_id stringlengths 7 16 | description stringlengths 55 95.2k |
|---|---|
pmc-6582472-1 | A 5-year-old girl with no past medical or surgical history had complained of waxing and waning pain around the umbilicus for a few years. On several occasions, she had visited her general practitioner due to abdominal pain, but no abnormalities had been noted. The pain recurred with greater severity, and abdominal ultrasound performed by her general practitioner revealed a cystic lesion in the right lower abdominal cavity. An abdominal abscess was suspected thus she was referred to a tertiary hospital.
On admission, the vital signs were as follows: blood pressure, 125/81 mmHg; pulse, 166 beats per minute; and temperature; 36.5 °C. On palpation, rebound tenderness was noted on the right iliac fossa. On laboratory investigation, the white blood cell count was 17,400/μL and the C-reactive protein level was 1.54 mg/dl. There was an elevation in the serum pancreatic amylase and lipase level (124 U/L, 114 U/L respectively). Contrast enhanced abdominopelvic computed tomography (CT) (Fig. ) revealed a cystic lesion measuring 6 × 5 cm with an enhancing thick wall in the ileal mesentery. The cystic lesion was tubular in shape and inflammation was suspected as the density of the adjacent fat was increased. A small enhancing nodule in the cyst wall was also noted. Retrospectively, a distinct blood vessel coursing on the dorsal side into the lesion was identified (Fig. ). The pancreas appeared within normal limits. The appendix was slightly enlarged and a diagnosis of appendicitis was equivocal.
An abscess in the mesentery was suspected, and the patient was put on nil by mouth and treated with antibiotics. There were significant improvements in the symptoms and the laboratory data on the second week and she was discharged on the fifth week. Follow-up enhanced CT at 3 months showed a significant decrease in size of the cystic mass (Fig. ). However, the abdominal pain persisted and the possibility of appendicitis with abscess formation was considered, hence laparoscopic appendectomy was performed. After appendectomy, the abdominal pain recurred and a slight elevation of the pancreatic enzymes persisted so a laparoscopy was repeated for possible resection of the mesenteric lesion. During laparoscopy, a cystic mass was found in the root of the iliac mesentery measuring approximately 2 × 1.5 cm in diameter. There was no communication between the mass and the nearby ileum. During the procedure, there was bleeding from the blood vessel coursing dorsal to the mass and hemostasis was required.
Pathologically, the wall of the lesion assimilated the small bowel wall with villi in the mucosa and under the mucosa there was pancreatic tissue consisted of acini, ducts and islets of Langerhans (Fig. ). The pancreatic duct appeared to open into the cyst. Therefore, a diagnosis of isolated enteric duplication cyst with Heinrich type I ectopic pancreas was made. Changes suggestive of post pancreatitis such as lymphocytes infiltration and acinar loss with islet-cell pseudohyperplasia were also seen in the pancreatic tissue. Postoperatively there were no complications and in the follow-up the serum pancreatic enzymes normalized. The patient reported no recurrence of abdominal pain since the resection of the lesion. |
pmc-6582513-1 | A man in his 40s looked for medical care at the local Emergency Department in a city 30 km from Rome, on 25 August and 31 August. He presented with several vesicular lesions on his right forearm and was initially treated with topical steroid therapy and parenteral ceftriaxone (1 g/day). Because his clinical condition deteriorated, he was referred to the Spallanzani Institute on 31 August, to a clinical unit different from that of Case 1.
At admission, he was in critical condition, with relevant bilateral oedema of the upper extremity up to the shoulders, associated with ulcerated and necrotising skin lesions covered by black eschars (). He revealed to be a farmworker but did not report any contact or exposure to sick animals. He had worked on a horse farm bordering the one where the anthrax epidemic had occurred.
Intravenous meropenem (1 g every 3 h), daptomycin (350 mg every 24 h) and clindamycin (600 mg every 6 h) were prescribed and his clinical condition improved promptly in the first 24 h.
Considering the similar clinical presentation of the two cases, the likely professional exposure and the geographical and suspected epidemiological link, a new clinical assessment was performed 24 h later and the patient finally recalled that he had been exposed to the blood of the dead cow inspected on 19 August by Case 1. The previous antibiotic therapy was stopped and he was successfully treated with intravenous ciprofloxacin (400 mg twice/day) and discharged after two weeks after hospitalisation. Oral ciprofloxacin (500 mg twice/day) was prescribed for a further 7 days.
Cultures of swabs from the blisters and ulcers of this patient were all negative, while the PCR for B. anthracis DNA from margin eschar fragments taken on 6 September was positive. Specific antibodies to B. anthracis were detected with a seroconversion during the convalescent phase at a titre dilution of 1:16 on Day 37 after exposure. |
pmc-6582524-1 | Our patient was a 31-year-old Japanese woman, gravida 1, para 1. She had no remarkable medical history, and there were no abnormalities during her medical examination. Her father and uncle had type 2 diabetes requiring insulin therapy. However, her sister did not have diabetes. The weight gain during her first 3 months of pregnancy was 1.1 kg. A regular examination at 29 weeks and 5 days was normal; her blood glucose level was 73 mg/dL, with negative levels of urinary glucose, and 3+ urinary ketone bodies.
At 30 weeks and 6 days, she was admitted with marked fatigue and dyspnea. However, she did not present with fever, headache, costochondritis, pharyngitis, diarrhea, maculopapular non-pruritic rash, nausea, or flu-like symptoms such as a sore throat, cough, or rhinorrhea.
On admission, she was alert. A physical examination revealed the following: temperature (T), 36.7 °C; pulse rate (P), 94 beats per minute (bpm); respiratory rate (R), 18/minute; and blood pressure (BP), 121/65 mmHg; fetal bradycardia was also observed, for which emergency cesarean section was performed, but the child was stillborn. The laboratory findings revealed diabetic ketoacidosis (DKA), with random sample glucose of 427 mg/dL, C-peptide reactivity (CPR) of 0.04 ng/mL, arterial pH of 6.91, bicarbonate of 6.7 mEq/L, and 3+ urinary ketone bodies. Despite the presence of DKA, the glycated hemoglobin (HbA1c) value was within the normal range (6.2%), and urinary CPR was extremely low at 0.5 μg/day. In addition, the serum elastase-1 and lipase levels were increased to 1939 (< 300) ng/dL and 119 (11–53) U/L, respectively. Antibody level against glutamic acid decarboxylase was 1.2 (< 1.5) U/mL. Neither anti-insulin nor anti-insulinoma-associated antigen-2 antibodies were detected. HLA II haplotypes showed DR8,12/DQ7,4. These results were indicative of a FT1D diagnosis (Table ).
She was diagnosed as having DKA due to FT1D. She was simultaneously treated with fluid replacement and continuous insulin infusion to maintain vital signs, plasma glucose, and electrolyte levels. On day 2, ketonuria, electrolytes, and vital signs had normalized. On day 3, the continuous infusion of insulin was withdrawn, and daily multiple insulin injections were administered with blood glucose monitoring. On day 24, she continued to undergo treatment with multiple daily insulin injection therapy with insulin aspart (25 U/day) and insulin degludec (12 U/day).
To investigate the association between viral infection and FT1D, we performed serological testing for several viruses such as parainfluenza virus 1–3, Coxsackievirus A2–7, 9, 10, 16, B1–6, cytomegalovirus (CMV), Epstein–Barr virus (EBV), and human herpes virus (HHV) 6 at day 3, day 17, and day 38 (Table ). A Coxsackievirus B1 antibody titer was significantly elevated from 1:32 to 1:256 (eightfold increase), whereas the other antibodies were not altered. |
pmc-6582561-1 | A 27-year-old male hypertensive patient was referred to our department on Nov 15, 2017, after right nephrectomy as well as left minimally invasive percutaneous nephrolithotomy (mini-PCNL). With a history of symptomatic kidney stones for more than 10 years and elevated SCr level for more than 3 years, accompanied with hypertension (peak 170/98 mmHg), the patient was scheduled to receive the renal transplantation.
The patient was rehospitalized on Apr 28, 2018, with SCr 1487 umol/L and BUN 33.47 mmol/L (shown in Additional file : Figure S1). He underwent renal transplantation that night, with a deceased donor, which was performed with Zero-Hour Implantation biopsy (ZHIB, as part of the routine renal transplant procedure, shown in Fig. a, 200X HE). The patient received standard immunosuppression with mycophenolate mofetil (MMF), tacrolimus (Tac) and methylprednisolone (Methylpred) besides hemodialysis (HD), as well as hypertension treatment. Additionally, he received the follow-up assessments including routine blood tests, blood biochemical analysis and therapeutic drug monitoring regularly (Additional file : Figure S1). However, it was less likely to have improvements in the renal graft function.
Due to the delayed graft function (DGF, SCr 585 umol/L and BUN 30.07 mmol/L), the patient received percutaneous needle core biopsy of renal graft on post-operative (post-op) day 46, guided by ultrasonography (USG) (Fig. b). Renal graft biopsy revealed acute T cell-mediated rejection (TCMR, 2015 Banff 1A) and extensive tubular damage, with the presence of the emerging deposition of CaOx crystals in the allograft (Fig. c, 200X HE and polarized light). Compared with ZHIB, recurrence of CaOx nephropathy was confirmed in the allograft kidney. Molecular genetic testing of the patient was carried out to identify the pathogenic variants (PV) in AGXT and GRHPR, in whom a homozygous genotype for three missense mutations in the GRHPR gene was found: first, a C to T transversion (c.295C > T) in exon 4 resulting in p.(Arg99*), nonsense PV; second, a G to A transversion (c.512G > A) in exon 6 resulting in p.(Arg171His), missense variant of uncertain significance (VUS); third, a G to A transversion (c.211G > A) in exon 2 resulting in p.(Ala71Thr), missense VUS (shown in Table and Fig. d). Based on the previous findings a diagnosis of PH2 was made.
We further analyzed the DNA of the patient’s family. Sequencing analysis of the GRHPR gene showed that the PV (c.295C > T) was also found in the patient’s mother, who presented asymptomatic kidney stones. Unfortunately, the other two VUS were detected both in the patient’s father and son (shown in Table and Fig. d).
The patient was treated with Pyridoxine (PN, Vitamin B6) and temporarily intensive HD besides the basic immunosuppression to suppress oxalate overproduction. Furthermore, for the failure of kidney-alone transplantation (KAT), we recommended the treatment scheme with the combined Liver-Kidney Transplantation (LKT) before the development of systemic oxalosis but was refused by the patient. The patient currently reentered to the maintenance HD in the clinic and looked forward to a chance of LKT in the future. |
pmc-6582561-2 | A 26-year-old male non-hypertensive patient was hospitalized on Aug 11, 2016, with an 8-year history of the elevated SCr, which included a 7-year history of maintenance HD. The next day, attributed to the preoperative SCr 893 umol/L and BUN 27.32 mmol/L, shown in Additional file : Figure S2), he was transplanted with a deceased kidney donor performed with routine ZHIB (Fig. a, 200X HE). The patient received standard triple immunosuppression following transplantation as well as the follow-up assessments regularly. Similarly, even though we substituted Cyclosporine A (CsA) for Tac, renal graft gradually developed DGF (SCr 534 umol/L and BUN 30.06 mmol/L, Additional file : Figure S2), and USG-guided renal biopsy was employed on post-op day 38 (Fig. b). Deposition of diffuse CaOx crystals as well as acute TCMR (2015 Banff 2A) was detected in renal graft biopsy (Fig. c, 200X HE and polarized light), whereas there were no oxalate crystals in the ZHIB. Molecular genetic testing identified two mutations in the AGXT gene: first, exon 1: c.33dupC (p. Lys12fs), frameshift PV; second, an A to T transversion (c.215A > T) in exon 2 resulting in p. Asn72Ile, missense VUS. Meanwhile, it was noteworthy that one mutation in the MUT gene (Exon11: c.1897G > C (p. Val633Leu), missense VUS) was detected in the patient. Thus, the patient was diagnosed with PH1 and treated with PN (400 mg, iv, QD) and temporary intensive HD.
Owing to the anemia (hemoglobin level, HGB, 57–74 g/L; shown in Additional file : Figure S2), bone marrow biopsy was conducted on post-op day 70, which showed intertrabecular spaces occupied by abundant CaOx crystals (Fig. d, 200X HE, 400X HE and polarized light). Apparently, the patient was diagnosed as systemic oxalosis of PH1 with CaOx crystals involving both the allograft kidney and bone marrow, the latter is the most crippling site of CaOx deposition. He was treated with concentrated red blood cells (CRBC) transfusion and recombinant human erythropoietin (rhEPO) to correct the anemia. Moreover, as renal graft loss, he returned to the maintenance HD and received the optimal immunosuppression. Up till now, the patient was investigated for the overall decline in health following allograft failure. |
pmc-6582561-3 | A 34-year-old male hypertensive patient was admitted to hospital on Oct 16, 2015, for the first time because of the elevated SCr level for more than 12 months. Presented with SCr 1222 umol/L and BUN 24.33 mmol/L (Additional file : Figure S3) as well as hypertension peaked at 180/100 mmHg, the patient waited for the renal transplantation.
The patient was readmitted to hospital on Jan 5, 2016. He received a kidney allograft on the next day with a deceased donor performed with routine ZHIB (Fig. a), followed by post-op standard triple immunosuppression besides HD. Furthermore, the follow-up assessments were executed nearly once a day (Additional file : Figure S3). However, the course was unfavorable with DGF emerging. USG-guided renal graft biopsy (Fig. b) was performed on post-op day 75, which documented acute TCMR (2015 Banff 2A) and extensive deposits of CaOx crystals in the interstitial tubule (Fig. c, 200X HE and polarized light). In view of the fact that no oxalate crystals deposited in the ZHIB, as well as the history of kidney stones in the recipient, recurrence of CaOx nephropathy following kidney transplantation was confirmed, which led to the diagnosis of PH. However, the diagnosis of PH must depend on the genetic testing. Unfortunately, molecular genetic testing of the patient was not carried out to identify the PV in AGXT, GRHPR or HOGA1 for some reasons.
Although the renal function of the patient was slowly repaired (SCr 260 umol/L; BUN 14 mmol/L), the treatment by HD was ineffective in treating excess oxalate besides the drug immunosuppression. Accompanied by the fever of 39.8 °C (Body temperature shown in Additional file : Figure S3) and intermittent cough, Pneumocystis jirovecii pneumonia (PJP) was confirmed in the patient on May 25 by fiberoptic bronchoscopic (FOB) biopsy (Fig. d, 600X HE and Methylamine silver) as well as subsequent Chest CT examination (Fig. e). The patient was initially treated with empirical anti-infection therapy (Micafungin) as well as γ-globulin. Meanwhile, standard triple immunosuppression was replaced with the pulse intravenous Methylpred. Even though the clinicians enhanced the anti-infection therapy with administration of trimethoprim-sulfamethoxazole (TMP/SMX) and voriconazole (VOR) orally, as well as meropenem (MEM) was used in the final stage, the patient died of severe pneumonia caused by P. jirovecii on Jun 28, 2016. |
pmc-6582589-1 | We hereby present the rare case of a 29-year-old white woman married for 5 years gravida 1 para 1 without significant personal or family history who found during breast self-examination a left breast mass. Then she went to a high medical center where she received breast ultrasound and a mammogram that revealed the presence of a left breast cancer classified 5 in the Breast Imaging Reporting And Data System of the American College Of Radiology, that is to say highly suggestive of malignancy (more than 95%). The radiological report noticed a mammary nodule at the level of the supero-external quadrant of the left breast of 2 cm long axis with the presence of two homolateral axillary lymphadenopathies of 1.2 cm and 0.8 cm. Then she was referred to us and admitted to the National Institute of Oncology in Rabat. The clinical examination confirmed the presence of a mobile mammary mass at the level of the supero-external quadrant of the left breast of 2 cm long, without inflammatory or cutaneous signs, nor mammalian flow, with just one mobile axillary homolateral suspicious ganglion of 1 cm.
First, we performed a micro biopsy with pistol to confirm histologically the presence of the cancer which turned out to be a non-specific infiltrating carcinoma grade 3 (differentiation 3, anisonucleosis 3 and mitotic index 3) of the Elston-Ellis modified Scarff-Bloom and Richardson staging with no intraductal component nor intravascular tumor emboli. She then had a thoraco-abdominopelvic computed tomography as part of her extension assessment, which was negative. Taking into consideration all these elements, we were able to classify the tumor cT1N1M0. We therefore decided to offer conservative treatment to the patient as soon as possible given the diagnosis of cancer at a relatively early stage, which she accepted.
Three weeks later, the patient underwent lumpectomy with ipsilateral axillary dissection. The one-month follow-up showed that the wound had healed well. There was no significant complication in the short term. The histopathology report confirmed the presence of infiltrating ductal carcinoma (WHO 2003) measuring 2.2 cm long axis, grade 3 (differentiation 3, anisonucleosis 2 and mitotic index 2) of the Elston-Ellis modified Scarff-Bloom and Richardson staging, with no intraductal component nor intravascular tumor emboli, with healthy exeresis limits: non-tumorous deep plane at 0.8 cm, healthy lateral margins closest to 0.2 cm; and at the level of axillary dissection 12 N(−)/12 N. The pathological staging of the tumor was therefore pT2N0M0. On the other hand, the histological examination of the axillary dissection revealed the presence of several epitheloid and gigantocellular granulomas centred by a caseous necrosis, typical of follicular ganglionic tuberculosis. The immunohistochemical profile of the tumor could be elaborated and revealed a strong expression of hormone receptors (oestrogen and progesterone), a 30% ki67 without overexpression of HER 2. According to the genomic classification of breast cancer, we could therefore classify this tumor as a luminal tumor A [].
Her file was discussed in a multidisciplinary consultation meeting during which several decisions were made. First, TB case was notified and Directly Observed Treatment Short course was given.
She received her anti-tuberculosis treatment with a course of 6 months according to the latest WHO recommendations of 2HRZE/4HR (2 months of the Isoniazid, Rifampin, Pyrazinamide and Ethambutol association followed by 4 months of Isoniazid and ethambutol biotherapy) []. Three weeks after the beginning of her anti-tuberculosis treatment, she began her adjuvant chemotherapy to prevent locoregional or general recurrence. This treatment was based on 3 courses of four-weekly FEC 100 (5-fluorouracil, Epirubicin, Cyclophosphamide) followed by 3 courses of four-weekly docetaxel. At the end of the chemotherapy she had an external radiotherapy by irradiation of the breast remaining after lumpectomy of 50 Gy with a complement of irradiation on the tumor bed of 15 Gy. Moreover, she also received an anti-estrogen hormone therapy (tamoxifen). Trastuzumab has not been proposed since the tumor does not overexpress HER 2 (human epitheloid growth factor receptor). Finally, given her young age, she had an onco-genetic consultation with search of genes BRCA-1 and BRCA-2, negative income for both.
