level was 5 mmol/L. Initial abdominal radiography showed diffuse bowel dilatation but no apparent air-fluid level and two circular radiopaque opacities in the bowel suggestive of metallic foreign bodies (). The patient was admitted to the paediatric intensive care unit for resuscitation. He was stabilized with intravenous fluids and ionotropic support. Emergency laparotomy revealed small bowel obstruction with extensive necrosis. Approximately 107 cm of gangrenous small bowel was resected, and end-to-end anastomosis was performed. Two magnetic beads sized 5 mm × 5 mm were found (), one in the small bowel and the other in the right colon. The magnetic beads were removed. Postoperative recovery was uneventful. Retrospective questioning of the parents revealed no history suspicious of FB ingestion. |
pmc-6512031-2 | A 9-year-old boy presented to the emergency department immediately after accidental ingestion of magnetic beads. The patient was asymptomatic and vital signs were stable. There were no signs of obstruction or perforation. Initial abdominal radiography showed five round radiopaque objects in the epigastrium (). Aggressive management was employed in view of the multiplicity of the beads ingested and potential risk of serious complications. Emergency oesophagogastroduodenoscopy showed no foreign body up to the second part of the duodenum. The beads had moved further beyond the duodenum. Laparoscopy was then performed which revealed a string of five magnetic beads adhered to each other in the small bowel. The beads were removed via enterotomy. The patient remained asymptomatic and stable and made an uneventful recovery. |
pmc-6512033-1 | A male patient, aged 19 years old, was referred to a private orthodontic practice. He was diagnosed with a bilateral Class I canine relationship and left Class I molar relationship in the permanent dentition with a crossbite of tooth 12 (Figures –). Patient chief complaint was to resolve the anterior crossbite with an invisible orthodontic appliance.
A lingual orthodontic appliance has been projected using a CAD/CAM digital workflow. The objective of the treatment was the resolution of anterior crossbite and retention of the case over time [].
As the patient wanted to avoid conventional vestibular orthodontic treatment, a lingual orthodontic appliance was chosen. Other alternatives to conventional visible metallic attachments would have been orthodontic treatment with ceramic brackets or aligners that are both effective to obtain tooth movement [, ]. The patient, however, wanted to avoid ceramic brackets because of the possibility of adverse staining midtreatment and refused aligners so as to avoid treatment with removable appliances.
Written informed consent was obtained by the patient to proceed with orthodontic diagnosis and treatment. The procedure for the virtual indirect bonding started with the polishing of all dental surfaces with pumice powder; then, intraoral scan () was performed by a 3D intraoral scanner (True Definition, 3M, US). NemoCast software (Dentaurum, Germany) acquired the 3D models and recognized the shape of each tooth and the gum. The software realized a virtual set-up, and then, the orthodontist placed the virtual brackets on 3D virtual models according to the lingual prescription (). The bracket placement tool ensured the precise bracket positioning according to the virtual set-up using the virtual bracket's position on the screen. Once all brackets were positioned on the 3D models, the software allowed designing a virtual transfer tray for indirect bonding (). A prototype of the digital transfer tray was manufactured using a rapid-prototyping machine (). exocad software (exocad GmbH, Germany) was used to create bilateral posterior overlays to act as customized bite raisers (Figures and ).
The patient wore protective glasses to prevent eye damage [], and the tooth surfaces were first etched with 37% orthophosphoric acid gel (3M, US) for 30 seconds () followed by washing and drying. A primer (Transbond XT, 3M, US) was applied in a thin film on the etched tooth surface (). 2D lingual brackets (Forestadent, Germany) were positioned inside the prototyped transfer tray, and an adhesive resin (Transbond XT, 3M, US) was applied over the bracket bases []. The brackets were then positioned in the maxilla on the upper teeth (), using the prototyped transfer jig, and were light cured for 60 seconds each by an LED lamp (Elipar DeepCure, 3M, US). Subsequently, the transfer tray was removed by forcing a probe in the fracture line (Figures and ) and separating the jig in two halves. The fracture line was created by the CAD software in order to be useful for an easy transfer tray removal.