Despite difficulties of therapeutic compliance, after 2 years the patient completed her cancer treatment and she cured of her tuberculosis. Being in remission, she is still on hormone therapy and consults every 3-months as part of her follow-up. |
pmc-6582828-1 | A 17-year-old adolescent nulligravid girl whose last menstrual period was four days back referred with the diagnosis of low lying myoma after being transfused with four units of blood. She presented to the Gynecology Outpatient Department on 22 July 2018, with history of irregular vaginal bleeding of 2-year duration.
She gives history of lower abdominal swelling which was small initially and progressively enlarged to attain the current size for the last 13 months. She is sexually active but not married. She has no history of abdominal pain, urinary, or bowel complaint, has no bleeding from other sites, and has no significant past medical and surgical history noted.
On examination, patient was conscious, coherent with blood pressure of 100/60 mmHg, pulse 80 /min, and temperature normal and has pale conjunctiva, cardiovascular, and respiratory systems normal. Abdominal examination revealed 20-week sized nontender, firm, irregular, fixed, and lower border unreachable mass. On vaginal examination, a firm huge nontender growth attached to the anterior vaginal wall. It was difficult to reach at the cervix. A clinical diagnosis of cervical myoma was made.
Her blood investigations on arrival showed hemoglobin of 9.7 gm/dL and were transfused one unite blood and the hemoglobin elevated to 10.7 gm/dL. Ultrasonography showed empty uterus with normal size echotexture and pushed up in to the abdomen. There is 10 by 12 cm hypoechoic mass arising from the cervix filling the vaginal canal with these the diagnosis of cervical myoma was made. The diagnosis necessitated Computed Tomography (CT) scan of the abdomen and pelvis which helped to know the extent of the mass and reported hypodense contrast enhancing mass seen on the uterus arising from the cervical region measures about 10.3 cm by 14.4 cm. The mass grows down in to the vagina and concluded with the diagnosis of contrast enhancing cervical mass likely myoma ().
Understanding the complexity of her surgery and possible postoperative morbidity and mortality, surgical, anesthesiology, nursing, and recovery room teams was assembled with gynecologic oncology team. She was prepared for elective laparotomy the day before the surgery. On 18 August 2018, the patient was taken to operating room, after the general anesthesia given; she was positioned in a supine position. The abdomen cleaned with povidone iodine and draped with sterile towels and midline vertical incision was made. Intraoperatively, bilateral fallopian tubes and ovaries, the uterus, and urinary bladder grossly look normal; there is 10 cm by 15 cm sized firm mass between the vagina and the lower uterine segment (). Vesicouterine peritoneum incised the bladder reflected away from the lower uterine segment and upper vaginal wall, about 4 cm vertical incision was made on proximal anterior vaginal wall, and dissection of the mass away from the anterior vaginal wall was tried but it was difficult to have a clear cleavage line to excise the whole mass. Then another 6 cm longitudinal incision was made on the posterior vaginal wall; sharp and blunt dissection were made to separate the bulk of mass away from the vaginal wall; finally excision of the mass from the base within 1 cm of normal vaginal tissue was performed to carefully remove a 15 × 10 × 7 cm encapsulated mass (). The anterior vaginal wall, extension of incision on the cervix, and posterior vaginal wall closed separately with vicryl number 0 in two layers (). Hemostasis was secured, correct count was reported, the fascia and skin were closed using delayed absorbable stitch. The surgery was completed after 2hrs and 20 minutes.
The excised mass was sent for histopathological examination; the patient recovered completely and discharged on 6th postoperative day. The final pathological diagnosis of the mass was vaginal angiofibroma (). |
pmc-6582834-1 | A 42-year-old multiparous woman presented with episodic, severe right upper quadrant pain associated with nausea and vomiting. Her past surgical history included a hysterectomy and left oophorectomy for unclear reasons. Several months prior she presented to another hospital for similar symptoms and was diagnosed with a hepatic mass. Physical examination demonstrated right upper quadrant tenderness without any palpable masses. Liver function and viral serologies for hepatitis B and C were normal. Tumor markers demonstrated normal CA 19-9 and AFP, with mildly elevated CA-125 40 U/mL (normal <38U/mL).
Computed tomography with intravenous contrast showed a 3.2cm x 4cm x 1.8cm multiseptated cystic lesion in the left hepatic lobe and an ill-defined heterogeneous hyperdensity within the peripheral right hepatic lobe measuring 3cm x 1.3cm (). Ultrasound-guided fine needle aspiration and core biopsy of the left hepatic lesion were inconclusive.
Further workup revealed a positive Echinococcal IgG antibody and she was started on Albendazole for a presumed hydatid cyst. After completion of therapy, she was scheduled for complete left lateral hepatic resection. However, she presented again several weeks later with progressive right upper quadrant pain. At this time repeat computed tomography redemonstrated the left hepatic mass which was unchanged in size but did not show the right hepatic lesion. Imaging also revealed a new pericardial effusion that was not present on previous imaging (). Her liver tests were the following: AST 485 U/L (normal 10-40 U/L), ALT 308 U/L (normal 5-40 U/L), ALP 50 U/L (normal 35-104 U/L), and total bilirubin 0.5 mg/dL (normal <1.0 mg/dL). Given the concern for pericardial involvement, she urgently underwent a laparoscopic left partial hepatectomy (segment II and partial segment III). The postoperative course was uneventful. Final pathology was consistent with hepatic endometriosis (Figures and ). After 2 months of follow-up, the patient was asymptomatic and liver tests normalized. She was started on medroxyprogesterone acetate and remains well to date. |
pmc-6582838-1 | A 71-year-old male patient visited another hospital due to abdominal pain 2 years and 7 months before visiting our hospital. He also had swelling of the lymph nodes in the neck and both inguinal regions. He had been diagnosed with peritoneal fibrosis, periaortitis, and bilateral parotitis based on a computed tomography (CT) scan () and physical examination findings. The serum level of IgG4 was 3260 mg/dl, and biopsy of the parotid gland showed infiltration of IgG4-positive plasma cells (). He was diagnosed with IgG4-RD and was treated with prednisolone (PSL) 20 mg/day. He soon stopped taking PSL by his own judgment because he completely recovered from abdominal pain. He did not visit any hospital for more than 2 years.
He then visited our hospital due to abdominal pain since the last 2 months. A CT scan showed a 44 mm diameter AAA with thickened adventitia (). It was diagnosed as IAAA. The CT scan also showed thickening of tissue around the ureters (), internal iliac arteries (), and femoral arteries (). The lymph nodes were also swollen (). A blood test showed high serum levels of IgG (4225 mg/dl) and IgG4 (1890 mg/dl) (). IAAA was larger than before, but rupture or impending rupture was not detected in the CT scan image. Recurrence or aggravation of IgG4-RD was strongly suggested. The frequency of abdominal pain decreased after hospitalization, and we observed him with antihypertensive medicine treatment. On day 6, however, he had severe pain from the neck to the abdomen, and his systolic blood pressure rose to over 200 mmHg. Simultaneously, cyanosis and strong pain appeared in both lower limbs, and, thereafter, muscular strength of both lower limbs weakened. The manual muscle testing result of the iliopsoas muscle, quadriceps femoris, and anterior tibialis muscle was grade 0. He also had urinary retention. Based on magnetic resonance imaging (MRI) findings (), the neurologist concluded that he had paraplegia from the 11th thoracic spinal cord level due to anterior spinal artery syndrome. The CT scan did not show aortic rupture. Soon after this episode, PSL 20 mg/day was restarted.
Even after restarting PSL treatment, he had abdominal pain several times and we could not completely exclude the possibility of the IAAA impending rupture. We performed endovascular stent grafting for the IAAA on day 13 after hospitalization. Under general anaesthesia, the bilateral inguinal portion was incised. The main body of the Excluder® (Japan GORE, Tokyo, Japan) was inserted and deployed through the left common femoral artery. Through the right common femoral artery, two stent graft limbs were inserted and deployed. Both internal iliac arteries were kept patent. Macroscopically, the adventitia of the right femoral artery was thickened. Microscopically, the intima and media showed atherosclerotic changes, without immunostaining of IgG4.
On day 6 after stent grafting, he had strong pain in both lower limbs and systemic cyanosis. His systolic blood pressure increased to over 200 mmHg. PSL dose was increased to 50 mg/day, and intravenous treatment of prostaglandin E1 was started.
On day 7 after stent grafting, he had bloody stools. Upper and lower intestinal endoscopy showed atrophic gastric mucosa, duodenal erosion, and ulceration and erosion from the sigmoid colon to the rectum. He was diagnosed with ischemic colitis. On day 34, he had strong pain in the abdomen and both lower limbs. A CT scan showed free air in the abdomen and emergent abdominal operation was performed. Macroscopically, he showed ischemic changes from the ileum to the upper rectum and perforation of the sigmoid colon. The intestine and peritoneum were strongly adhesive, and we could not observe some IgG4-RD related organs, such as pancreas and biliary tract. The lymph nodes were not swollen in the mesentery. The wide portion from the ileum to the rectum was resected and colostomy was performed. The pathology of the intestine showed cytomegalovirus colitis and ganciclovir was soon started. He was transferred for rehabilitation to another hospital on day 124 after admission and on day 111 after stent grafting.
About one month later, he was transferred to our hospital again with acute myocardial infarction. Aspiration of the thrombus and stenting for the occluded left descending artery were performed (). Three months later, he came to our hospital again with intestinal haemorrhagic shock. He died of massive bleeding from colostomy. Autopsy revealed wide range of intestinal erosion with ischemia (). IgG4 staining and cytomegalovirus test results of the intestine were negative. |
pmc-6582852-1 | A 27-year-old male with keratoconus presented at the Cornea and Anterior Segment Department with blurred vision that was difficult to correct with eyeglasses. In his right eye, his UCVA was 20/100 and his manifest refraction was -0.75-1.25 x 50° with BCVA of 20/30. In his left eye, his UCVA was 20/25 with -0.25-0.50 x 75° and BCVA of 20/20 (). ICRS was implanted in his right eye and crosslinking in his left eye.
The ICRS (KeraRing, Mediphacos, Belo Horizonte, Brazil) used in this study was a 160° segment ring made of polymethyl methacrylate (PMMA) implanted in the corneal tunnel (). Both ICRS nomograms, Ferrara and KeraRing, indicated different ring position (Figures -), so a temporal 160°/200-micron ring was utilized. A femtosecond laser (Z6; Ziemer Ophthalmic Systems AG, Port, Switzerland) was used to make an incision at 114° (the steepest meridian), K1 42.3 D, and K2 47.4 D, and a 371um deep corneal tunnel was created (corresponding to 75% of the corneal thickness of 495 um at the thinnest point of the tunnel path), with a 355-degree length only 20 degrees more than the normal size of the ICRS. This longer channel allowed repositioning of the ring if necessary.
The thickness and degree of arc of the ICRS were selected and their location was planned according to the cone location on axial topography measured via Scheimpflug imaging (Pentacam; Oculus GmBH, Wetzlar, Germany), with a 5.5 mm diameter depending on the nomogram results.
Postoperative treatment included 0.3% topical tobramycin with 0.1% dexamethasone (Tobradex; Alcon, Fort Worth, TX, USA) four times a day for 2 weeks; the latter was then tapered over 4 weeks. Preservative-free artificial tear substitute (Lagricel Ofteno; Sophia, Guadalajara, Mexico) was used four times a day for 2 weeks.
Two months later the patient's UCVA was 20/100, his BCVA was 20/30, and manifest refraction was +1.50-1.75 x 10°, indicating a refractive failure. However, his RMS improved from 14.750 um to 10.346 um ().
According to the postoperative keratometric Scheimpflug map, the ring was moved 50 degrees counterclockwise according to the topographic results (). One-month after repositioning, the patient's UCVA was 20/25, his BCVA was 20/20, his manifest refraction was -0.50 x 90°, and his RMS had decreased to 5.392 um.
At a one-year follow-up, his right eye was UCVA 20/30-2, his manifest refraction was +1.00-2.00 x 90 degrees with BCVA of 20/20, and his RMS was 5.605 um (). |
pmc-6582852-2 | A 26-year-old male presented at the Cornea and Anterior Segment Department with low vision in the right eye, which presented UCVA of 20/100 BCVA 20/30 and manifest refraction of -0.75-3.5 x 50°. His left eye had UCVA of 20/25, BCVA of 20/20, and manifest refraction of -0.25-0.50 x 75°. Keratoconus was diagnosed in both eyes; femto ICRS was indicated for the right eye and follow-up for the left eye ().
The two nomograms proposed, KeraRing and Ferrara, indicated different ring positions (Figures -), so a temporal 160°/150-micron ring () was utilized. A femtosecond laser (Z6; Ziemer Ophthalmic Systems AG, Port, Switzerland) was used to make an incision at 99.7° (the steepest meridian by topography), K1 42.5 D, and K2 46.1 D. Due to a pachymetry of 512 microns at the tunnel zone, a 371 um deep corneal tunnel (corresponding to 75% of the corneal thickness) was created with a 355-degree tunnel if repositioning was necessary.
Postoperative treatment included 0.3% topical tobramycin with 0.1% dexamethasone (Tobradex; Alcon, Fort Worth, TX, USA) four times a day for 2 weeks; the latter was then tapered over 4 weeks. Preservative-free artificial tear substitute (Lagricel Ofteno; Sophia, Guadalajara, Mexico) was used four times a day for 2 weeks.
Two months later, the patient's UCVA was 20/150, his BCVA was 20/40, and his manifest refraction was -1.00-2.00 x 50°, indicating a loss of one line of vision (). Additionally, his RMS decreased from 13.970 um to 11.327 um. The patient's outcome was not as planned, and his visual acuity was worse after the ICRS implantation. Therefore, the ring was moved 45 degrees counterclockwise in the tunnel according to the keratometric Scheimpflug map ().
One month after repositioning, the patient's UCVA improved to 20/40, his BCVA to 20/20, and his manifest refraction to +0.50-2.00 x 90°. His RMS decreased from 11.327 um to 8.275um.
At a one-year follow-up, his right eye was UCVA 20/40-2, his manifest refraction was +0.50-2.00 x 90 with BCVA of 20/25+2, and his RMS was 7.569 um (). |
pmc-6582856-1 | A 72-year-old female was admitted to the intensive care unit for AKI and severe shock. Her medical history included type 2 diabetes mellitus, Alzheimer's disease, hypertension, dyslipidemia, gastroesophageal reflux disease, and obstructive sleep apnea. The patient had no history of underlying chronic kidney disease.
During the three-day period before admission to the hospital, the patient was feeling unwell and increasingly somnolent, had significantly decreased oral intake, and was vomiting. She denied any fever, night sweats, or sick contacts. There was no history of diarrhea. Her medications included valsartan, metoprolol, rosuvastatin, aspirin, canagliflozin, sitagliptin, metformin, insulin degludec and aspart, donepezil, citalopram, gabapentin, and pantoprazole. Canagliflozin 300mg prescribed once daily was initiated approximately 18 months prior to presentation and was added to the antihyperglycemic agents that are listed. Otherwise, her medications were not changed during the 18 months prior to her presentation to the emergency room. She was not using herbal products or any other over-the-counter medications and did not ingest alcohol.
At presentation, the patient was somnolent, responding only to painful stimuli. Vital signs at presentation were the following: blood pressure 97/36 mmHg, heart rate 76 beats/min, respiratory rate 28 breaths/min, temperature 37.2°C, and SaO2 97% on nasal prongs. Physical examination was otherwise unremarkable. A Foley catheter was inserted which revealed minimal urine output. A point-of-care venous blood gas showed the following results: pH 7.00 (normal 7.35-7.45), pCO2 29 (normal 37-43 mmHg), bicarbonate 7 (normal 22-26 mmol/L), lactate 11.9 (normal 0.5-2.5 mmol/L), sodium 122 (normal 134-144 mmol/L), potassium 7.4 (normal: 3.5-5.5 mmol/L), and anion gap 48 mmol/L. There was an absence of ketones in the urinary dipstick. Laboratory evaluation revealed markedly elevated creatinine level at 1154 (normal: 45-95 μmol/L). A complete blood count was unremarkable except for an elevated white blood cell count with a result of 24.5 x 109/L (normal: 4-11x109/L). Blood cultures did not result in any growth obtained at the time of presentation. A CT scan of the abdomen and pelvis did not show any evidence of hydronephrosis or hydroureter. Urine microscopy showed many granular casts. In the absence of other causes for her clinical presentation, the patient was diagnosed with AKI secondary to ischemic ATN, with canagliflozin use likely an important contributing factor in the presence of a possible viral infection that led to the nausea and vomiting.
She was immediately started on renal replacement therapy. She initially received continuous veno-venous hemofiltration (CVVH) and was subsequently converted to intermittent hemodialysis (IHD) when she became hemodynamically stable. The patient was anuric when the CVVH was initiated; however during the third week of her admission, the patient was noted to have a small quantity of urine output. Her renal function recovered gradually after 8 weeks of IHD (). |
pmc-6582860-1 | A 19-year-old female with no significant past medical history was referred to the emergency department from her antenatal clinic for further evaluation of fever 102 F, headache, malaise, nausea, and vomiting of 4-days duration following mild sore throat. She was in her 28th week of gestation on presentation. Physical examination showed an obese and anxious-appearing lady in mild respiratory distress with gravid abdomen. She weighed 142 kilograms (kg) with a BMI of 44.3. Her blood pressure was 111/60 mmHg, respiratory rate 24, heart rate 111, temperature 101.8 F, and oxygen saturation 96% on 2 liters (L) nasal cannula oxygen. She had dry mucous membranes, with no oral thrush or ulcers. Her abdomen was gravid with focal areas of tenderness, bowel sounds were heard, and fetal movement was detected. Pulmonary and cardiovascular examinations were unremarkable.
Her initial laboratory tests were significant for white blood cell (WBC) count of 11.8 K/uL with 78% neutrophil predominance and lymphopenia (0.77 K/uL). Although she had no urinary symptoms, urinalysis showed bacteriuria, confirmed as group B streptococcus agalactiae (GBS) with cultures. Initial chest X-ray and liver enzymes on admission were unremarkable. She was started on intravenous fluids for suspected gastroenteritis and oral cephalexin for asymptomatic GBS infection. Stool studies for clostridium difficile, comprehensive panel, ova, and parasites were ordered which all returned negative. However, she continued to spike fever through the third day of hospitalization prompting expansion of antibiotic coverage to Vancomycin and Zosyn (). Liver transaminases which had been normal began to spike. Aspartate aminotransferase (AST) was 190, alanine aminotransferase (AST) was 135, and alkaline phosphatase was 110 while bilirubin remained normal. Serum Tylenol and hepatitis A, B, and C serologies were negative. Infectious Disease (ID) consultation was requested who recommended further infectious work-up with CMV, EBV, HIV, HSV, Rickettsia, and Leptospira serologies.
The patient continued to spike high grade fever reaching 103 F on day 4 of admission despite being on broad-spectrum antibiotics and negative infectious work-up including the aforementioned serologies. She also developed bloody diarrhea and acute kidney injury (AKI) prompting evaluation for possible Hemolytic Uremic Syndrome (HUS) and autoimmune diseases which all returned negative as well. Right upper quadrant ultrasound showed normal liver and gallbladder morphologies. Magnetic resonance imaging of the abdomen and pelvis without contrast showed diffuse T2 hyperintensity throughout the liver compatible with acute liver injury but was negative for any focal intra-abdominal or pelvic abscess.
One week into her hospitalization, her AST and ALT kept trending up reaching a peak of 1712 U/L and 845 U/L, respectively (), mildly elevated alkaline phosphatase 140, INR 1.6, normal total bilirubin, absolute lymphocyte count of 0.11 K/uL, persistently negative blood cultures, and serum creatinine increased to 2.9 mg/dL, all concerning for a disseminated process. She became increasingly lethargic, tachycardic, and tachypneic necessitating care in the intensive care unit. With uprising liver enzymes and negative extensive infectious and rheumatologic work-up including Hep A, B, C serologies and PCR, EBV, CMV, Parvovirus, Rickettsia, and Leptospiral serologies, as well as negative HSV 1 and 2 IgM and IgG antibodies, antinuclear, and anti-liver-kidney microsomal antibodies, normal cryoglobulins, ceruloplasmin, alpha 1 antitrypsin and serum copper levels, and largely unremarkable imaging and stool studies, a decision was made to proceed with a liver biopsy. At this time, empiric acyclovir was started while other antibiotics were discontinued per ID recommendations.
Within the first 24 hours of starting acyclovir, AST and ALT began to downtrend while fever and diarrhea improved (). Despite negative HSV-2 serologies, the PCR was reported on hospital day-8 with a very high viral load >100 million DNA copies/ml following which she reported a remote history of cold sores but denied a history of genital ulcers. A diagnosis of HSV hepatitis was made and she was transitioned to Val-acyclovir 1 gram three times daily to complete 14 days of therapy, then to resume acyclovir 400 mg three times daily until delivery to prevent recurrence. On the 12th day of hospitalization, she was discharged home without needing a biopsy anymore and with complete resolution of symptoms and improvement of liver enzymes and AKI; AST 72, ALT 208, alkaline phosphatase 150, total bilirubin 0.5 and creatinine 0.5. She remained symptom-free with complete normalization of creatinine and liver enzymes at outpatient follow-up two weeks after discharge. She was eventually delivered of a healthy baby boy via cesarean section at 38 weeks of gestation with no peripartum complications. |
pmc-6582862-1 | A 32-year-old Caucasian female, G3 P1011 with a singleton intrauterine pregnancy at 23 5/7 weeks of gestation presented with acute onset of fever to 39.5 degrees Celsius, fatigue, and frontal headache during the summer. Remote medical history was significant for a benign pancreatic mass abutting the spleen that was treated surgically with removal of mass along with partial pancreatectomy and total splenectomy. She lived with her partner in a wooded area of New York State and reported daily trips to the beach. She denied any rashes, insect, or tick bites, but her husband recalled seeing a tick on his leg a few days prior to her presentation. She denied rash, myalgias, arthralgia, gastrointestinal symptoms or anorexia, sore throat, dry cough, neck stiffness, abdominal pain, dark urine, conjunctival injection, shortness of breath, or hyperesthesia. Prenatal care had been otherwise uncomplicated.
On initial laboratory evaluation, white blood cell count was 7.9 [K/uL], hemoglobin 12.5 [mg/dL], platelet count was 219 [K/uL], alanine aminotransferase (ALT) 77 [U/L], and aspartate aminotransferase (AST) 84 [U/L]. Peripheral blood smear revealed red blood cell intracellular parasites morphologically consistent with babesia species. Laboratory test results were consistent with likely acute infection by babesia serologically, with positive tests for IgM antibodies and IgG antibodies (each at a titer of > 1:1024 by immunofluorescence), and real-time polymerase chain reaction (PCR) was positive for Babesia microti (Mayo Clinic Laboratories, Rochester, MN). The DNA target for the PCR assay used by this laboratory is a gene encoding the nuclear small subunit ribosomal RNA (SS-rDNA) specific to babesia species. Initial parasite density was 1%, consistent with mild disease. Serum serology was negative for B. burgdorferi (Lyme disease), and Lyme IgG/IgM antibody index was 0.08 [0.01-0.89 index]. Maternal serum IgG/IgM antibodies for both Anaplasma phagocytophilum and Ehrlichia chaffeensis were <1:64 [reference <1:64 titers] and <1:20 [reference <1:20 titers], respectively, (Mayo Clinic Laboratories, Rochester, MN). Treatment was initiated with oral atovaquone 750 mg twice a day and oral azithromycin 500 mg once, followed by 250 mg daily. Fetal assessment was normal and weight was appropriate for gestational age. Umbilical artery and middle cerebral artery Doppler studies were normal (S/D 2.3 and MCA 1.38 MoM, respectively).
Parasite density was monitored daily and initially increased to 6% over the first three days of therapy. Antibiotic regimen was transitioned to quinine 648 mg every eight hours and oral clindamycin 600 mg every eight hours because of concern for vertical transmission with subsequent fetal sequelae and rapidly increasing maternal parasitemia despite therapy. Electrocardiogram was performed daily, as quinine is known to prolong the QT interval at standard doses []. After six days of antimicrobial therapy, she continued to experience fevers and rigors. Hemoglobin decreased to 8.9 [mg/dL], lactate dehydrogenase [LDH] >2000 [U/L], and serum haptoglobin < 20 [mg/dL]. The platelet count was mildly depressed but did not decrease below 125 [K/uL] at any point during her hospital course. Total bilirubin level was also elevated to 2.8 [mg/dL], consistent with hemolytic anemia. On the seventh day of therapy, oral atovaquone 750 mg twice a day was added to the quinine and oral clindamycin regimen in attempt to treat persistently rising parasitemia and worsening hemolysis.
Total red blood cell exchange procedure was performed because infection was refractory to standard antibiotic therapy for this extended time period. Central venous access was obtained and red blood cell exchange was performed at 24 4/7 weeks of gestation with six units of packet red blood cells. Fetal status was monitored and was reassuring before and after exchange transfusion.
Following red blood cell exchange transfusion, daily maternal serum parasite density measured <1% for four consecutive days. Oral azithromycin was substituted for quinine because of maternal QT prolongation (corrected QT interval 647 [ms]) and concern for quinine-induced hemolysis. Maternal QT interval returned to baseline within 24 hours of discontinuation of quinine. After improvement in her clinical status she was discharged from the hospital with oral atovaquone 750 mg two times a day, oral azithromycin 250 mg daily, and oral clindamycin 600 mg every eight hours. Her treatment course is outlined in . Following therapy, she continued to be anemic secondary to continued hemolysis despite resolution of parasitemia with hemoglobin 7.4 [mg/dL], reticulocytes 7.2 [%], total bilirubin 4.4 [mg/dL], lactate dehydrogenase 2369 [U/L], and serum haptoglobin <20 [mg/dL] and so received simple transfusion of 2 units of packed red blood cells. Maternal blood type was Rh negative, and Rh immune globulin was administered at 32 weeks of gestational age instead of our standard 28 weeks of gestational age because of transfusions. Following six weeks of antimicrobial therapy she was transitioned to daily oral azithromycin 250 mg for the duration of her pregnancy, with all subsequent peripheral blood smears negative for parasites.
Labor was induced electively with prostaglandins and oxytocin at 40 weeks of gestational age. Primary cesarean delivery for nonreassuring fetal status was uncomplicated. Postpartum repeat PCR for Babesia species (Mayo Clinic Laboratories, Rochester, MN) and peripheral blood smear were negative and so azithromycin was discontinued. Placental pathology noted a heavy for gestational age placenta and was significant for many mononuclear cells with folded nuclei in fetal vasculature with occasional nucleated erythrocytes, low grade chronic villitis with lymphohistiocytic inflammation, and chorioamniotic membranes with scattered macrophages. Immunohistochemical staining of mononuclear cells suggested a mixture of myeloid, monocytic, and erythroid cell lines consisted with a leukoerythroblastic reaction.
At time of delivery, the neonate was noted to have a diffuse, generalized nodular “blueberry muffin” petechial rash on his body including face, palms and soles, trunk, and all four extremities. Hepatomegaly was also noted on physical exam. Laboratory evaluation was notable for thrombocytopenia of 79 [K/uL], elevated lactate dehydrogenase to 3650 [U/L] and reticulocyte percent 6.3 [%], and elevated AST of 152 [U/L].
Initially thought to be secondary to vertical transmission of babesiosis, the neonate was treated with antibiotic therapy for three days until testing (including peripheral blood smears for parasites, real-time Babesia species PCR, and Babesia IgM antibodies [reference <1:20]) resulted negative (Mayo Clinic Laboratories, Rochester, MN). Neonatal Babesia IgG antibody was 1:256 [reference <1:64] which was attributed to passive placental passage. Other possible causes of “blueberry muffin” rash were also considered and evaluation for other infectious etiologies such as herpes simplex virus, rubella, parvovirus, cytomegalovirus and Lyme, Ehrlichia and Anaplasmosis, and noninfectious etiologies were negative. Skin biopsy was performed that confirmed extramedullary hematopoiesis. Etiology was attributed to Rh-incompatibility given maternal Rh negative and fetal Rh positive and Coomb's positive status. The neonate was discharged after monitoring in the neonatal intensive care unit and had close follow-up with pediatric infectious disease, dermatology, and hematology services with improvement of petechial rash at time of discharge. |
pmc-6582874-1 | A 60-year-old woman presented to our hospital with severe nausea, vomiting, watery diarrhea, and burning epigastric pain for a duration of one week. Her epigastric pain was associated with severe acid reflux, which had been intermittently present for a duration of two years and was resistant to over-the-counter low-dose proton pump inhibitor (PPI) therapy. Her past medical history was negative for any evidence of gastrointestinal (GI) bleed. Interestingly, the patient had a daughter who had been diagnosed with multiple endocrine neoplasia (MEN) type 1 a year prior to presentation. On physical exam, she was afebrile with stable hemodynamics. Abdominal palpation revealed mild epigastric tenderness without any guarding or rigidity. Cardiopulmonary exam was within normal limits.
Significant laboratory findings included WBC count of 15,000/microL, potassium of 3 mmol/L, magnesium of 0.7 mg/dL, and calcium of 11.8 mg/dL. Lipase level was within normal limits. Other pertinent laboratory values included fasting serum gastrin level of 1603 pg/mL (0-180 pg/mL), chromogranin A level of 14600 ng/mL (0-100 ng/mL), prolactin hormone level of 21 ng/mL (2-29 ng/mL), and parathyroid hormone (PTH) level of 473 pg/mL (10-65 pg/mL). She did not have any history of prior gastric surgeries, gastroparesis, or renal disease, to possibly explain her elevated gastrin level. An infectious workup for her diarrhea, including Clostridium difficile toxin and a stool PCR panel for common enteric pathogens, was negative. Subsequently, an extensive workup for evaluation of MEN was done, which revealed a unilateral parathyroid adenoma on neck imaging and diffuse stomach wall thickening along with pancreatic cystic lesions in body (1.2 cm) and tail (0.7 cm) on abdominal MRI (). Testing for pituitary disease was negative.
An esophagogastroduodenoscopy (EGD) was performed for further evaluation of her symptoms, which revealed severe reflux esophagitis, diffusely hypertrophic gastric rugae and multiple postbulbar ulcers in the duodenum (Figures , , and ). Endoscopic ultrasound (EUS) subsequently revealed diffuse thickening of the gastric rugae, predominantly of echo-layers I-III (). In addition, the patient was found to have a cystic lesion in the pancreatic neck with thick hypoechoic walls (). Random biopsies of the gastric antrum and body revealed patchy chronic gastritis with intestinal metaplasia () while FNA from pancreatic cyst revealed well differentiated NET ().
The patient eventually underwent a distal pancreatectomy and parathyroidectomy with clinical improvement. The remainder of her hospital course was uncomplicated and she was discharged home on high-dose PPI and octreotide. |
pmc-6582892-1 | A 75-year-old woman from Ghana with medical comorbidities of hypertension (not on an ACE inhibitor) and chronic cough was referred to our gastroenterology (GI) clinic for management of suspected gastroesophageal reflux disease (GERD) as the cause of chronic cough. As per the patient, she had been having a chronic cough for more than ten years. The cough was nonproductive, without any aggravating or relieving factors. She had reported postprandial heartburn. She recalls that cough started before her heartburn. She reported using albuterol and proton pump inhibitors (PPIs) without improvement in her cough. She did notice some improvement in her heartburn. She had never smoked, and her PPD was negative. There was no prior or current occupational exposure or pet exposure.
She underwent extensive otolaryngology evaluation including a laryngoscopy that showed evidence of chronic laryngopharyngeal reflux. She had been evaluated by pulmonologist and underwent spirometry, imaging studies, bronchoscopy, and fractional exhaled nitric oxide (FENO) testing and all the test results were normal.
She had been prescribed various therapies including oral, nasal, and inhaled corticosteroids, montelukast, and proton pump inhibitors without any improvement in cough.
Because of her typical GERD symptoms, she had a 48-hour Bravo pH testing done in 2011. The study revealed 4.9% of the time with pH below 4 and a total of 106 reflux episodes consistent with GERD. She was evaluated for the surgical intervention, and as a preoperative work-up, high-resolution esophageal manometry was performed. The manometric finding consistent with GERD revealed a hypotensive lower esophageal sphincter (LES). Subsequently, the patient underwent laparoscopic Nissen's fundoplication in 2012.
After fundoplication, she was symptom-free. However, in few months her cough recurred but absence of heartburn and overt acid reflux symptoms was intriguing. She again sought evaluation for cough. She underwent EGD and Bravo pH testing in 2013. There was no evidence of esophagitis and the endoscopic evidence of the fundoplication was appreciated. The ambulatory pH test revealed 0.1% of total acid exposure time and no symptom correlation. She had moved to Ghana for a brief period and did not seek any medical attention. She continued her PPI with no significant improvement.
Gastroenterology consultation was sought again as the cough persisted. The physical examination including vital signs was unremarkable. She underwent a repeat Bravo pH study in February 2018 that revealed zero acid exposure and the study was not consistent with GERD ( and ).
Subsequently, a high-resolution esophageal manometry was done in April 2018 with the findings indicating ineffective esophageal motility ().
A second opinion was sought from the gastrointestinal motility expert for the medical management of ineffective esophageal motility disorder. Given the absence of dysphagia, she was not considered a good candidate for medical management. To rule out nonacid reflux as a possible etiology of recurrent cough, patient was offered 24-hour Multichannel intraluminal impedance (MII) assisted pH monitoring. However, given the chronic cough and nasal discomfort, patient declined further intervention. Repeat laryngoscopy revealed laryngeal edema. To better assist with esophageal clearance, she was recommended to take frequent sips of water. She was suggested to carry a water bottle and take 1 to 2 sips of water every 15 minutes. Subsequently, patient presented to our clinic for a follow-up visit and was excited to report that her cough after years of work-up and medication use had finally subsided. She reported compliance with frequent sips of water. Patient had multiple interval follow-up for next 6 months where she reported continued absence of cough with multiple sips of water during the day. |
pmc-6582900-1 | A 20-year-old woman from Honduras presented on 3/20/2018 with her adoptive mother for a normal gynecological annual exam with a chief complaint of never having a menstrual period. She had lacked access to gynecological care in Honduras and had thus never been previously evaluated by a gynecologist. Her first sexual encounter was at age 17 and she reported having penetrative vaginal intercourse on a few occasions, although she was not currently sexually active at the time of evaluation. Previous sexual history included one male partner, condom use for protection against sexually transmitted infections, and good sexual function. Importantly, she had never experienced poor sexual satisfaction or dyspareunia. Her known family history was limited but was significant for a cousin who had also never had a menstrual period and a maternal grandmother who had had a brain tumor of unspecified type. She took no medications, and her only allergy was penicillin, with no reaction documented. She denied tobacco or illicit drug use and used alcohol occasionally. She denied any breast lumps, masses, nipple discharge, breast pain, excessive facial/body hair, abdominal/pelvic pain, genital lesions, rashes, or pruritus.
On physical exam, her vital signs were within normal limits and her body mass index (BMI) was 25.51. She was alert and oriented and in no acute distress. Neck was without lymphadenopathy or thyromegaly. Breasts were well-developed with no masses, tenderness, or discharge, Tanner stage V. Abdomen was soft and nontender. Gynecological exam showed no lesions and normal adult female pubic hair pattern, Tanner stage V. The cervix was unable to be palpated or visualized. The uterus was notpalpable and no adnexal masses were appreciated. The vagina was normal in appearance, measured 8 cm, was two fingerbreadths in diameter, and was without bleeding or discharge. The urethra was normal in appearance. The patient was cooperative, with appropriate mood and affect.
In light of the patient's presentation, transabdominal/transvaginal ultrasound was performed on 3/22/2018, which revealed bilateral remnant uteri with no endometrium appreciated (). The right remnant of the uterus was more prominent compared to the left. The cervix was not imaged. Follicle-containing ovaries were imaged transvaginally and transabdominally and were normal in size and appearance bilaterally, definitively excluding the presence of testes and effectively ruling out congenital androgen insensitivity syndrome (CAIS). There were no ovarian cysts or masses imaged. No fluid was appreciated in the cul de sac.