On the lower jaw, prototyped overlay were set using an adhesive technique (). Orthodontic treatment progress included 0.012 inch, 0.014 inch, and 0.016 inch nickel titanium archwires, followed by 0.16 stainless steel wire. The patient was checked each month, and wires were changed after 2 months each. Finally, brackets were removed, and teeth were polished (Figures and ). An upper splint and an upper Essix removable appliance were placed in order to guarantee posttreatment stabilization [, ]. |
pmc-6512040-1 | The patient is a 70-year-old man, with a history of nonvalvular atrial fibrillation (NVAF) with prior stroke, chronic obstructive lung disease, hypertension, diabetes mellitus, chronic foot ulcers, and frequent falls, who initially presented with a fall 5 days after Watchman™ device placement. His history of NVAF was complicated by multiple episodes of syncope, despite various strategies including antiarrhythmic therapy. He was previously on dabigatran which resulted in severe bruising and rivaroxaban which was intolerable due to headaches. After discussion with his cardiologist, a Watchman™ device was placed given his high thrombotic risk and prior complications with oral antithrombotic therapy (CHADS2-VASC2 score of 6 and HAS-BLED score of 4) [, ]. After device placement, he was started on apixaban 5 mg twice daily plus aspirin 81 mg daily, with the plan for continuation for the following 45 days. However, 5 days after device placement, he presented to the emergency room after a fall, with lethargy, fever, and hypotension. He was found to be in septic shock from methicillin-resistant staphylococcus aureus (MRSA) bacteremia. His hemodynamics and mental status initially improved in the intensive care unit (ICU) on vasopressors, stress dose steroids, and broad-spectrum antibiotics, which were subsequently narrowed to intravenous vancomycin. In addition, his apixaban was transitioned to intravenous unfractionated heparin upon presentation to the ICU. In spite of hemodynamic improvement, his mental status worsened. Magnetic resonance imaging (MRI) of the brain revealed multiple acute small punctuate infarcts in the left corona radiata, right occipital cortex, and right frontal deep white matter. These multifocal strokes were thought to be cardioembolic, not septic in origin. Upon further questioning, the patient admitted to missing doses of apixaban post LAOO device placement. A transesophageal echocardiogram (TEE) was obtained; no thrombus in the left atrial appendage was noted, and there were no vegetations or patent foramen ovale. A well-positioned 27 mm Watchman™ device was visualized occluding the LAA orifice with a 1.3 mm paradevice leak, stable from prior. Given high concern for potential seeding of the newly implanted Watchman™ device, a six-week course of intravenous vancomycin plus oral rifampin was recommended by infectious disease consultants as a means of targeting MRSA and additionally preventing biofilm formation and device seeding. This was to be followed by a prolonged suppressive course of oral antibiotics.
The patient improved clinically with recovery of mental status and was deemed ready for transition from unfractionated heparin to an oral anticoagulant. Given the concerns about significant drug interactions with either warfarin or a DOAC with concomitant rifampin, a strong cytochrome (CYP) 3A4 inducer, strong permeability glycoprotein (Pgp) inducer, and moderate CYP2C9 inducer, a shared decision was reached to use weight-based LMWH with enoxaparin to complete the 45 days post Watchman™ device implantation.