A follow-up MRI of the pelvis without and with contrast performed on 4/4/2018 demonstrated absence of the cervix, bilateral homogeneous enhancing uterine buds within the pelvis measuring 3.0 × 1.7 × 2.3 cm on right, 2.9 × 1.7 × 2.6 cm left. The upper one-third of the vagina was absent, while the lower two-thirds were present. The right ovary was of normal size, measuring 2.8 × 1.4 × 1.6 cm, and with multiple normal ovarian follicles. The left ovary was also of normal size measuring 2.1 × 1.5 × 1.9 cm, with multiple normal ovarian follicles and 1 cm left corpus luteum. Trace physiological free fluid was identified. No enlarged lymph nodes were noted. The bladder was unremarkable. The urethra was notable for 2 adjacent tiny cystic structures abutting the right posterior aspect of the external urethral meatus measuring 0.6 cm and 0.3 cm, respectively, most compatible with tiny Skene gland cysts. Together, the findings of primary amenorrhea, normal ovaries and female secondary sexual characteristics, and aplasia of the uterus with absence of the cervix and upper one-third of the vagina, were consistent with a diagnosis of MRKHS.
A subsequent biochemical analysis was performed to further support the diagnosis of MRKHS. Levels of estriol, follicle-stimulating hormone (FSH), luteinizing hormone (LH), total testosterone, and anti-Müllerian hormone (AMH) were all within normal limits, again consistent with the diagnosis of MRKHS (). The patient declined karyotype analysis due to financial limitations.
Because of the well-known association between MRKHS and anatomical abnormalities of the urological system [], a renal ultrasound was performed on 4/20/2018, which demonstrated normal bilateral kidneys, no evidence of hydronephrosis, and no evidence of contour deforming mass or shadowing renal calculus. Bilateral ureteral jets were demonstrated on color Doppler, and no distal hydroureter was seen. Thus, our patient displayed characteristics of MRKHS type I.
Upon hearing the diagnosis, our patient was anxious, especially with regard to future reproductive prospects; however she was counseled for 45 minutes with her stepmother/guardian present regarding the implications of the diagnosis and reproductive options such as use of a surrogate to carry a pregnancy for her. She expressed gratitude at the end of the encounter for the information and services provided and was offered follow-up as needed.
Upon last follow-up (August, 2018), the patient was doing well at age 21, living with her supportive stepmother, and not sexually active. |
pmc-6583233-1 | A 76-year-old Japanese man who had experienced recurrent chronic bronchiolitis with Pseudomonas aeruginosa infection for over 10 years presented with a 6-month history of haematuria/proteinuria and purpura. A skin biopsy revealed cutaneous leukocytoclastic vasculitis, and a renal biopsy showed pauci-immune crescentic glomerulonephritis. Serum examination by immunofluorescence showed the patient was negative for P-ANCA, but positive for C-ANCA. Routine enzyme-linked immunosorbent assay (ELISA) revealed that he was negative for both MPO-ANCA and PR3-ANCA. The titer of serum immune complexes (C1q binding assay) was 50.0 μg/mL (normal range; < 3.0 μg/mL). Further ELISA assay (ANCA panel kit, Euro Diagnostica) revealed that the antigen for C-ANCA was BPI (titer; 6.5 O.D. ratio, ) and other atypical ANCAs including azurocidin, cathepsin G, elastase, lactoferrin, and lysozyme were negative. Based on these findings, the patient was diagnosed with BPI-ANCA-associated systemic vasculitis (AAV). He was treated with prednisolone with antibiotics for GNB infections and his clinical findings were recovered ().
To evaluate the significance of BPI-ANCAs in vasculitis, healthy donor neutrophils were treated in vitro with BPI-ANCA immunoglobulin (Ig)Gs derived from the patient. BPI-AAV IgGs-treated neutrophils underwent Sytox Green-positive NET formation with histone citrullination under TNFα stimulation in a manner similar to that of MPO-AAV IgGs-treated neutrophils (). Since the polyclonal ANCA-IgGs were extracted from patient sera using Protein G column, to elucidate the pathogenicity of BPI-ANCA, a TNFα-primed neutrophils were treated with monoclonal BPI-ANCA and control antibody in the presence of recombinant BPI. Although the monoclonal BPI-ANCA did not induce NET formation, the immune complexes (ICs) of recombinant BPI and BPI-ANCA induced TNFα-dependent NET formation with hypercitrullination (). To elucidate the phenomenon that TNFα accelerated the ICs-induced NET formation, the expression of BPI in neutrophils with or without TNFα stimulation was evaluated by immunostaining. TNFα up-regulated BPI expression in neutrophils and the overexpressed BPIs were translocated to cell surface (). |
pmc-6584173-1 | A 55-year-old man came to our hospital with complaints of vague abdominal pain, burning micturition, hematuria, increased frequency and urgency of urine, more during night and pain during defecation since four months. He was referred to the urology department where the clinical examination was done including digital rectal examination which revealed an enlarged prostate. Further, more hematological investigations were done with no abnormal findings aside from prostate-specific antigen (PSA) which was mildly raised with a value of 12.4 ng/ml. He had no remarkable medical history.
The patient was further investigated in our radiology department where an abdominal ultrasonography and intravenous pyelography (IVP) was done. On ultrasonography, he was diagnosed with moderate hydroureteronephrosis with chronic cystitis with significant post void urine and increased volume of the prostate which measured approximately (75)cc with grade-III prostatomegaly and a large cystic swelling within prostatic parenchyma. On IVP it was diagnosed as moderate hydroureteronephrosis with significant residual post void urine.
Contrast-enhanced computed tomography (CECT) was done with axial images of delayed phase which showed well defined large cystic lesion measuring approximately 7.8 x 7.1 cm in the mid and left pelvis likely arising from the prostate. Lesion demonstrated multiple enhancing polypoid soft tissue components along the wall. The lesion abutted urinary bladder anteriorly, rectum medially and levator ani muscle posteroinferiorly with loss of fat planes as shown in Figure .
There was also mid circumferential wall thickening of the urinary bladder with few internal iliac lymph nodes as shown in Figure .
Bone scan and computed tomography (CT) of chest, abdomen, and pelvis were negative for metastasis.
Histological sections from the specimen, as seen in Figures -, consist of 19 out of 22 prostatic transurethral resection of prostate (TURP) chips which showed tumor composed of medium-sized glands with irregular outline and a smooth inner surface and scanty intervening stroma. Ducts were lined by pseudostratified columnar epithelium with abundant amphophilic cytoplasm and intraluminal eosinophilic secretion. Anisonucleosis was seen. On histopathological sections, diagnosis of ductal adenocarcinoma of the prostate was made with Gleason score 5+4=9, grade group V (WHO 2016 classification).
The patient underwent combination treatment open radical retropubic prostatectomy along with androgen blockade drugs, bicalutamide, and goserelin for six months. An 8-10 cm incision was made (well below the belt-line) in the skin between the umbilicus and the top of the pubic bone. The pelvis was then explored and the important structures such as the urinary bladder, prostate, urethra, blood vessels, and nerves were identified.
The prostate along with cyst was removed from the urethra below and the bladder above, and the bladder and urethra were reconnected. The yellowish color fluid was found in the cyst. The blood vessels leading to and from the prostate were divided and tied off. A catheter running through the penis into the bladder was typically required for at least a week after surgery. A surgical drain was left in the pelvis to allow drainage of blood and other fluid. Additional components of the operation include:
- Lymphadenectomy: Left sided internal iliac lymph nodes were removed. Prostate cancer often spreads to nearby lymph nodes in the early stages, especially the sentinel lymph node.
- Nerve-sparing surgery was attempted to protect the cavernous nerves of the penis, which control erection. These nerves are very thin and fragile and run next to the prostate and may be destroyed during surgery, leading to impotence.
- Bone scan and CT chest, abdomen, and pelvis were negative for metastasis; radiotherapy was not given to the patient. Serum PSA level was within normal range of 4 ng/ml and under follow up. |
pmc-6584174-1 | A 79-year-old male with a past history of aortic valve replacement presented to our hospital with sudden onset of lightheadedness and fatigue. On initial assessment, he was hypotensive with blood pressure (BP) 95/54, and pulse rate 168 beats per minute. Electrocardiogram (EKG) showed a wide complex tachycardia with a ventricular rate of 156 beats per minute (Figure ).
Direct cardioversion was attempted followed by a loading dose of amiodarone and the patient subsequently electrically converted to sinus rhythm. Repeat EKG showed normal sinus rhythm with left bundle branch block (LBBB) and rate of 78/minute (Figure ).
The patient was transitioned from amiodarone infusion to oral metoprolol. Further work-up revealed elevated creatinine, normocytic anemia, and elevated pro-brain natriuretic peptide (BNP). Echocardiogram showed a normal left ventricle ejection fraction, diastolic function, and a normally functioning bio-prosthetic valve. Chest X-ray showed a left hilar and suprahilar opacity (Figure ).
Computed tomography (CT) of the thorax with contrast further elaborated a 6.3 x 5.8 cm left upper lobe mass extending into the left hilum and encasing the left pulmonary arteries and veins (Figure ).
Associated findings were mediastinal lymphadenopathy involving the left superior mediastinum and CT-guided biopsy of the lymph node showed metastatic lung adenocarcinoma. Given the extensive involvement of mediastinal structures, the tumor was deemed unresectable and a decision was made to treat it with palliative chemotherapy. Metoprolol was continued for rate control and the patient continued to remain in sinus rhythm. |
pmc-6584187-1 | A 20-year-old woman presented to the emergency department with complaints of severe abdominal pain, multiple episodes of vomiting and obstipation for one day. She was a known case of abdominal tuberculosis and had received anti-tubercular treatment for two months. Vital signs showed a blood pressure of 100/64 mm Hg, heart rate of 110 per minute, respiratory rate of 18 per minute, oxygen saturation of 96% on room air and a temperature of 100.5°F. She was cachectic with a weight of 28 kg which was less than 5th percentile for gender and age matched normal population. Physical examination revealed diffuse abdominal tenderness and rigidity. Her erect abdominal radiography showed gas under right dome of the diaphragm. Clinical diagnosis of perforation peritonitis was made and the patient was wheeled to the operating room for emergent laparotomy. Intraoperatively, a 3 cm x 2 cm single ileal perforation was found 1 foot proximal to the ileocecal junction, with unhealthy bowel margins and multiple mesenteric lymphadenopathy. Resection of perforated bowel segment with proximal ileostomy and mucus fistula of distal ileum was performed. Later, histopathology report confirmed tubercular etiology showing caseous necrosis in mesenteric lymph nodes and epithelioid granuloma in ulcer edge biopsy. Two days after the surgery, the patient was started on oral feeds and was accepting meals orally subsequently. Ten days following surgery, the patient had multiple episodes of bilious vomiting with stoma output decreasing to less than 100 mL per day. Her abdomen remained flat with no increase in bowel sounds. Abdominal radiography showed dilated stomach with no air fluid levels. Oral feeds were stopped and a nasogastric tube was placed for drainage of gastric contents. A contrast enhanced computed tomography (CECT) abdomen was done to determine the cause of obstruction which revealed a decreased aortomesenteric angle of 15° (Figure ).
As a result of this decreased angle, an abrupt collapse of the third part of the duodenum was seen along with proximal duodenal and stomach dilation (Figure ).
Based on the clinical features and CECT findings, diagnosis of SMA syndrome was made and the patient was managed conservatively. The patient was not tolerating enteral feeds and no relief of postprandial abdominal pain was noticed even on changing to left lateral decubitus or prone position. Considering the patient’s nutritional condition, total parenteral nutrition (TPN) was started. Anti-tubercular drugs were continued with oral sips. She was started on 1500 kcal/day (50 kcal/kg/day), taking care of refeeding syndrome and was gradually titrated up to provide maximal caloric support. Monitoring of weight, blood glucose, electrolytes, liver function test, and lipid profile was done to prevent any TPN complications. A good clinical response was seen after starting TPN. The patient’s weight gradually increased to 34 kg in three weeks and gradually the stoma output increased and nasogastric drainage decreased. TPN was tapered off gradually with concurrent starting of oral feeds. On discharge, the patient was advised a high caloric and protein diet with small frequent meals. A follow-up CECT was done after six weeks from discharge which showed an increase in aortomesenteric angle from 15° to 21° (Figure ) with the patient being asymptomatic and a total weight gain of 12 kg post-surgery. |
pmc-6584600-1 | An 8-year-old neutered male domestic short hair cat weighing 5.0 kg presented to a surgical referral center for right caudal lung lobectomy following diagnosis of a right caudal lung lobe mass on thoracic radiographs () (day 0). The patient was asymptomatic for respiratory disease and the radiographs were performed by the primary care veterinarian as part of a diagnostic work-up for a newly detected I/VI parasternal systolic heart murmur. The patient had a medical history of chronic crystaluria and had been treated previously for urethral obstruction. The patient had no known travel history.
ECG and echocardiogram were performed by a board certified veterinary cardiologist and revealed no significant abnormal findings. Complete blood count (CBC) and blood chemistry were performed and were within normal limits. Given the patient's geographic location (Arizona, USA) coccidiomycosis (“Valley Fever”) titers were assessed via serology and found to be negative.
Computed tomography (CT) (Toshiba Aquilion 16) of the thorax was performed and revealed an amorphous contrast enhancing mass in the peripheral right caudal lung lobe () without evidence of regional lymphadenopathy. Differentials for the mass included a bronchogenic carcinoma or a non-discrete inflammatory or infectious lesion. The patient was taken to surgery and a right caudal lung lobectomy via a right 6th intercostal thoracotomy was performed. The excised lung lobe was submitted for histopathological assessment and microbial culture. A thoracotomy tube was placed and the surgery was concluded without complication. The patient recovered successfully and was discharged from hospital the following day on oral anti-inflammatory (Robenacoxib, 6 mg PO SID), opioid (Buprenorphine, 0.1 mg PO TID) and antibiotic (Amoxicillin-Clavulanic Acid, 62.5 mg PO BID) medications. Three days following surgery, histopathology results were available and revealed a widespread lipoid/xanthogranulomatous pneumonia with scattered intra-lesional organisms consistent with Cryptococcus spp. (). No evidence of neoplasia was detected. Five days following surgery, aerobic culture results were available and revealed the isolate C. gattii. No organisms were isolated on anaerobic culture.
Following the returned histologic and culture diagnosis, Cryptococcus titers were serologically assessed for confirmation and found to be positive (1:256) (day 10). The cat was started on anti-fungal therapy (Fluconazole 100 mg SID) and has remained free of clinical disease at the time of last follow up (60 days). |
pmc-6584812-1 | A 16-year-old female patient was investigated for hypothyroidism due to hair loss. During the routine physical examination, two thyroid nodules were identified; therefore, the patient was referred to the Endocrine Unit of the 2nd Department of Medicine, Semmelweis University, for further endocrinological investigation in 2013. Thyroid Stimulating Hormone (TSH) level was within the physiological range (2.44 mU/L; reference value: 0.35–4.94 mU/L). Due to the present thyroid nodules thyroid ultrasound examination was carried out. Two hypoechoic, inhomogeneous masses with microcalcification were identified in both lobes of the thyroid gland. Biopsy and fine-needle aspiration biopsy (FNAB) were performed. Pathological examination revealed invasive growth of tumor cells with coarsely granulated chromatin and high number of mitosis. Calcitonin immunohistochemistry showed strong positivity in 100% of the cancer cells. Computerized tomography (CT) confirmed two masses: in the right lobe the tumor diameter was 2.2 cm, whereas in the left lobe 0.6 cm. The patient’s serum calcitonin (501 pg/ml; reference value: 0–6 pg/ml) and carcinoembryonic antigen (CEA, 7.4 ng/ml; reference value: 0–4.3 ng/ml) levels were markedly elevated. Plasma parathormone level (37.9 pg/ml; reference value: 15–65 pg/ml) and 24-h collected urine metanephrine (178 mcg/24 h; reference value: 64–302 mcg/24 h) and normetanephrine (385 mcg/24 h; reference value: 162–527 mcg/24 h) levels were within reference range.
A total thyroidectomy was performed and pathological examination of the specimen confirmed MTC. Metastases were detected in three lymph nodes removed from the left side, whereas four lymph nodes removed from the right side were metastasis free and no distant metastasis was detected. The TNM classification of the tumor was pT2N1a (). Although the serum calcitonin and CEA levels significantly decreased after the surgery, complete biochemical remission has not been achieved since, and the serum calcitonin levels has shown uncertain progression in the range of 31–178 ng/L during the clinical follow-up in the last 6 years. Based on the postoperative staging CT scan, one small residual thyroid tissue was suspected without any sign of metastases. Repeated ultrasound and FNAB confirmed the lesion to be scar tissue and did not affirm the presence of residual thyroid tissue. Thyroid stimulating hormone levels increased after the total thyroidectomy to 31.1 mU/L, and after successful thyroid hormone substitution with L-thyroxine, physiological values were reached as TSH concentrations varied between 0.128 and 3.843 mU/L (reference value: 0.35–4.94 mU/L).
After genetic counseling, the patient underwent genetic testing () of RET gene, and a pathogenic heterozygous mutation (p.Cys634Trp) was identified (). Two other synonymous frequent RET polymorphisms (rs1800860 and rs1800861) were also detected. The p.Cys634Trp is a well-known mutation associated with MEN2A syndrome; therefore, the patient underwent detailed clinical, laboratory, and imaging studies for manifestations of MEN2A. Adrenal and/or extra-adrenal paraganglial tumors were ruled out by CT imaging of the head, neck, chest, and abdominal region. No other neoplastic lesions were observed. Biochemical data for free catecholamines and metanephrines in the 24 hour urine revealed no sign of catecholamine or metanephrine secreting tumor. Serum intact parathormone was within the normal range, and no sign of parathyroid malfunction was detected. During the last 6-year follow-up, the proband is still free of any MEN2A associated lesions.
Due to the fact that MEN2A syndrome is inherited in an autosomal dominant way, genetic counseling and genetic testing of first degree relatives are indicated (). The detailed family history revealed that the patient’s mother and her aunt had breast cancer and both died due to brain metastases. This unexpected clinical presentation was suspicious to hereditary breast cancer syndrome. However, no samples for mutation analysis of BRCA1/2 genes were available from these two affected relatives. Hereditary breast cancer is also an autosomal dominant disorder mutation, and thus, analysis is recommended for the first-degree relatives (). Accordingly, the mutation analysis of BRCA1/2 genes identified a pathogenic, heterozygous frameshift BRCA1 mutation (BRCA1 Ile90Serfs, NC_000017.10:g.41256905_41256917 delTGAAAAGCACAAA) in the proband (). Clinical examination and breast magnetic resonance imaging (MRI) revealed no sign of malignancy in the breasts, and 6 years after initial examination still no signs of breast or ovarian cancer are present. After these striking results, the patient’s family members underwent genetic counseling again and, after obtaining informed consent, underwent mutation testing for the RET exon 11 and BRCA1 genes. The proband’s brother (age: 36 years) and his two sons (age: 4 and 6 years) were also heterozygotes for the RET p.C634W mutation, but the BRCA1 mutation was absent in them. In her brother, two thyroid masses and elevated serum calcitonin level (215 pg/ml) were observed. Total thyroidectomy was performed, and his serum calcitonin returned to normal range. Two sons of the proband’s brother underwent prophylactic thyroidectomy, and the histopathological examination of the removed tissues showed no signs of MTC. The proband’s father did not carry the RET mutation and the BRCA1 mutation. The pedigree of the family is shown in . |
pmc-6584842-1 | A 36 years old male patient came with complaints of swelling in the right side of the scrotum since 1 year not associated with pain. The right side scrotal swelling was gradually progressive and on palpation it was hard and non tender. USG of the scrotum was done, which showed a well defined heterogenous legion of size 6.4 × 5.9 × 4 cms in the right scrotal sac arising from the lower pole of right testis with multiple cystic areas within suggestive of likely neoplastic etiology. Blood tumor markers showed raised levels- B-HCG 27.76 miu/ml, A.F.P- 251.69 ng/ml, Sr. LDH-642 units/L.