The patient refused repeat TEE at 45 days, and anticoagulation therapy was continued with enoxaparin until the device could be evaluated. Two months after Watchman™ device placement, a repeat TEE was performed, revealing improvement in the paradevice leak from 1.3 mm to 1 mm, and the antithrombotic therapy was transitioned to DAPT with aspirin 81 mg daily and clopidogrel 75 mg daily. A repeat brain MRI obtained 4 months post LAOO device placement noted a right cerebellar chronic hemorrhage with numerous susceptibility foci distributed in the bilateral supratentorial and infratentorial compartment predominantly in the periphery of the brain parenchyma. Given the short interval since the prior MRI, the presence of these foci was concerning for microemboli of likely cardiac origin. A repeat TEE did not reveal a thrombogenic focus; however, there was persistence of paradevice leak at 1.3 mm. Anticoagulation therapy with apixaban was resumed, and DAPT was discontinued, due to the persistent device leak. The patient continues to struggle with worsening frequent falls; however, he remains functional at home with continued rehabilitation with physical therapy. He continues to take apixaban, living with the risks of associated bleeding that Watchman™ device placement was intended to reduce. |
pmc-6512045-1 | The patient is a 43-year-old male with a history of a traumatic left distal biceps tendon rupture (now, three years status post uncomplicated repair), remote right shoulder pain managed successfully with physical therapy without recurrence, and chronically low testosterone managed with weekly testosterone injections—who presented one day after a traumatic bilateral shoulder injury. The patient describes an ejection over the handlebars of his bicycle, landing in a “push-up” position, shoulders abducted to approximately 90°, and elbows flexed to 90°. He noted deep shoulder pain and internal rotation limitations, bilaterally. At the time of presentation, his pain had mildly improved, but functional status of both shoulders remained unchanged. On exam, his left shoulder was tender to palpation anteriorly with passive forward flexion to 140° and 4/5 strength, passive abduction to 120° and 4/5 strength, external rotation to 20° and 4/5 strength, internal rotation to 10° and 3/5 strength, a positive liftoff test, positive bear hug test, and a positive belly press test. His right shoulder was tender to palpation anteriorly with passive forward flexion to 125° and 4/5 strength, passive abduction to 90° and 4/5 strength, external rotation to 20° and 4/5 strength, internal rotation to 5° and 2/5 strength, a positive lift-off test, and a positive belly press test. X-rays of both shoulders were obtained with anteroposterior (AP), scapular-Y, and axillary views, which showed no signs of fracture, dislocation, or deformity, bilaterally. A noncontrasted MRI () obtained two days later demonstrated complete rupture of the right subscapularis tendon, just distal to the musculotendinous junction; complete rupture of the left subscapularis tendon, just distal to the musculotendinous junction; and bilateral type 4 SLAP lesions. There were no other signs of osseous or soft tissue injury; the remainder of both rotator cuffs was intact, and there was no fatty infiltrate in either subscapularis muscle belly. The patient was reevaluated in the office 10 days postinjury, and a video was obtained of his preoperative examination (), at which point he was scheduled for arthroscopic subscapularis tendon repair of the right shoulder (2 weeks postinjury), as this was his dominant side, and then the left shoulder seven weeks later. Intraoperative findings () included complete subscapularis rupture on both sides, with type 4 SLAP lesions and associated partial tear of the long head of the biceps at its labral origin. As suggested on MRIs, the remainder of the rotator cuffs was intact. Given the delayed nature of the left-sided surgery, there were some muscle retraction and scarring of the left subscapularis that increased the difficulty of surgery, though both tendons were adequately repaired without need for soft tissue grafting or augment. |
pmc-6512066-1 | A 68-year-old female presented to the emergency room with a 3-day history of acute abdominal pain, nausea, and emesis. She had not felt well for several weeks with poor appetite, intermittent abdominal cramping, and right flank pain. Significant comorbid conditions included hypertension and hyperlipidaemia; she was an active smoker. Medical history included abdominal hysterectomy, and as a child, she underwent lumbar surgery with resection of a right paravertebral sarcoma, for which she also received chemotherapy and radiation. No medical records for this disease were available and the patient could not give additional information. As a sequela of her treatment, she had a large bulge in her right flank with significant soft tissue swelling and telangiectatic skin. She knew about the lumbar hernia, which developed few years after her surgery, but she was never symptomatic and did not desire repair.
On examination, she was tachycardic and short of breath. Her abdomen was significantly distended and tender, and there was a large bulge noted in her right flank. Her white blood count was elevated at 14 K/microL, serum sodium (132 mEq/L) and chloride (93 mEq/L) were low, and creatinine was borderline elevated (1.7 mg/dL), reflecting dehydration. Pain control was achieved and she was resuscitated with 2 liters of normal saline while oral contrast was given. CT scan showed a large, incarcerated, posterolateral abdominal hernia with free fluid and possible free air and distended bowel loops indicative of obstruction (Figures and ). A nasogastric tube was placed and 1500 mL of small bowel content was evacuated and she was posted for emergency surgery.
She was placed supine and the abdomen was accessed with a 5 mm Kii Fios first entry port (Applied Medical, Rancho Santa Margarita, CA) in the left upper quadrant (LUQ). Pneumoperitoneum was established and a second 5 mm trocar was placed under visual control in the left lower quadrant (LLQ). Multiple distended small bowel loops and a distended ascending and transverse colon were visualized. Superior to the proximal transverse colon and lateral and inferior to the liver, the roof of the hernia with the hepatic flexure trapped inside was seen. The small bowel segment trapped inside the hernia was reduced and found to be hemorrhagic but there was no perforation. The small bowel was eviscerated from a 4 cm minilaparotomy using her old subumbilical midline incision, and the hemorrhagic loop did not recover and seemed unsalvageable (). The small bowel distally was collapsed. The healthy segments proximal and distal to the damaged loop were aligned, hold stitches were placed, and through the enterotomies for the linear stapler, the proximal small bowel was decompressed. The anastomosis was created using a GIA-75 stapler, and the damaged small bowel loop () was resected together with the common enterotomy with a second transverse staple load. Pathology confirmed extensive hemorrhagic necrosis of the mucosa extending in some areas into the muscle layer. The bowel was placed back into the abdomen, the minilaparotomy was closed, and pneumoperitoneum was reestablished. Visualization was now much improved. The anastomosis was laid in the RUQ. The hernia was noted to be large, with the hepatic flexure partially trapped in it (). Decision was made not to repair the hernia at this stage due to the contamination from the damaged small bowel loop, but laparoscopic hernia closure using a mesh was planned to take place within few days.
Three days later, the patient complained of increasing abdominal distension and abdominal and right flank pain. X-ray showed increased distension of small bowel loops and recurrent incarceration of bowel was suspected. At emergency laparotomy through extension of the previous incision cranially and caudally, significantly dilated small bowel loops, transverse colon, and right hemicolon were noted. These were decompressed through the nasogastric tube but no bowel was incarcerated within the hernia. A large hematoma was found in the right flank, lateral and dorsal to the ascending colon. The white line of Toldt was opened, and a portion of the hematoma was carefully suctioned out, and a pad was placed to tamponade the retroperitoneum. No acute bleeding source was identified. The ascending and transverse colon were mobilized out of the hernia. The duodenum was kocherized and the Gerota fascia was exposed. We opted not to mobilize the right kidney due to fear that a major retroperitoneal bleed could be caused from the source that had fed the hematoma and was now tamponaded. The hernia borders could be determined but no primary closure was possible. Therefore, the defect was covered using a 20 × 20 cm BIO-A mesh (Gore, Flagstaff, AZ). The mesh was placed laterally and dorsally onto the Gerota fascia as the kidney was covering this portion of the hernia and tucked under liver segment 6. Absorbable tacks were used to secure the mesh into the anterior and lateral abdominal wall. The duodenum and right colon were placed into the created mesh pocket. A 19-Blake drain was placed on top of the mesh, next to the colon.