A Contrast enhanced CT of the abdomen showed a well defined heterogenous lesion of size 4.8 × 3.4 cms is noted arising from the lower pole of left kidney of a possible neoplastic etiology.
Patient underwent right sided high inguinal orchidectomy and the specimen was sent for histopathological examination (). On gross and microscopic examination, it was revealed that the specimen is suggestive of mixed germ cell tumor: embryonal carcinoma, teratoma and seminoma.
Later, a CT guided core biopsy of the left renal mass was done. The biopsy report suggested epithelial renal tumor, probably oncocytoma/low grade renal cell carcinoma (RCC).
Patient was given six chemotherapy cycles of Etoposide-Cisplatin regimen. After completing Etoposide-Cisplatin regimen patient underwent a review CT abdomen which suggested of a heterogenous mass from lower pole of left kidney like Oncocytoma? ? Renal cell carcinoma.
The patient underwent left partial Nephrectomy(). The histopathological examination of which was suggestive of clear cell variant of renal cell carcinoma – Grade II. |
pmc-6584846-1 | A 62-year-old male, with a history of non-ischemic cardiomyopathy presented with new onset atrial flutter. He reported shortness of breath, diaphoresis and lightheadedness for ~ 1 week as well as progressive cervical adenopathy, fevers, and unintentional weight loss. Laboratory evaluation demonstrated white blood cell (WBC) count of 17.3 ×109/L with 37% (6.4 ×109/L) plasma cells, hemoglobin of 11.6 g/dL, and platelet count of 53 ×109/L. Contrast-enhanced computed tomography (CT) scans of the chest, abdomen, and pelvis revealed non-bulky cervical, axillary, mediastinal, retroperitoneal and inguinal lymphadenopathy, ranging in size between 1.4 cm and 2.5 cm, as well as splenomegaly of 15.5 cm, without discrete lesions. Shortly after admission, the patient developed acute renal failure and serum electrolyte abnormalities consistent with tumor lysis syndrome (TLS) (creatinine of 4.2 mg/dL, potassium of 5.4 mmol/L, phosphate of 5.3 mg/dL, urate of 11.9 mg/dL, lactate dehydrogenase of 368 IU/L). Peripheral blood flow cytometry revealed that 46% of the leukocytes were represented by polyclonal plasma cells (CD19+, CD20−, CD22−, CD45+(moderate), CD38+(bright), CD56−, CD117−, CD138+, HLA-DR+(heterogeneous), surface κ or λ−, cytoplasmic κ +(subset), cytoplasmic λ+(subset)). Serum protein electrophoresis (SPEP) and immunofixation electrophoresis (IFE) showed abnormally high gamma globulin levels (IgA 1200 mg/dL and IgG 4200 mg/dL) without a monoclonal paraprotein. Serum kappa and lambda light chain levels were modestly elevated (92.6 mg/dL and 73 mg/dL, respectively) but the ratio was normal (1.27).
There was initial concern for an infectious etiology for the patient's presentation given the signs of systemic inflammatory response syndrome (tachycardia, leukocytosis, fever) and circulating polyclonal plasma cells. Blood cultures and viral studies were performed and were notable for a detectable plasma EBV viral load, which was quantified by qRT-PCR as 71,000 copies/mL of pEBVd, and approximately 24 h later increased to 1.05 ×106 copies/mL. Human immunodeficiency virus (HIV) and hepatitis C serologies were negative, hepatitis B serology was consistent with prior immunization, and plasma cytomegalovirus DNA (pCMVd) was negative. Serum antinuclear antibody (ANA) titer for initial workup of autoimmune disease was also negative. After bone marrow and lymph node biopsies were obtained, dexamethasone 40 mg daily was initiated, as concerns for an EBV associated lymphoproliferative disorder (EBV-LPD) were high. In addition, the patient was started on antiviral therapy with ganciclovir 2.5 mg/kg IV every 12 h for possible active EBV infection. The drug was stopped after 5 days as the treating physicians concluded that the risk of drug toxicity outweighed the benefit, and the data to support its use in this setting were insufficient. Despite these measures and aggressive supportive care, within 1 week of admission, his clinical course progressed to multiorgan failure requiring mechanical ventilation and ultimately cardiac arrest requiring extracorporeal membrane oxygenation (ECMO).
Bone marrow exam revealed hypercellularity (90%) with 30–40% plasma cells (which was deemed to be reactive because of polyclonal kappa/lambda expression), large lymphoid aggregates with predominance of T cells, and scattered EBV+ B cells. Molecular studies for T-cell receptor (TCR) gamma gene and immunoglobulin heavy chain (IGH) gene rearrangements were performed on the blood sample. A monoclonal TCR gamma gene rearrangement was detected; clonal IGH gene rearrangement was not detected. The left inguinal node showed completely effaced normal lymph node architecture with a diffuse infiltration of atypical small to medium sized lymphocytes in a background of vascular proliferation, plasma cells, and scattered immunoblasts. By immunohistochemistry and flow cytometry, there was a diffusely increased T cells infiltrate admixed with scattered B immunoblasts. The T cells expressed CD2, CD3, CD5, CD7, PD-1, and were negative for CD5, CD10, BCL-6, and all B-cell antigens analyzed. CD4 to CD8 ratio was within normal range. Scattered large immunoblasts were positive for CD20 and EBV (by EBER in situ hybridization). CD21 revealed several expanded clusters of follicular dendritic cells. In situ hybridization for immunoglobulin kappa and lambda light chains revealed numerous polyclonal plasma cells. Molecular studies for TCR gamma gene and IGH gene rearrangements were repeated on the lymph node. Identical clonal TCR gamma gene rearrangement was detected in the lymph node. Similarly, clonal IGH gene rearrangement was not detected. Representative images are shown in .
In aggregate, these findings were thought to be most consistent with a diagnosis of AITL. Pathologically, the complete effacement of nodal architecture, diffuse involvement of bone marrow, and the presence of clonal T-cell gene rearrangement rule out conditions of reactive EBV infection such as infectious mononucleosis and chronic active EBV infection. Unfortunately, the patient failed to improve with dexamethasone and the family decided to withdraw care. A request for autopsy was declined. |
pmc-6584909-1 | A 31-years old male patient with no medical comorbidity presented to our facility. Six years prior to this presentation he had an isolated left testicular mass detected on physical examination and CT which proved to be a malignant germ cell tumour. He underwent left orchiectomy but retroperitoneal lymph node dissection (RPLND) was not carried out as the CT showed no enlarged lymph nodes and histopathology showed the presence of a teratoma and yolk sac tumour. No further details however were mentioned. Accordingly, he received four cycles of chemotherapy (Cisplatin, Bleomycin, and Etoposide). He did well for six years, had no major complaints, and underwent only one follow up ultrasound which showed no suspicious abdominal swellings. He presented to us with on and off upper abdominal pain of six months duration, with no other associated symptoms. On physical examination he was vitally stable with a soft and lax abdomen but mild tenderness in the upper central part of the abdomen and a deep mass. Groin examination revealed signs of a left orchiectomy; the right testicle was in normal position with no swelling or other abnormality detected. Abdominopelvic US showed a large retroperitoneal semisolid mass measuring 8 by 6 cm, abating the body and tail of the pancreas. Chest and abdominopelvic CT scan with IV and oral contrast were carried out and showed two lesions, one abating the head of the pancreas (3 × 2 cm in size) and the other baiting the body and tail of the pancreas with clear separation distal (). All tumour markers, including B-HCG, AFP, CA 19-9, CEA, and CA 19-9, were within the normal range. The patient underwent midline laparotomy and complete resection both retroperitoneal masses with preservation of the pancreas (). The recovery was uneventful, and the patient was discharged five days postoperatively. Histopathology of the masses showed a metastatic germ cell tumour of the teratoma component in the background of lymphoid tissue (). Thus, the tumour board diagnosed this as stage IV metastatic germ cell tumour of the teratoma, and recommended to commence the patient on chemotherapy (Cisplatin, Bleomycin, and Etoposide). The patient tolerated four cycles of chemotherapy. The follow-up PET scan at six months postoperatively showed no trace or recurrence of the tumour (). Currently, the patient has been followed up with ultrasound for two years with no recurrence. |
pmc-6584983-1 | A 47-year-old man was referred to our hospital with a 3-month history of abdominal distension, intermittent abdominal pain and nausea. Despite weight loss of 4 kg, the patient had no symptom of fever, chronic cough or night sweats. He had no history of abdominal surgery, liver cirrhosis or chronic hepatitis virus infection, but suffered from tuberculous pleurisy about 20 years ago. Physical examination revealed gross abdominal distension without tender and ascites of unknown aetiology. Laboratory blood analyses including serum tumor markers, erythrocyte sedimentationrate (ESR), adenosine deaminase activity (ADA) and anti-tuberculosis antibody (TB-Ab) were all within normal limits, and tuberculin skin test was negative. The results of antibody testing for HIV were negative. Chest radiograph did not demonstrate features suggestive of pulmonary tuberculosis. Plain upright abdominal X-ray showed some air in the colon without presence of air fluid levels in the loops or free gas under the diaphragm (Fig. a). Contrast-enhanced computed tomogram (CT) of the abdomen (Fig. b, c) revealed dilatation of the duodenum loops (*) and congregated small gut loops (black arrowhead) trapped in the massive ascites surrounded by a membrane (white arrowhead). Gastroscopy revealed no signs of malignancy. A peritoneal tap was performed twice and yielded blood stained ascitic fluid but no malignant cells or acid-fast bacilli. The ascities was exudates in nature and ascities ADA was within normal ranges. The patient refused laparoscopy examination and was discharged without a definitive diagnosis and further treatment.
Two months later, the patient was presented to our hospital again with persistent symptoms. Despite abdominal distension, nausea and vomiting, he also complained of increasing fatigue, emaciation, and 10-kg weight loss for the recent two months. Laboratory blood analyses revealed that serum ADA was 21 U/L (normal range 4–18 U/L), ESR was 28 mm/h (normal range 0–15 mm/h), but TB-Ab was negative. Ascitic fluid obtained by paracentesis was still negative for bacteria, acid-fast bacilli and malignant cells, but ascities ADA was significantly elevated (41 U/L). Serum CA-125 was also significantly elevated (97.39 U/ml, normal range < 35 U/L). In addition, despite the presence of well-encapsulated fluid collection and central accumulation of the small intestine (Fig. a), CT scan also revealed smudged appearance of the greater omentum (Fig. b,*), as well as multiple small nodules and sheetlike lesions on the parietal peritoneum (Fig. . s, arrowhead), which the radiologists considered to be peritoneal carcinomatosis (PC). Mesenteric lymphadenopathy was not observed on CT scan. The presumptive diagnosis was PC or tuberculous peritonitis in our patient, however, other differential diagnosis, such as peritoneal mesothelioma, could not be completely ruled out.
Diagnostic laparoscopy was performed in our patient, and the entire small bowel was found to be encapsulated in a dense, white, fibrous, cocoon-like membrane filled with a large amount of brownish ascites (Fig. d). Multitudinous miliary nodules or tubercles were seen on the parietal peritoneum and greater omentum. Biopsy of the nodules and plaques revealed caseous granulomatous inflammation and the specimen were stained positive for acid-fast bacilli. Histopathology of the cocoon wall revealed fibrocollagenic tissue with granulomatous inflammation, and culture of the biopsied specimen grew Mycobacterium tuberculosis 3 weeks later. A diagnosis of tuberculous abdominal cocoon was made. Complete excision of the thick membrane and lysis of adhesions were tried but failed due to the extreme difficulty in separating the fibrotic tissue from the abdominal wall and small bowel. Since there were no perforated, ischemic or dilated bowel loops, the operation was completed after obtaining samples for diagnostic biopsy.
After operation, the patient had recurrent episodes of partial bowel obstruction that was resolved with conservative management. The patient was given rifampicin, isoniazid, ethambutol, and pyrazinamide for 2 months, followed by rifampicin and isoniazid for 4 months. By the end of the 2 month of anti-tuberculous therapy, serum ADA, ESR and CA 125 levels have returned to normal. Though a repeat CT scan of the abdomen and pelvis continued to show the small bowel wall thickening and membrane covering the small bowels (Fig. a, arrowhead), and gastrografin meal follow through study revealed adherent small bowel loops with delayed transit time (Fig. b), however, the ascites has greatly diminished and the patient was functioning normally. No further surgical intervention was planned for him, and he was followed up symptom-free for 6 years. |
pmc-6584991-1 | An 18-year-old Chinese female student presented at our hospital with a 1-day history of fever (temperature 39.5 °C), severe stomachache and headaches, and gradually diminished vision in the left eye. She had a history of intermittent headaches for 5 months and experienced blurred vision with shadows and floaters on the left eye for 10 days in 2016. Clinical examination in the left eye showed that the best corrected visual acuity (BCVA) was 20/40 (Snellen chart). Ultrasound imaging revealed the presence of a cyst in the vitreous cavity on the left eye. Fundus examination showed a free-floating and semi-translucent vitreous cyst in the mid-vitreous cavity and retinal vasculitis with optic disc edema. The patient underwent a 23-gauge pars plana vitrectomy (Constellation System, Alcon). The cyst was extracted in whole (Fig. a) with laser photocoagulation. The flat mount of the cyst was identified as oval cysticercus with a length of 0.5 cm, containing invaginated a scolex with hooklets (Fig. b).
Magnetic resonance imaging (MRI) with Gd-DTPA as the contrast agent was performed. MRI of the orbits and brain showed well-defined ring-enhancing cystic lesions with eccentric scolexes in the extraocular muscles, brain parenchyma (Fig. a), tongue, face, neck muscles, and cutaneous tissues (Fig. b). Abdominal MRI revealed round hyperintense lesions in the diaphragm, abdominal muscles, erector spinae, and psoas magnus muscles in T2-weighted images (T2WI) with an identifiable hypointense nidus in T1-weighted images (T1WI) and T2WI. Cystic lesions were found to be scattered around muscle tissues of the whole body (Fig. c and d).
Anti-T. solium cysticercal IgG antibodies were detected in the serum and cerebrospinal fluid (CSF) by enzyme-linked immunosorbent assay (ELISA; JL0702193, Jianlun Biology Technology Co., Ltd., Guangzhou, P.R. China). The CSF pressure was higher than 320 mmH2O and biochemical, cytological, and microbiological examinations of the CSF showed 8*106/L WBC, higher protein level (56 mg/dL; normal range 15–40 mg/dL), normal glucose level (2.39 mmol/L in the CSF versus 4.31 mmol/L in blood), and negative bacterial culture.
Systemic oral albendazole and steroid treatment were initiated after the ophthalmic operation. In the first course, albendazole at a quarter of the total dose (20 mg/kg body weight daily, three times a day for 10 days) during the first course with 5 mg of intravenous dexamethasone was given daily. An acute response was observed on the third day, which was attributed to local inflammation due to the larvae death. The intravenous dose of dexamethasone was increased to 10 mg daily until albendazole treatment was discontinued. The patients received albendazole at a total dose of 20 mg/kg body weight daily for 10 days, and another two courses of albendazole equal dose with 3 months treatment interval. Most of cysts, especially intraparenchymal cysts, were disappeared (Fig. ). |
pmc-6584991-2 | A 24-year-old Chinese male with no medical history was admitted to the Department of Neurology in one institution of the Henan Province with a persistent diminution of vision in the left eye, along with headaches and without seizure since 2012. The patient had been living in a rural area in the Henan Province and have never been to other provinces. He had no fever upon hospital admission. The brain MRI showed multiple left parietal cysts at the vesicular stage with edema. MRI of the orbits showed one well-defined ring-enhancing cystic lesion with eccentric scolex in the left extraocular muscle. X-ray imaging of both arms showed multiple nodular calcifications.
The detection of anticysticercal IgG antibodies by ELISA (JL0702193, Jianlun Biology Technology Co., LTD, Guangzhou, P.R. China) was positive in the serum and CSF. CSF biochemical, cytological, and microbiological examinations showed 5*107/L WBCs, higher protein level (66 mg/dL), normal glucose level in the CSF and blood, and a negative bacterial culture.
After the successful ophthalmic surgery, systemic oral albendazole and steroid treatment were initiated. In the first course, the patient received albendazole (20 mg/kg body weight daily, three times a day for 10 days) with 5 mg of intravenous dexamethasone daily, and the patient had a serious headache on the 3rd day, which was attributed to local inflammation caused by the death of the larvae. Intravenous dexamethasone was increased to 10 mg daily until the albendazole treatment was discontinued. He received two additional courses of albendazole with 3-month intervals. After three treatment cycles, most of the neural cysts were effectively destroyed, and some were calcified. |
pmc-6584991-3 | A 42-year-old Chinese male farmer with no medical history was admitted to the Department of Neurology of one institution in the Heilongjiang Province with a possible tonic-clonic seizure witnessed by his family, along with reduction of vision in the right eye since 2015. He had been living in a rural area in Heilongjiang Province for 10 years without traveling to other areas. White tapeworm segments were found in his feces. The brain MRI showed multiple parietal cysts at the vesicular stage with edema in the frontal, parietal, and occipital white matter.
MRI of the orbits showed one well-defined ring-enhancing cystic lesion involved the right superior rectus. X-ray imaging of the chest showed multiple nodular calcifications. The detection of anticysticercal IgG antibodies by ELISA (JL0702193, Jianlun Biology Technology Co., LTD, Guangzhou, P.R. China) was positive in the serum and CSF. CSF biochemical, cytological, and microbiological examinations showed normal WBCs, normal protein level (23.52 mg/dL), normal glucose level in the CSF and blood, and a negative bacterial culture.
After the successful ophthalmic surgery, systemic oral albendazole and steroid treatment were initiated. In the first course, the patient received albendazole (20 mg/kg body weight daily, three times a day for 10 days) with 5 mg of intravenous dexamethasone, along with an oral antiepileptic drug. The patient had severe epilepsy on the 3rd day, and intravenous dexamethasone was increased to 10 mg daily. At the same time, antiepileptic therapy was given until albendazole treatment was discontinued. The patient received three additional courses of albendazole with 3-month intervals. After four treatment cycles, most of the neural cysts were effectively destroyed. |
pmc-6585016-1 | A 72-year-old Korean man with a past medical history of ICD insertion for idiopathic ventricular fibrillation (device: Medtronic Protecta XT VRD354VRM; lead: Medtronic Sprint Quattro Secure Model 6947) presented with a 1-month history of complaint of a dry cough. He had a 50–pack-year history of smoking. His family history was negative for any malignancy. Chest x-ray and contrast-enhanced computed tomography showed a conglomerate nodal mass in the left central lung and left hilar area (Fig. a, b). Bronchoscopy was performed, and the cell block obtained from a needle biopsy was evaluated. A photomicrograph of the bronchoscopic biopsy showed a nest of atypical cells that squeezed hyperchromatic nuclei (Fig. c). IHC showed that these cells were positive for neuroendocrine markers, such as CD56 and chromogranin, and negative for CD45RO. The patient had an elevated serum lactate dehydrogenase level (337 U/L). Positron emission tomography excluded any additional disease localizations (Fig. d). The patient was diagnosed with a limited stage of small cell lung cancer in the left lung (cT4N2M0 by TNM staging).
The patient was recommended for concurrent chemoradiotherapy (CCRT), but he refused CCRT because of fear of toxicity. The tumor showed partial remission after four cycles of chemotherapy (cisplatin 25 mg/m2 on days 1, 2, and 3 and etoposide 100 mg/m2 on days 1, 2, and 3). He was referred for sequential thoracic radiotherapy. After a multidisciplinary meeting, we decided to treat him with radiotherapy and that the condition of his ICD would be monitored by a cardiologist during radiotherapy.
The primary tumor, regional gross lymph nodes, and surrounding normal structures were contoured in radiotherapy planning computed tomography. For ICD delineation, three parts of the ICD were contoured: the body of the ICD, the leads, and the electrode. Dose calculation was performed using analytical anisotropic algorithm (version 8.9.17). The goal of treatment planning was to achieve a dose to the target volume greater than 97% of the prescribed dose while minimizing the dose to surrounding normal organs and avoiding the directly irradiated field of beam arrangement into the contour of the lead and electrode. The prescribed dose was 60 Gy in 30 fractions five times per week. On dose-volume histogram (DVH) analysis, the mean and maximum doses of ICD were 0.73 Gy and 1.43 Gy, respectively, in the body. The mean and maximum doses of the lead and electrode were 17.12 Gy and 55.72 Gy in the lead and 1.81 Gy and 7.10 Gy in the electrode, respectively; this was because parts of the lead and electrode were inevitably in the irradiation fields. Radiation was delivered by linear accelerator (Varian Clinac 21EX; Varian Medical Systems, Palo Alto, CA, USA) using 10-MV 3-fixed photon beams in two sequential dosimetric treatment planning steps (Fig. ).
Prior to each treatment, we placed a magnet on the ICD to suspend tachyarrhythmia detection because this can prevent ICD failure, and we used electronic portal imaging for image guidance. The ICD dose was calculated using a metal oxide semiconductor field effect transistor (MOSFET) dosimeter above an external magnet superposed on the ICD during every treatment. The mean and maximum doses estimated at the body of the ICD in vivo were 0.89 Gy and 2.23 Gy, respectively.
The function of the patient’s ICD was checked daily, and no change was observed during radiotherapy. The patient showed good tolerance without severe complications. Computed tomography performed at 4 weeks after radiotherapy showed good response with tumor regression. The patient remained in complete remission without ICD dysfunction more than 36 months after treatment completion. |
pmc-6585036-1 | A 22-year-old young Asian lady presented to emergency dept. with short history of bluish discoloration of her skin, primarily over lips, tongue, hands and feet. She also had complaints of gradually progressive dyspnea, palpitation and apprehension. She denied any history of fever, cough or sputum production. For these complaints she visited different health facilities & was given nebulization, supplemental oxygen, but symptoms didn’t improve.
Past history included painful red nodules over extensor surface of lower limbs, which resolved with steroids use.. However they reappeared many times later and did not regress much with steroids afterwards. Later on she was diagnosed to have erythema nodosum, and was prescribed steroids once again.
On examination, she was dyspneic, centrally cyanosed with 86% O2saturation at room air. Rest of the examination was unremarkable. She was given supplemental oxygen via face mask but saturation didn’t improve more than 88%. All her laboratory investigations were normal Table . Her ABGs at maximum O2 revealed PaO2 88 (60–90 mmHg), pH 7.41 (7.36–7.46), PCO2 38.2 (34-46 mmHg), HCO3 26 (22-27 mEq/L). ECG as well as Cardiac enzymes were normal. CTPA done to rule out Pulmonary Embolism was also normal. No circulatory or ventilatory abnormality was found to explain cyanosis. Her cyanosis with normal PaO2 was the pointer to the diagnosis.
All her medical record along with history revealed, she has been taking Dapsone for treatment of her skin problem. This led to the final diagnosis of Dapsone induced Methemoglobinemia. Unfortunately MetHb level estimation is not possible in the country. She was treated with methylene blue and showed complete recovery. Methlene Blue was administered intravenously at dose of 1 mg/kg body weight. This dose was repeated once more and patient was later on discharged. She was doing fine on follow-up. |
pmc-6585050-1 | The patient was a 68-year-old woman with severe LBP, which had deteriorated over the past 5 years. She was unable to walk without support for < 100 m and experienced restrictions in her activities of daily living. Based on clinical symptoms and radiographic findings (Fig. a), adult spinal deformity (ASD) with sagittal imbalance was initially suspected, and corrective spinal fusion surgery, extending from the thoracic spine to the pelvis, was planned. No remarkable findings were observed in MR images (Fig. b). Bone SPECT/CT of the lumbar spine revealed a localized radiotracer uptake in the endplates of the L3/4 and L4/5 intervertebral disk (Fig. c). A discoblock of L3/4 and L4/5 dramatically relieved her LBP for several days. Based on this finding, the patient underwent an oblique lumbar interbody fusion of L3/4 and L4/5, with percutaneous pedicle screw fixation, without direct correction and decompression (Fig. d). The severity of her LBP was sufficiently relieved after surgery which improved her activities of daily living, which had been maintained for 3 years after surgery. |
pmc-6585050-2 | The patient was a 74-year-old woman with a degenerative kyphoscoliosis of the lumbar spine (Fig. a), who had been experiencing moderate LBP for several years. However, her LBP had gradually worsened over the past 2 months, without any trauma, to the point of limiting her activities of daily living. Based on findings on plain radiographs and CT and MR images, corrective spinal fusion surgery, from the thoracic spine to the pelvis, was planned. However, bone SPECT/CT of the lumbar spine revealed a localized radiotracer uptake in the lower endplate of L4 (Fig. b), indicative of a minor fracture. A retrospective review of MR images confirmed the presence of a low-intensity area in the lower endplate of L4 on T1-weighted images and a local high-intensity signal on T2-weighted images (Fig. c). These signal changes were difficult to differentiate from the degenerative changes and deformity of the lumbar spine. A lumbar orthosis was applied, with a decrease in LBP and healing of the endplate fracture, although the degenerative lumbar deformity remained, with persisting moderate LBP. |
pmc-6585050-3 | The patient was a 67-year-old woman who underwent a posterior lumbar interbody fusion of L3/4 at another hospital and who was treated using oral steroids for rheumatoid arthritis. She experienced a severe LBP that gradually worsened over the past 1 month, without any trauma. Based on findings on plain radiographs and CT and MR images, L2 spondylolisthesis with sagittal imbalance was identified (Fig. a), and extended spinal fusion surgery was considered. However, bone SPECT/CT of the lumbar spine showed a localized radiotracer uptake in the right transverse process of L3 (Fig. b). A retrospective review of CT images identified the non-traumatic insufficiency fracture of the right transverse process of L3 (Fig. c). The fracture was not identified with MR images (Fig. d). A successful decrease in LBP and healing of the endplate fracture was achieved with a conservative treatment using a lumbar orthosis. |
pmc-6585068-1 | A 33-year-old woman presented with complaints of progressive backache and lower limb aching 7 months after laparoscopic right ovarian cystectomy, the pathology of which proved to be mature teratoma. Sonographic examination revealed a mass in the right iliac. Single-photon emission computed tomography showed a malignant metastatic lesion in the hip bone, and pathological review of the first surgical section suggested the possibility of an immature teratoma. Despite 11 courses of cisplatin-based combination chemotherapy and two courses of radiotherapy, the disease progressed during treatment, with multiple metastases in the right iliac fossa, iliac, sacrum and liver on imaging. Biopsy of the liver lesion proved it to be a metastasis. Subsequent palliative radiotherapy did not relieve her pain. Palliative surgery was performed with partial hepatectomy of the left lobe of the liver and resections of the omentum and metastatic lesions. The final pathological exam showed metastatic PNET from the ovaries. The patient declined further treatment and died 2 years after the bone metastasis. |
pmc-6585068-2 | A 59-year-old woman had undergone exploratory laparotomy and pelvic mass resection with elevated CA-125 (105 U/ml). Pathology suggested a lipoid cell tumor. The pelvic mass recurred 3 months after the primary surgery, and a second laparotomy revealed multiple solid tumors in the pelvic and abdominal cavities. Tumor cytoreductive surgery was performed with total hysterectomy, bilateral salpingo-oophorectomy, appendectomy, infracolic omental excision, and metastatic lesion resection, with a residual tumor less than 1 cm. The final pathological diagnosis was primary ovarian PNET. Immunohistochemical (IHC) staining showed a positive reaction for (CD)-99, neuron-specific enolase (NSE), vimentin and smooth muscle actin (SMA) but was negative for synaptophysin, alpha fetoprotein (AFP), (EMA), calretinin, a-inhibin, Melan A, and desmin. The patient refused further adjuvant chemotherapy and was lost to follow-up. |
pmc-6585068-3 | A 67-year-old woman presented with abdominal distention and changes in bowel habits that had occurred over the previous 4 months. She had undergone transabdominal hysterectomy and left salpingo-oophorectomy for some benign diseases 2 years prior. Colonoscopy and colonography indicated sigmoid colon adhesions and stenosis (Fig. ). Ultrasonography revealed a smooth-surfaced pelvic mass of approximately 22 cm in diameter. Tumor marker analysis revealed elevated CA-125 (104.2 U/ml). Tumor cytoreductive surgery was performed with right salpingo-oophorectomy, appendectomy, infracolic omental excision, and partial excision of the sigmoid colon and small intestine. IHC analysis revealed positive staining for Ki-67 (labeling index, 50%), neurofilament (focal+), NSE, nestin, synaptophysin (focal+), P16 and P53 but was negative for CD-99, glial fibrillary acidic protein (GFAP), octamer-binding transcription factor (OCT) 3/4, S-100, estrogen receptor (ER), paired box gene 8 (PAX-8), progesterone receptor (PR), Wilms tumor (WT)-1, creatine kinase (CK)20, CK7 and calretinin (Fig. ). The final pathological diagnosis was primary ovarian PNET extending to the serosa of the rectum, sigmoid colon, appendix and small intestine as well as the lymph nodes around the colon. The disease was stage III according FIGO criteria []. The patient refused further treatment and died 6 months after her initial symptoms were observed. |
pmc-6585068-4 | A 14-year-old girl presented with abdominal distention for 2 weeks, which worsened over 1 week. Sonographic examination revealed a 30 cm × 20 cm pelvic mass with the possibility of ovarian origin. Abdominal and pelvic magnetic resonance imaging (MRI) revealed a large mass in the abdominal and pelvic cavity, which showed a low and intermediate T1 signal intensity and a high, heterogeneous T2 signal intensity, with good enhancement (Fig. ). Analysis of tumor markers revealed elevated CA-125 (473.0 U/ml), lactate dehydrogenase (LDH, 682 U/l) and NSE (18.2 ng/ml); levels of AFP, CA-199, CEA and β-human chorionic gonadotropin were normal. Laparotomy revealed a large solid mass, measuring approximately 30 cm in diameter and originating from the right ovary, and an omental mass measuring approximately 5 cm in diameter. Frozen tissue sections suggested a poorly differentiated carcinoma. Resection of the right annex, biopsy of the left ovary, omentectomy, and appendectomy were carried out, with a residual tumor less than 1 cm. IHC staining showed a positive reaction for CD99 but negativity for synaptophysin, calretinin, CD30 (Ki-1), CD10, cytokeratin (AE1/AE3), S-100 and vimentin. The final pathological diagnosis was primary ovarian PNET with diffuse metastasis to the omental and Douglas cavity. The disease was stage IIIC according to the FIGO criteria. Adjuvant chemotherapy was administered with paclitaxel (175 mg/m2) plus carboplatin (AUC 6). Contrast-enhanced computed tomography of the abdomen and pelvis revealed tumor recurrence during the course of chemotherapy. The patient died of disease progression; her overall survival (OS) time was 5 months. |
pmc-6585077-1 | A 22-year-old woman came to our department with complaints of intermittent palpitation and shortness of breath for 6 months. Physical examination was unremarkable. Echocardiography revealed that there was an ASD with the diameter of 10 mm. Enhanced computed tomography (CT) demonstrated a huge well-defined and homogeneous cystic mass, in left superior anterior mediastinum and in close proximity to pulmonary trunk (Fig. a and b), no significant artery feeding were found. Cancer antigen 125 was higher than normal. The uniportal video-assisted thoracoscopic surgerywas performed with the preoperative diagnosis of cystic teratoma. The first striking intraoperative finding was the completely CAP. A large well-defined cystic lesion (9 cm × 9 cm × 10 cm) was visualized, which bulged through the aortopulmonary window (Fig. ). We performed a mini-incision to cystic lesion, from which large amounts of white viscous liquid was sucked. Finally the basal segment of mass was separated carefully from mediastinal pleura (Fig. c). Besides, we did not do surgical intervention to CAP and ASD. Histopathology revealed that grossly, the lesion had a smooth inner wall with gray gelatinous material. And cystic dilated bronchus, lung tissue and its own pleura could be seen under microscope (Fig. d). The final diagnosis of this patient was ELS, not cystic teratoma. We reassessed the enhanced CT, but we still could not found a significant feeding artery for ELS.The patient recovered well and in excellent condition during follow-up,with no intermittent palpitation and shortness of breath. |
pmc-6585093-1 | A 91-year-old post-menopausal woman without diabetes mellitus or hypertension presented with shortness of breath, fever up to 38.5 degrees, anuria, and conscious disturbance for two days. Tracing back her history, she has dementia for 20 years with chronic kidney disease in stage 5, and she has not received any bowel or adnexal surgery. Last year, a transvaginal ultrasound had been performed by the gynecologist for a palpable pelvic mass, but only endometrial hyperplasia was impressed. Upon this admission, physical examination revealed a palpable mass as well, but there was no evident tenderness initially. Her body mass index was 23 Kg/m2. Laboratory test showed leukocytosis, azotemia with blood urea nitrogen 117 mg/dL, creatinine 12.9 mg/dL, C-reactive protein 26.2 mg/dL, procalcitonin 2.5 ng/mL, and pyuria. We initiated hemodialysis therapy for her uremia. Stool routine and culture showed negative results, indicating that colitis or gastrointestinal bleeding is less likely.