CT scan the next day identified a 2 cm pseudoaneurysm of a side branch of a lumbar artery as source of the hematoma (Figures and ). This was subsequently embolized by interventional radiology (). The postoperative course was complicated by Clostridium difficile-associated colitis, but ultimately, the patient recovered fully. At 6-month follow-up, she had gained weight, had continued to refrain from smoking, and had no evidence for recurrent hernia. On CT scan, the mesh covered the previous defect (Figures –) and no recurrent hernia developed. Tissue changes in the area of the previous radiation were still visible, and percutaneous biopsy was recommended to exclude development of a secondary malignancy, which the patient at this time declined. |
pmc-6512067-1 | In 2016, a 74-year-old woman diagnosed with IgA κ-type smoldering MM was hospitalized after developing malaise and fatigability. Physical findings at admission included pale palpebral conjunctiva and edema of the bilateral lower limbs. Laboratory test results were as follows: calcium, 11.4 mg/dL; hemoglobin, 6.1 g/dL; creatinine, 1.54 mg/dL; total protein, 6.4 g/dL; albumin, 3.2 g/dL; IgA, 2923 mg/dL; IgM, 29 mg/dL; IgG, 2253 mg/dL; beta-2-microglobulin, 17.6 mg/L; free kappa light chain, 82.0 mg/L; and free lambda light chain, 18.7 mg/L. Computed tomography (CT) revealed extramedullary tumor in the mediastinum, bilateral axilla, and pulmonary hilum. On fluorescence in situ hybridization, t(14; 16), t(4; 14), t(11; 14), and deletion 17p results were all negative. Symptomatic myeloma (the International Staging System (ISS) stage III and revised ISS stage III) was diagnosed. After high-dose DEX therapy, BOR plus DEX therapy was administered. Although partial response was obtained with four courses, IgA levels had exacerbated by the end of eight courses, and progressive disease was determined. Treatment was switched to LEN (15 mg/day) plus DEX therapy; however, severe cytopenia developed, and treatment was discontinued after one course. CT revealed extramedullary tumor in the para-aortic region, which was thought to indicate disease progression, in addition to low uptake throughout the entire liver (). Therefore, treatment was changed to CFZ (20 mg/m2 on days 1 and 2; then 36 mg/m2 on days 8, 9, 15, and 16) in combination with LEN (5 mg/day) and DEX (KRd) therapy. After administration of KRd therapy, peripheral oxygen saturation had been reduced to 88% early on the next day, and transnasal oxygen therapy was initiated. The brain natriuretic peptide level increased to 418 pg/mL; however, there was no increase in myocardial escape enzymes. Ejection fraction (EF) reduced from 59% to 28% (), and radiography revealed pulmonary congestion with pleural effusions. Thus, CFZ-induced acute heart failure was diagnosed. On day 2 of the administration, CFZ was discontinued. Despite the use of norepinephrine, dobutamine, furosemide, and human atrial natriuretic peptide, diuresis was not improved. The patient's respiratory condition deteriorated, and on day 5 following the CFZ administration, noninvasive positive pressure ventilation was initiated. Oxygenation did not improve, and the patient died early morning on day 8 following the CFZ administration. Autopsy was performed following death with the consent of the patient's family.
Macroscopic findings on autopsy revealed multiple vertebral osteolysis, hepatosplenomegaly, renal enlargement, and innumerable lymph node lesions in the paratracheal, tracheal bifurcation, and para-aortic areas. Pathological findings of the bone marrow showed basophilic cells with an eccentric nucleus and proliferation of large cells, which were positive for IgA on immunostaining (). The spleen exhibited invasion of similar cells replacing the splenic white pulp, as well as invasion into the splenic red pulp. In the liver, tumor cell invasion was accompanied by destruction of the limiting plates, primarily in the portal region. With the tumor cell invasion, destruction or loss of interlobular ductules and dilation of bile canaliculus were observed. In the kidneys, tumor cell invasion of the interstitial tissue was observed, accompanied by glomerular and renal tubule destruction and loss. The distal convoluted and collecting tubules were filled with eosinophilic amorphous deposits, which was positive for κ light chain (Figures and ). There was no obvious thrombus in the coronary artery of the heart, and no infarction was observed in the myocardium. Invasion of inflammatory cells, primarily CD3- (cluster of differentiation 3-) positive lymphocytes, was observed between the myocardial cells, and these myocardial cells showed basophilic degeneration. There was no clear tumor cell invasion or amyloid deposition, and very little myocardial cell necrosis was observed. Between the myocardial cells, fibrosis and disarray were observed (Figures –). |
pmc-6512394-1 | A 52-year-old obese (100 kg, 1.80 m) Caucasian man presented in January 2017 with multiple nodules with purulent drainage on the upper extremities persisting for more than 10 years. Several attempts of treatment with oral antibiotics had been unsuccessful. The number of nodules was increasing over the time. He had a medical history of chronic inflammatory demyelinating polyneuropathy diagnosed in 2000. Therefore, he was on immunosuppressive therapy with methylprednisolone 20 mg per day and azathioprine 200 mg per day.
Additionally, he was on medication with acetylsalicylic acid after a myocardial infarction in 2010 and antihypertensives. Physical examination showed a violaceous firm nodule with purulent drainage over the proximal phalanx of the left middle finger () and up to 20 reddish to violaceous firm nodules up to 4 × 2 cm in size on the right arm (). Additionally, there were extensive well-demarcated, erythematous macules with scaly borders on the chest. All finger- and toenails showed onychodystrophy and yellowish discoloration. Furthermore, physical examination revealed an enlarged lymph node in the left axilla. Abdominal ultrasound revealed a slight hepato- and splenomegaly. Biochemical examination showed an elevated white blood cell count (16.300 /μl) with relative lymphocytopenia, low hemoglobin (9.9 g/dl) with iron deficiency and elevated HbA1c (7.9%). Other routine laboratory tests were unremarkable. Screening for human immunodeficiency virus and tuberculosis was negative. Direct microscope examination by potassium hydroxide (KOH) preparation of scales from a lesion of the chest, of nail scrapings and of pyogenic fluid of a nodule showed each branched septate hyphae. Fungal culture of each of the above mentioned specimens revealed T. rubrum. Bacterial cultures were negative. A biopsy specimen of a nodule from the right forearm showed a dermal abscess with massive neutrophils in the center and macrophages in the border area (). The Periodic Acid Schiff (PAS) staining showed branched septate hyphae in the transition zone between granulomatous inflammation and abscess (). Fungal culture of the biopsy specimen also showed T. rubrum. Culture for non-tuberculous mycobacteria was negative. Furthermore, a biopsy specimen from the erythematous macula on the chest showed the histopathologic picture of tinea superficialis with branched septate hyphae in PAS staining. The patient was diagnosed as deeper dermatophytosis by T. rubrum presenting in form of multiple dermal abscesses. Treatment was started with oral itraconazole 200 mg per day and local application of ciclopirox twice a day. The patient did not show up for follow up and medication was only unsteadily taken. Four months later the patient presented again in our department with now multiple well-demarcated erythematous scaly plaques on the trunk and growing size of the abscesses on the right elbow. He had presented to an office-based doctor, where a drug eruption was assumed, why treatment with itraconazole was stopped. However clinical examination and biopsy specimen of the plaques revealed tinea superficialis. Nevertheless, a treatment change to griseofulvin 500 mg twice a day was conducted. Again, the patient took the medication only unsteadily which resulted in an improvement of the clinical outcome but no cure with nodules still present after 6 months. |
pmc-6513512-1 | Our patient was a 75-year-old Japanese woman. Two days prior to being examined at our hospital in January 2017, she became aware of mild bleeding due to gum injury caused by a maxillary denture fracture. After the initial bleeding, she experienced pain around the gingiva of the left maxillary second premolar (tooth No. 25) and left facial swelling, for which she was examined at the Emergency Department of our hospital. Acute odontogenic infection was considered, and she was referred to our department.
She had a history of aplastic anemia, renal dysfunction, bronchial asthma, and osteoporosis. She had no history of allergy, and she was currently taking orally administered cyclosporine, febuxostat, montelukast sodium, and theophylline. Epoetin beta pegol, a continuous erythropoietin receptor activator (CERA), was administered intravenously once a month.