After two weeks of antibiotic treatment, leukocytosis, pyuria, and sepsis resolved, but intermittent fever lasted along with pelvic tenderness. We thus consulted the gynecologist again, who then arranged an urgent abdominal computed tomography (CT) because of the highly possible surgical requirement upon consultation. The CT scan disclosed the presence of a huge cystic mass 13.5 × 11.8 cm with internal septation and mural solid component without any obvious fat stranding at lower abdomen nor any evidence of acute colitis. The urinary bladder was compressed by it (Fig. ). No significant enlarged lymph nodes were found. Mucinous cystadenoma with ovarian torsion was suspected, and thus surgical intervention was arranged. During the surgery, a 12 × 10 × 10 cm right tubo-ovarian abscess with 800 mL of pus-like content was drained. Right salpingo-oophorectomy and pus culture were performed. The pathological examination showed ovarian tissue with acute and chronic inflammation, inflammatory exudate, and granulation tissue formation, which were compatible with that of a tubo-ovarian abscess, and its pus culture yielded Escherichia coli. Antibiotics were administered based on the culture sensitivity test, and her infection ultimately resolved thereafter. |
pmc-6585389-1 | A 52–54-year-old man experienced sudden severe headache accompanied by nausea and vomiting for 2 days, and had an mRS score of 1. CT performed at another hospital showed SAH (), and the patient was transferred to our hospital. MRI performed in our hospital showed a left brainstem infarction (). Based on the diagnostic criteria for VBD on CT and MRI (–), the neurologist and radiologist diagnosed this patient has having VBD. Given the complexity of the lesion, endovascular treatment using the overlapping stent technique was considered a feasible treatment modality. The intraprocedural DSA images confirmed the diagnosis of VBD (). Two low-profile visible intraluminal stents (4.5 × 30 mm and 5.5 × 25 mm) and two Solitaire® stents (both 6 × 30 mm) were inserted into the diseased artery (). DSA performed immediately post-operatively revealed good reconstruction of the basilar artery, and dense embolization with coils in the aneurysm. However, after inserting the stents, slow blood flow was observed in the basilar artery according to immediate post-procedural angiography. We immediately administered tirofiban, including a loading dose of 0.6 mg by intravenous injection and 0.25 mg/h by constant-rate intravenous infusion until noon of the second post-operative day. After undergoing treatment with tirofiban, blood flow velocity in the patient's basilar artery normalized. Following the endovascular procedure, this patient developed no new symptoms compared with his preoperative condition. During 6 months of post-treatment follow-up, his clinical symptoms completely resolved, providing an mRS score of 0. Follow-up imaging showed good reconstruction of the basilar artery and complete occlusion of the aneurysm on DSA (), and no change in the area of brainstem infarction on MRI () compared with preoperative MRI. |
pmc-6585389-2 | A 48–50-year-old man experienced dizziness and weakness in his limbs for 1 month, and had an mRS score of 2. MRI and DSA performed in our hospital validated the diagnosis of VBD involving the basilar artery (). We performed endovascular treatment with the overlapping stent technique to reconstruct the lumen of the basilar artery. Four Solitaire® stents (three 6 × 30 mm and one 6 × 20 mm) were inserted into the basilar artery. DSA performed immediately post-operatively revealed no change in the diameter of the basilar artery with obvious stasis of the contrast agent (). The patient underwent clinical and/or imaging follow-up examinations at 5, 17, 24, and 48 months after the procedure. At 5 months during follow-up, the patient underwent repeat DSA, which showed no change compared with immediately-post-operative angiography, and he had no obvious symptoms. At 17 months during follow-up, he underwent DSA again, which showed deterioration compared with the DSA performed 5 months after the procedure, although he continued to have no obvious symptoms. At 24 months during the follow-up, the patient presented with headache and diminished visual acuity. Follow-up CT at 5 months () and MRI at 24 months () demonstrated severe compression of the brainstem with dramatic progression. DSA follow-up at 24 months showed that the length and diameter of the diseased artery had deteriorated (). Relatives confirmed that this patient died from pneumonia secondary to severe brainstem compression 48 months post-operatively. |
pmc-6585389-3 | A 58–60-year-old man experienced intermittent dizziness for 1 month, accompanied by occasional nausea and vomiting. CT, MRI, and magnetic resonance angiography performed at a local hospital showed left cerebellar infarction, and VBD involving the left vertebral artery and basilar artery (). The patient had an mRS score of 1. After transferring to our hospital, the patient underwent DSA, which confirmed the diagnosis of VBD () and occlusion of the right vertebral artery. The neurologists and radiologists opted to perform endovascular treatment with the overlapping stent technique. Two PEDs (4.75 × 35 mm and 5.0 × 35 mm) were inserted into the basilar artery and left vertebral artery () and we used adjunctive coils to occlude the distal portion of the right vertebral artery. DSA performed immediately post-operatively showed good reconstruction of the diseased artery (). During 6 months of post-treatment follow-up, the patient had a good clinical outcome with an mRS score of 0. Imaging showed that the diseased arteries were well-reconstructed, with complete occlusion of the right vertebral artery (), and there was no change in the infarction area of the left cerebellum () compared with preoperative MRI and DSA. |
pmc-6585653-1 | A 72-year-old woman was admitted because of fecal occult blood and severe anemia. Colonoscopy revealed an advanced obstructive tumor in the sigmoid colon, which was diagnosed as a well-differentiated adenocarcinoma by biopsy. The patient had had no previous abdominal surgeries. Laboratory examination showed severe iron-deficiency anemia (hemoglobin, 4.7 g/dL), elevated serum carcinoembryonic antigen (16.0 ng/mL; reference range, 0–4 ng/mL), and normal carbohydrate antigen (CA19-9; 9 U/mL; reference range, 0–37 U/mL). Chest radiograph revealed dextrocardia and a right subphrenic gastric bubble (Figure ). CT confirmed SIT, showing complete transposition of the thoracic and abdominal viscera (Figure ). CT also revealed a tumor in the sigmoid colon, with no lymph node metastasis or distant metastasis (cT3, cN0, cM0 cStage IIA according to the UICC-TNM classification) . We determined that curative operation with laparoscopic sigmoidectomy was feasible.
For surgery, the patient was placed in the lithotomy position under general anesthesia. In a reversal of the normal setup, the operating surgeon and scopist were situated on the patient's left side, and the first assistant on the right. First, a lap protector mini (Hakkou Shoji, Nagano, Japan) was inserted through a 30-mm transumbilical incision. Next, an EZ access port (Hakkou Shoji) was mounted onto the lap protector mini, and a 12-mm camera port was placed through the EZ access port. For the operating surgeon, a 12-mm trocar was placed in the left iliac fossa and a 5-mm trocar in the left flank. For the first assistant, 5-mm trocars were placed in the right iliac fossa and the right flank (Figure ). Abdominal air pressure was set to 10 mmHg.
Via a medial approach, the sigmoid colon was mobilized with an ultrasonic harmonic scalpel. The right ureter was confirmed on the dorsal side. The inferior mesenteric artery was identified and isolated. The inferior mesenteric artery was clipped with endoscopic vascular clips and then divided (D3 lymph node dissection). Next, the inferior mesenteric vein was divided. After mesenteric treatment was performed on the anal side 10-cm from the tumor, detachable forceps were applied to the immediate side of the line to be dissected, and the intestinal tract was washed from the anus. The distal side was divided using an endoscopic linear stapler (Powered ECHELON FLEX® ENDOPATH Stapler; Ethicon, Tokyo, Japan), which was inserted from the 12-mm trocar in the left iliac fossa (Figure ). The pneumoperitoneum was stopped, and then the colon was divided at the proximal side of the tumor. After the pneumoperitoneum was restored, anastomosis was completed using a circular stapler (CDH29; Ethicon).
The operation time was 195 min with low blood loss. The postoperative course was uneventful, with no complications. Pathological examination revealed a well-differentiated adenocarcinoma: 55 × 45 mm, pT3 (SS), int, INFb, ly2, v0, PN0, pN0 (0/35), pPM0 (125 mm), pDM0 (105 mm), pRM0, pStage IIA. |
pmc-6586101-1 | A seven-month-old boy was referred for evaluation of a painless mass of the left wrist. His parents noticed a soft tissue mass 4–5 mm in diameter four months earlier with progressive enlargement. He had a benign birth history and no history of trauma, vascular access, or inherited vascular, collagen, or rheumatologic disorders.
On examination, the patient had a 1 cm bluish, non-tender, pulsatile mass over the left radial artery (). A Doppler-assisted Allen test raised concerns regarding the patency of the involved artery. Arterial duplex revealed a 9 × 9 mm suspected true aneurysm with normal flow distally. Magnetic resonance angiography (MRA) demonstrated a 1.1 × 0.8 × 1.0 cm mass arising off the radial artery with retrograde filling from the superficial palmar arch and thrombosis proximal to the lesion (). Surgical excision with possible reconstruction was recommended.
Intraoperatively, the arterial lesion was noted to be multi-lobular, focally bluish, and adhered to the flexor carpi radialis sheath, with the volar carpal branch of the radial artery exiting the mass. (). The radial artery was dissected to healthy vessel wall (proximally to the mid-forearm and distally past the wrist flexion crease). Trial clamping of the radial artery proximally and distally demonstrated maintenance of brisk distal capillary refill throughout, confirming a complete, patent arch. The radial artery was transected and the abnormal segment excised. Robust arterial backflow was noted from the distal stump of the radial artery following transection. Reconstruction with vein graft was not felt to be necessary and would additionally result in significantly prolonged anaesthesia time for such a young patient. The radial artery was therefore simply ligated.
On histopathology, the specimen exhibited medial attenuation with large areas of complete media loss, consistent with pseudoaneurysm (). Also noted was intimal fibroplasia with incorporating mural thrombus. These changes were noted to be most consistent with fibromuscular dysplasia of the medial type. The differential diagnosis included heritable connective tissue disorders such as type IV Ehlers-Danlos (EDS), Loeys-Dietz, and Marfan syndromes.
Given the abnormal pathology and young presentation age, the patient underwent a genetics workup to evaluate for possible connective tissue disorders. He was noted to have a large body habitus and hyperelastic skin, concerning for EDS. MRA exams of the carotids, brain, chest, abdomen, and pelvis, recommended by vascular surgery at the age of thirteen months to assess for any further large/medium vessel lesions, were unremarkable. A cardiovascular genetics workup of sixteen genes associated with EDS, Marfan, and familial thoracic aortic aneurysm and dissection (FTAAD) found no pathogenic variants.
At three years postoperatively, the patient used both hands symmetrically. There was no clinical evidence of growth arrest or cold intolerance. Ongoing monitoring for additional vascular abnormalities is planned. |
pmc-6586103-1 | A 60-year-old man presented for evaluation of new-onset thumb pain 4 months after trapeziectomy and carpal-metacarpal (CMC) arthroplasty with suspensionplasty. His primary complaint was a palpable, painful nodule at the ulnar base of the thumb. He experienced occasional shooting pain to the tip of the thumb while playing drums, as he was a semiprofessional drummer. Magnetic resonance imaging (MRI) was ordered, which showed only local soft tissue reaction, likely consistent with scar tissue or local thickening (). Surgical exploration revealed a thrombosed corkscrew ulnar digital artery () similar to vessel appearance in hypothenar hammer syndrome (HHS). A digital Allen's test was performed, which demonstrated vascular patency to the thumb. Excision without reconstruction was elected at this time. The segment that was resected was sent for permanent pathology in formalin (). The patient reported symptomatic relief, including while playing drums. |
pmc-6586447-1 | A 7-yr old girl was transferred to Mofid Hospital, ShahidBeheshti University of Medical Sciences, Tehran, Iran with limping and pain in lower extremities and acute paraplegia. She had no history of direct trauma to back, no previous disease, no medication and no family history of neurologic disorders.
In her history, she had a ring dancing just for one time, and no history of trauma. In physical examination, she was good in general appearance with BP: 90/60, HR: 80, RR: 20, T: 36.5 axillary. She was unable to bear weight. Deep Tendon Reflexes (DTR) in lower extremities had increased with extensor plantar response. Cranial nerve and cerebellar examination were normal. Laboratory tests include complete blood count (CBC), prothrombin time, partial thromboplastin time, biochemistry and inflammatory markers were normal. Her MRI showed spinal subdural hematoma extended from T2 to T6 vertebra (,). The patient underwent laminectomy from T2 to T5 and about 70 cc of subdural hematoma was evacuated. One month after the surgery, the patient's neurological deficit resolved completely. |
pmc-6586448-1 | A 14.5-year-old girl was referred to the Rheumatology Clinic, Mofid Children's Hospital, Tehran, Iran in Jan 2016 with a history of weight loss, photosensitivity and violaceous rashes on her upper eyelids about 6 months ago. She had no complaints of fever, weakness, malaise, myalgia or arthralgia. The informed consent was obtained from the parents of the girl.
She was first treated by a dermatologist with a course of topical betamethasone, intramuscular glucocorticoids, and chlorpheniramine, but there was no significant improvement. On physical examination, there were bilateral heliotrope rashes on her upper eyelids extended to the ears. Malar rashes on her cheeks were noted, connecting together on the nose. Erythematous papular lesions were seen on her anterior and posterior chest (V sign), anterior and posterior neck (shawl sign) and also on her abdomen and flanks. She showed violaceous papules on her interphalangeal joints (Gottron’s papules) and extensor aspect of her forearms. Nail folds changes with periungual erythema were prominent. On her palate erythema and petechia were revealed. Proximal and distal muscle strength of limbs were normal and no muscle tenderness was noted.
The laboratory tests showed a complete blood count with a microchrome microcytic anemia compatible with minor beta thalassemia. Blood Urea Nitrogen (BUN), creatinine (Cr), creatine phosphokinase (CPK), aldolase, lactate dehydrogenase (LDH), aspartate transaminase (AST), alanine transaminase (ALT), C3, C4, CH50, protein C and S were all within normal range. Fluorescent antinuclear antibodies(FANA), anti-cyclic citrullinated peptide (CCP), perinuclear- antineutrophil cytoplasmic antibodies (P-ANCA), cytoplasmic-antineutrophil cytoplasmic antibodies (C-ANCA), anti-double strand-DNA (ds-DNA), anti topoisomerase1 (SCL-70), anti jo1, anticentromere and anti-ribonucleoprotein (RNP) were negative.
Capillaroscopy of nail fold showed angiogenesis, avascular area, irregularly enlarged loops, isolated micro-bleeding, megcapillary, and tortuosity. Electromyography (EMG) and nerve conduction velocity (NCV) were normal. In abdominopelvic sonography, liver echogenicity was mildly increased suggesting of grade I fatty liver. MRI of the thigh was normal (). Skin biopsy of the trunk lesions reported as lichenoid tissue reaction with superficial perivascular dermatitis and dermal mucin deposition compatible with collagen vascular disease (). Muscle biopsy was taken from biceps muscle and neither inflammation nor perifasicular atrophy pattern was seen (). Cardiopulmonary investigation showed no abnormality on chest X-ray and echocardiography.
Treatment was started on Feb 2016 with ophthalmic ointment of hydrocortisone, hydroxychloroquine sulfate 6.5 mg/kg/d and prednisolone 1 mg/kg/d.
After 10 wk, all cutaneous findings were resolved; therefore, the dosage of prednisolone was gradually tapered to 0.25 mg/kg/d over 6 months. During 36 month’s follow-up (Feb 2019), the patient had no manifestations of muscular, cutaneous and cardiopulmonary involvement. |
pmc-6586455-1 | A 9-yr-old boy was referred to Loghman Hospital, Tehran, Iran, in 2018, with speech regression, seizure, and ataxia. He was developmentally normal until the age of 4 yr when his symptoms initiated with behavioral disturbances including attention deficit, auditory agnosia, decreased verbal communication, speech repetition, and IQ deterioration. He had first episodes of generalized tonic-clonic seizure with upward gaze and jaw locking during sleep and also intermittent urinary incontinence when he was 6 yr old. The child started symptoms of autism spectrum disorder such as echolalia, poor eye to eye contact and repetitive behavior.
On neurological examination, the patient had no focal signs. Motor and sensory examination of four extremities were intact. Primary laboratory studies included blood cell count (CBC), renal and liver function tests were normal. CSF examination was within normal limit. The child had normal brain MRI and auditory brainstem response (ABR). Brain perfusion SPECT showed decreased perfusion in left centrotemporal lobe (). Amplitude EEG showed bilateral synchronous spike-wave complex in the temporal regions ().
On the basis of presentation, EEG and SPECT study, LKS diagnosis was confirmed and methylprednisolone, valproate sodium, levetiracetam, IVIG and speech therapy were initiated; however, the patient continued generalized tonic-clonic seizure in sleep, severe behavioral disorders and aphasia. Due to poor response to treatment, symptoms progression and relative parents, whole exome sequencing was performed, which revealed mutation in exon 5 of NAGLU gene suggesting MPS IIIB. Although the patient had not any sign of coarse face or dysostosis multiplex (), high urinary glycosaminoglycan excretion confirmed MPS III diagnosis.
Informed consent was taken from patients parents.
The study was approved by the Ethics Committee of Shahid Beheshti University of Medical Sciences, Tehran, Iran. |
pmc-6586471-1 | A 6-year-old boy presented with a Gustilo type IIIB open fracture with a segmental bone loss in his left tibia. His left leg was struck by the tire of an automobile. The boy's attendants brought two bone fragments recovered from the scene of the accident. The extruded bones were a segment with a length of 5.5 cm and a cortical bone with a length of 4 cm. The periosteum was stripped from the fragments, but the neurovascular structures of the left leg were intact (
).
The bone fragments were structurally important. In the operating room, a thorough and meticulous irrigation and a debridement of the bone and the soft tissues of the open tibia fracture were performed in accordance with the principles of open fracture management. There were a well-preserved periosteum and soft tissue envelope around the bone defect.
The two extruded tibial fragments were cleaned and washed with copious amount of normal saline to remove the gross contaminations. The bone fragments were then scrubbed with 10% povidone–iodine for 20 minutes followed by 20-minute soaking in 2% chlorhexidine gluconate solution. They were then rinsed with normal saline before the reimplantation in the defect area (
). The leg was immobilized by a long leg cast, and intravenous cefazolin and gentamicin were administered for 7 days. His postoperative course was uneventful. After 3 months, the patient was able to bear his complete weight and walk without aids, and the knee and ankle joints' range of motions were normal.
and
demonstrate complete union of the fracture in appropriate alignment after 4 months. The extruded fragment was incorporated into the callus of the fracture site. |
pmc-6586731-1 | The patient was a 60-year-old man. Abdominal ultrasonography indicated that he had an abdominal mass. He reported having perceived lower abdominal pain 7 months before the initial examination, but it spontaneously remitted. The patient had no fever nor any history of conditions such as hypertension. A blood test revealed that he did not have anemia, thrombocytopenia, or coagulation abnormalities. The patient demonstrated slightly elevated levels of carcinoembryonic antigen (4.7 ng/ml) and soluble interleukin-2 receptor (603 U/ml), tumor markers that are elevated in gastrointestinal cancers and malignant lymphoma. Contrast-enhanced CT showed a 33× 31 mm abdominal aortic aneurysm and a 106 × 81 mm mass peripheral to the abdominal aorta. The mass did not feature a contrast effect, and its margin was enhanced in the venous phase (Fig. ). Sigmoid wall thickening and inferior vena cava thrombosis were also observed (Fig. ). We suspected the patient had a neoplastic disease, such as a malignant lymphoma or sigmoid colon cancer. Detailed examinations were consequently performed, and direct oral anticoagulant therapy was initiated to treat the inferior vena cava thrombosis.
The FDG-PET revealed an abnormal accumulation of FDG in the margin of the mass and the sigmoid colon, but not in any other organs. Colonoscopy showed only mild inflammation in the sigmoid colon, and a sigmoid colon biopsy revealed no significant findings. Chronic inflamed fibrous connective tissue was harvested from the periaortic mass using CT-guided biopsy. There were no findings indicative of infection or neoplastic disease, thus precluding a pathological diagnosis.
At 1 month after the initial examination, intermittent claudication occurred, prompting the performance of follow-up contrast-enhanced CT scanning. On CT scanning, we found an arterial occlusion from the infrarenal abdominal aorta to the bilateral common iliac arteries, the right external iliac artery, and the right internal iliac artery (Fig. ). These findings indicated the potential progress of the tumor to the aorta. Via a midline incision, biopsies of the mass and the lymph nodes peripheral to the sigmoid colon were obtained. However, these biopsies showed only connective tissue with inflammation and fibrosis and did not yield any significant findings. Since the developments, we elected to resect the mass and perform bypass surgery for definitive diagnosis and treatment after 3 weeks from biopsy.