On initial examination, she presented with marked swelling and tenderness from her left lower eyelid to her left cheek and to the left side of her upper lip. Spontaneous pain was present on the left temporal area. The stump of tooth No. 25 was intact, with swelling and tenderness around the gingiva. Pulsation was perceived upon palpating the buccal gingiva. A panoramic radiograph revealed radiolucency surrounding apical region of tooth No. 25. (Fig. ). A simple computed tomography (CT) image revealed extensive inflammation, with adipose tissue opacities in the soft tissues of her left cheek (Fig. a). Although no abnormal image was seen in either of the maxillary sinuses, there was radiolucency surrounding apical region of tooth No. 25 (Fig. b). Blood tests at the initial examination revealed a leukocyte count of 6700/μL, which was a normal value; however, her C-reactive protein (CRP) concentration was high at 7.15 mg/dL. Our patient’s platelet count was slightly lower at 97,000/μL; this was attributed to her history of aplastic anemia. The blood urea nitrogen concentration was 34.1 mg/dL; the serum creatinine concentration was 1.43 mg/dL, which is indicative of renal dysfunction (Table ).
The buccal gingiva corresponding to the apical region of tooth No. 25 revealed swelling; as pulsation was perceived upon palpating the region, the Hematology Medicine Department of our institution was consulted regarding incision and drainage of the pus. Although anemia was present, her leukocyte count was normal, so it was considered safe to proceed with the intervention. On the same day, a fine-needle aspiration was performed under local anesthesia, and the suctioned pus was submitted for a bacterial culture test. Incision and drainage of the pus were then performed, followed by the placement of a gauze drain. She was hospitalized immediately, and anti-inflammatory therapy with intravenously administered ampicillin/sulbactam was initiated. As she had renal dysfunction, the dose of the antibiotic was lower than normal; ampicillin/sulbactam 9 g/day was administered. The Hematology Medicine Department was currently treating our patient with cyclosporine for aplastic anemia; after consultation, continuation of the oral treatment was permitted as long as her leukocyte count remained stable. Blood tests performed on day 5 of hospitalization revealed a leukocyte count of 5200/μL and a CRP value of 12.33 mg/dL, with no marked changes; however, she exhibited a fever of approximately 38 °C, so two sets of blood cultures were performed. Reddening around the gingiva of tooth No. 25, widespread facial swelling, and tenderness had all improved, but there was no improvement in the left temporal headache. In addition, there was onset of pain in the left side of her neck on the same day. Since the neck pain was mild, her condition was monitored by follow-up observations. Blood tests performed on day 9 of hospitalization showed a procalcitonin (PCT) concentration of 0.2 ng/mL, which was negative. Her leukocyte count was 6500/μL, and the CRP concentration was 25.62 mg/dL, indicating marked worsening of inflammation. A chest radiograph and culture tests (blood, urine, sputum, and fecal cultures) were performed to assess any remote infections, and the infection control team was asked to perform an examination. The chest radiograph revealed no evidence of pneumonia or other infections, and all culture tests were negative. In addition, inflammation of her left cheek improved, so the worsening of the inflammatory values could not be considered a causal factor. Head, neck, and chest contrast CT images were obtained on day 10 of hospitalization to more accurately identify the source of infection. Calcification around the odontoid process of the second cervical vertebra was observed and based on the history of temporal and neck pain, CDS was suspected (Fig. a, b). She was immediately referred to the Orthopedic Surgery Department for consultation, and CDS was considered the most likely cause of her increased leukocyte count and CRP value. Orally administered acetaminophen 600 mg/day was initiated to provide symptomatic therapy. Blood tests on day 11 of hospitalization revealed a leukocyte count of 4700/μL and a CRP level of 12.82 mg/dL, which constituted marked improvements. On day 13 of hospitalization, the symptoms were in remission, and she was discharged. Tooth No. 25, which was considered the cause of the buccal swelling on the left side, was extracted on a subsequent out-patient visit (Fig. ). After tooth extraction, symptoms of neck pain and temporal pain improved, completing the orthopedic surgery intervention. |
Subsets and Splits
Exclude ER emergencies
Retrieves 100 descriptions that do not contain the terms 'ER' or 'emergency', providing a basic filter of the dataset.