Surgery was performed using a transperitoneal approach via a midline incision. Adhesion was observed peripheral to the abdominal aorta, the bilateral iliac arteries, and the retroperitoneal mass. The abdominal aortic lumen was completely occluded by an old thrombus and plaque. Removal of the thrombus revealed a 35-mm intimal defect consistent with the direction of the retroperitoneal mass (Fig. ). Communication was observed between the intimal defect site and the retroperitoneal mass, which was filled with a partially organized old thrombus. During the intraoperative rapid pathological examination, the tissue inside the mass was diagnosed as a fibrin clot. The tumor tissue was not assessed. The area from the infrarenal abdominal aorta to the right common femoral artery and the left common iliac artery was bypassed using a Y-shaped woven Dacron graft. Pathologic examination showed that the mass tissue was a partially organized fibrin clot, thus agreeing with our intraoperative rapid diagnosis. On the basis of the intraoperative and pathologic findings, the patient was diagnosed with CCR-AAA with abdominal aortic occlusion. His intermittent claudication improved, and he was discharged on day 19 after surgery. Six months after the operation, the retroperitoneal mass disappeared on CT. |
pmc-6586734-1 | A 55-year-old female underwent total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and partial transverse colectomy for advanced left ovarian cancer 2 years ago. She was diagnosed with ovarian serous adenocarcinoma and multiple peritoneal metastases based on operative and pathological findings (International Federation of Gynecology and Obstetrics: FIGO stage IIIc). After the operation, she received chemotherapy with paclitaxel and carboplatin, which is regarded as the standard therapy for advanced ovarian cancer, against the residual peritoneal metastases for 8 months. After the chemotherapy, she achieved a clinical complete response on abdominal computed tomography (CT). In addition, the serum tumor marker cancer antigen 125 (CA 125) level, which was elevated to 274.3 U/ml before the treatment, decreased to the normal range.
Two years after the initial surgical treatment for the ovarian cancer, she complained of dark urine, yellowish discoloration of the eyes, and loss of body weight. The serum CA125 level increased to 50.9 U/ml. Carbohydrate antigen (CA) 19–9 and carcinoembryonic antigen (CEA) levels were normal. The total bilirubin level was elevated at 10.4 mg/dl. Abdominal CT showed intrahepatic bile duct dilatation due to an approximately 10-mm solid tumor at the hepatic hilum (Fig. ). Simultaneously, several irregular peritoneal nodules (less than 15 mm in size) were detected in the pelvic cavity. Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed uptake in the hepatic hilum tumor (standardized uptake value (SUV) max 8.2) and the pelvic nodules (SUV max 4.7). Contrast-enhanced magnetic resonance imaging (MRI) findings also showed a high-attenuation intraductal mass at the hepatic hilum and dilation of the intrahepatic bile duct (Fig. ). Endoscopic retrograde cholangiography (ERC) revealed the defect at the bifurcation of the common hepatic duct (Bismuth-Corlette classification type II) and endoscopic nasobiliary drainage was performed for obstructive jaundice (Fig. ). Intraductal ultrasonography (IDUS) suggested the tumor was located in the bile duct wall (Fig. ). Tumor biopsy and brush cytology of the bile duct indicated atypical cells suspicious for carcinoma; however, a definite histopathological diagnosis could not be obtained because of the limited biopsy samples. Preoperatively, she was diagnosed with a hepato-hilar bile duct tumor, concurrent with recurrent peritoneal metastasis of ovarian cancer. The recurrent peritoneal dissemination was regarded to be controllable by paclitaxel and carboplatin, because it developed 14 months after stopping chemotherapy. Curative resection was suggested for treatment of the hepato-hilar bile duct tumor, because a definite histopathological diagnosis was not obtained and primary cholangiocarcinoma could not be ruled out. The abdominal CT demonstrated that the tumor was in contact with the right hepatic artery, suggesting arterial infiltration. We planned a right hepatectomy and performed preoperative percutaneous transhepatic portal embolization (PTPE). The CT volumetry 3 weeks after the PTPE demonstrated that the volume of the left lobe increased from 390 to 511 ml. She underwent right hepatectomy with caudate lobectomy and extrahepatic bile duct resection.
Gross description indicated that the tumor was at the bifurcation of the common hepatic duct (Fig. ). Histopathologically, tumor cells did not invade the liver parenchyma (Fig. a, b). The tumor was localized in the biliary wall and extended to the subserosal layer of the hepatoduodenal ligament, whereas direct exposure to the hepatoduodenal ligament was not detected. Dysplastic cells formed tubular structures and showed nuclear hyperchromasia. Psammoma bodies, which are small calcifications, were seen in the tumor. The histopathological features were similar to the pathology of the primary ovarian cancer. In the native biliary epithelium, no areas resembling dysplasia were seen. Peritoneal washing cytology suggested a serous adenocarcinoma. Immunohistochemical staining revealed that the tumor cells were positive for cytokeratin-7 (CK-7) and Wilms tumor-1 (WT-1) and negative for cytokeratin-20 (CK-20) and estrogen receptor (ER) (Fig. c–e). These findings suggested that the biliary tumor was derived from the ovarian cancer. Peritoneal metastases were scattered around the peritoneum, greater omentum, and diaphragm. The final diagnosis was an ovarian cancer-associated metastasis to the bile duct and recurrence of lymph node and peritoneal metastasis of ovarian cancer.
Her postoperative course was unremarkable. According to the chemotherapy sensitivity test, she received chemotherapy with paclitaxel against the residual peritoneal metastases for 7 months and achieved a clinical complete response. However, progression of the peritoneal dissemination and lymph node metastasis was confirmed 28 months after the operation. |
pmc-6586919-1 | We report on a 20- year-old healthy male, who presented at the surgical emergency department complaining about pain at the left forearm and wrist ensuing a fall from his motorcycle without third party interference. The patient reported of an initial driving speed of 10–20 mph. Helmet and protective clothing was worn.
Due to the anamnestic information and clinical examination an x-ray of the left hand and forearm was performed. It showed no pathologic results ().
With respect to the clinical suspicion of an osseous injury to the left wrist, positive fovea ulnaris sign and Triangular Fibro- Cartilage Complex (TFCC) load test, an additional computed-tomography was initiated. It revealed the diagnosis of a distal, non-displaced, intraarticular ulna fracture with involvement of the ulnocarpal and the radioulnar joint (, ).
The patient got an outpatient treatment immobilized by an intrinsic plus forearm cast and an appointment for a clinical 1- week follow-up was scheduled.
By this point, local examination showed a moderate swelling of the wrist. He described slight pain of the ulnar wrist and forearm. Due to this, the pronation and ulnar deviation was compromised. The patient was treated with a closed forearm brace for another 4 weeks under avoidance of supination and pronation.
He was seen again 5 weeks after trauma denying any pain. Neither swelling, nor instability of the DRUJ was eminent. Painless range of motion with solely terminal limitations (pro-/ supination 85/0/85°, dorsal extension/ palmar flexion 35/0/50°, radial abduction/ ulnar abduction 25/0/30°). A performed x-ray of the left wrist showed no secondary dislocation. The patient was discharged with the proviso of full weight bearing and free range of motion.
Following a planned outpatient MRI of the wrist a 10- week follow- up of the patient showed no significant changes of the results. The MRI revealed the integrity of the TFCC and an osseous consolidation of the fracture with a persistent bone marrow edema (). No further follow-up has been scheduled. |
pmc-6587240-1 | A 74-year-old indigenous Malaysian man, an ex-smoker of tobacco, with underlying severe aortic stenosis, atrial fibrillation, hypertension, and chronic kidney disease presented to us with a 6-month history of lethargy, subjective loss of weight, loss of appetite, and night sweats associated with a sharp, persistent right-sided headache and left hip pain. He denied any pruritus. He denied chronic cough, and had no significant travel history or high risk behavior. He had no family history of malignancy. He had no baseline ultrasound of the genitourinary tract, having refused investigation of his chronic kidney disease previously.
Prior to this current admission, he had been admitted two times over the past 4 months for hyponatremia and normochromic normocytic anemia, with initial serum sodium measuring 120 mmol/L and 124 mmol/L, respectively. Peripheral blood film showed features suggestive of iron deficiency anemia; concurrent with a serum iron level of 9.7 umol/L with calculated transferrin saturation of 21.2%. Ferritin and B12 levels were normal while a fecal occult blood test was negative. On both occasions, he received intravenously administered saline and subsequently was discharged with orally administered sodium supplements. Tests for thyroid function and cortisol levels from a previous admission were normal.
On admission, he was afebrile with a blood pressure of 130/70 and pulse rate of 70. An examination revealed a mildly cachexic man with generalized disuse atrophy of all limbs. Functionally, he was unable to ambulate due to left hip pain. There was tenderness at his left hip with reduced passive and active movement due to pain, and a bony protuberance at the posterolateral aspect of the right side of his scalp. There were no neurological deficits, and respiratory and abdominal examinations were unremarkable.
On examination of the skin, there were diffuse brown well-circumscribed pigmented lesions of undetermined onset over his face and trunk suggestive of multiple seborrheic keratoses. The lesions over his posterior trunk were distributed in a symmetrical “Christmas-tree” pattern (Fig. ).
An electrolyte investigation showed low serum sodium of 119 mmol/L. All other electrolytes were within normal range, and there was no derangement of liver enzymes. A chest X-ray on admission showed bilateral multiple irregular nodules and prominent hilar opacities (Fig. ). In view of long-standing headache, we proceeded with non-contrasted computed tomography of his brain, which revealed a well-defined round hyperdense lesion at the right high frontal cortex measuring 0.7 × 0.7 cm, associated with perilesional edema (Fig. ). There was a lytic lesion at the right parietal bone with expansile soft tissue component (Fig. ). Resource limitations precluded a follow-on magnetic resonance imaging (MRI). Computed tomography of his thorax, abdomen, and pelvis revealed a heterogeneously enhancing soft tissue mass at the right kidney measuring 3.8 × 4.4 × 3.7 cm, with no evidence of obstruction or hydronephrosis (Fig. ). There was evidence of bilateral metastatic lung nodules, and multiple hilar and paratracheal nodes (Fig. ). There was no evidence of aortic aneurysm. There was also a 5 × 5cm lytic lesion at the left ilium, consistent with his left hip pain (Fig. ).
Further biochemical investigation showed increased urine osmolarity of 303 mOsmol/kg and urine sodium of 48 mmol/L; in view of prior normal thyroid function, cortisol level, and clinical euvolemia, we diagnosed him as having syndrome of inappropriate antidiuretic hormone secretion (SIADH). Fluid restriction was commenced and his sodium levels recovered to 131 mmol/L over 5 days. He was referred to the Dermatology team; in view of the findings of metastatic renal carcinoma, the skin lesions were attributed to Leser–Trélat syndrome.
Having been thoroughly counselled on his condition, he refused renal and skin biopsy and was not keen for further intervention. Despite the absence of a histopathological diagnosis, the constellation of clinical and radiological features was suggestive of a renal malignancy with metastasis, and he was referred to the Palliative Care team for further management. |
pmc-6587250-1 | A 28 year-old male was found to have a slightly increased serum creatinine (1.7 mg/dl) during the admission to ER because of abdominal pain. Since both his past medical history and an abdominal ultrasound performed on admission were unremarkable he was discharged with the indication to see a Nephrologist. At the Nephrology outpatient clinic, because of the reduced eGFR and of the presence of proteinuria (300 mg/24 h), an hospitalization for further investigations was planned.
While waiting for the admission, a couple of weeks later, the patient was admitted to the emergency room in another hospital for abdominal pain and dyspnea. On admittance the ECG tracing performed showed sinus rhythm with diffuse repolarization abnormalities. Further exams revealed an increase of troponin (54 ng/ml) and transaminases levels (GOT 50 U/L; GPT 125 U/L) and a cardiac ultrasound showed a severe left ventricular dysfunction with right heart failure. Patient was thus admitted to the coronary intensive care unit. During his hospital stay the possibility of acute myocarditis was excluded because of the absence of a recent viral syndrome and the negativity of IgM antibodies against the viruses most commonly affecting the cardiovascular system and because of the low inflammatory indexes. An abdominal ultrasound demonstrated abdominal and pleural effusions, regular size and morphology of spleen and liver, no pancreatic changes; both kidneys had normal size but showed irregular margins and a reduced thickness of the cortex, no hydronephrosis nor stones were observed. A magnetic resonance demonstrated an increased volume of the cardiac chambers with a severe reduction of the function of both ventricles (EF20%), however no signs of reduced myocardial perfusion, valvular defects or other tissue changes such as inflammation, fibrosis or edema could be demonstrated. A chest CT scan confirmed the presence of pleural effusion and showed parenchymal atelectasias mainly involving the lower lobes of the lungs. The patient was discharged after 11 days on diuretics (furosemide 100 mg/daily), B-blocker (carvedilol 37.5 mg/daily), acetyl salicylic acid (Aspirin 100 mg/day) and an Ace inhibitor (Ramipril 5 mg/day) with a diagnosis of dilated cardiomyopathy and severe left ventricular dysfunction complicated with cardiogenic shock and anasarca. Few days later an EKG ergometric test revealed no areas of inducible ischemia. A further admission to the Cardiology Unit to define the effect of treatment was performed a week later. Cardiac ultrasound confirmed the left ventricular dysfunction (EF 23%), a coronary angiography was negative and a right chamber catheterism and endomyocardic biopsy demonstrated no specific lesion nor inflammatory infiltrate. The patient was then admitted to our Nephrology Unit, as planned, to evaluate the cause of the chronic kidney disease. On admission the patient appeared healthy and well oriented, his blood pressure was 110/60 mmHg, heart rate 56 bpm, physical examination was unremarkable except for a 1/6 intensity systolic heart murmur better audible on mitral valve area. ECG tracing showed sinus bradycardia. Laboratory studies revealed an increased serum creatinine (1.87 mg/dl), eGFR 48 ml/min/1.73 m2, hypercalcemia (serum calcium 11.5 mg/dl), hemoglobin 15.1 g/dl, sodium 135 mEq/L, potassium 4.37 mEq/L, phosphate 3.1 mg/dl, serum albumin 4.07 g/dl. The patient denied use of vitamin D analogs thus in consideration of the unexplained hypercalcemia other exams were performed. Thyroid hormones, calcitonin, vitamin D, serum ACE, phosphate and calcium urinary excretion rate were all within the normal range. PTH was lower than 3 pg/ml. To rule out multiple myeloma serum protein electrophoresis, serum and urine immunofixation, and spine and skull x-ray were performed and did not show any pathological changes.
Renal biopsy was finally performed without complications and the patient was discharged after 24 h. While waiting for the renal biopsy pathology report, medications were not modified, but for an increase of the dose of furosemide because of the hypercalcemia. The renal biopsy contained eight glomeruli none of which was globally or partially sclerotic and no pathological changes were observed at glomerular level, vessels were also unremarkable. Tubulointerstitium was the only affected compartment with a moderate to severe inflammatory infiltrate, some calcium crystals in the tubular lumen and two granulomas with multinucleated giant cells (Figs. and ). No necrosis could be observed within the granulomas. A diagnosis of granulomatous interstitial nephritis due to sarcoidosis was made.
When the patient was called to discuss his kidney biopsy, his serum creatinine had further increased (3.5 mg /dl), eGFR 22 ml/min/1.73 m2 and so had his serum calcium (13.3 mg/dl), serum albumin 4.06 g/dl. For this reason, he was hospitalized again and treated with pulse methylprednisolone (250 mg/day for three days) and then with maintenance dose prednisone (50 mg/day). Serum creatinine decreased in a few days and so did his serum calcium. Since the episode of acute kidney injury the patient has been followed at our unit and at the unit of Cardiology. Renal function returned to normal (serum creatinine 1.1 mg/dl, eGFR 91 ml/min/1.73 m2) and heart function markedly improved over the following two years (EF 49% vs 37% vs 32% vs 23%). The diuretic and cardiologic therapy was significantly reduced to only 25 mg of furosemide and a small dose of beta blocker. The steroid was gradually tapered to 5 mg/day of prednisone for one year and then stopped. |
pmc-6587272-1 | A 39-year-old man visited the dental clinic due to the evolution of his worn teeth, in particular the maxillary incisors. His medical history revealed massive soft drink consumption. Clinical and radiological examinations showed the presence of amalgam at the maxillary right first molar, and resin filling with carious recurrence at the maxillary right first and second molars. Moreover, the mandibular left first molar was subject to carious recurrence below its metal-ceramic crown, requiring its reconstruction (Fig. ). The loss of non-carious substance affects the maxillary incisors, canines and pre-molars. Severe erosion of type “grade 2” was detectible, with the loss of enamel and dentin surface exposure. The occlusion study underlined that the prosthetic space left by substance losses was not sufficient to obtain the necessary thickness of reconstitution materials. The examination showed no pain during temporomandibular joint and muscular palpation. The patient didn’t report elements suggestive of bruxism. However, the necessary increase in the OVD was estimated at 1 mm inter-incisal. Facial and dental aesthetic analysis revealed no facial asymmetry and no deviation of horizontal facial lines. Analysis of the shape of the maxillary incisors confirmed the loss of substance at the free edge of the incisors and canines, which was the cause of the disturbances of the curvature of the aesthetic frontal curve (Fig. ).
In this clinical context, the complete rehabilitation of the mouth was proposed to this patient. After discussion with the patient, the decision was made to take a very conservative and reasoned approach to the treatment. The integrity of the residual dental tissues was preserved using adhesive techniques favouring minimalist preparations and restoring the substance losses, by adding the materials composing the restorations []. The increase in the OVD to 1 mm was stabilized by the restorations and by passive dental overeruption [, ]. All these treatments were to be accompanied by stopping soft drink consumption.
The treatment plan was performed in three steps. The first clinical appointment was dedicated to maxillary and mandibular impressions (Hydrocolor 5; Zhermack, Badia Polesine, Italy) and the facial arc (Facial arc Quick; FAG Dentaire, France). The increase of the OVD was obtained on the semi-adjustable articulator (Quick Master 1000 B2; FAG Dentaire, France) and a bite wax recorded in centric relation. The first laboratory steps consisted in also making a wax-up of the OVD at + 1 mm on the anterior guidance pin. The technician performed a full-mouth wax-up in three steps (premolar/molar sector right and left; incisor-canine sector), excluding the second and third molars. For each wax-up step, a silicone key (Zetalabor and Zetaflow; Zhermack, Badia Polesine, Italy) was fabricated (Fig. ). Thus, a total of three-silicone keys per arch were prepared. This technique ensured optimal repositioning and thus high fidelity with the wax-up. On the second clinical appointment, each silicon key loaded with Bis-Acryl-resin (Luxatemp Star, DMG Fabrik GmbH, Germany) was positioned in the patient’s mouth to build the full mock-up. This Bis-Acryl-resin presented a high breaking and bending tolerances increasing mock-up’s clinical survival No teeth were touched during this clinical visit. Only etching points (Gel Etchant; Kerr Dental, California USA) were applied to obtain better adhesion and durability of the mock-up (Fig. ). A second mock-up visit enabled clinical validation of the aesthetic, phonation and occlusal functions (Fig. ). The patient and his family validated the new incisor morphology. Anterior guidance, teeth occlusion and the increase of the OVD were confirmed by the integrity of the mock-up and the conservation of the occlusal contact points. The absence of the mock-up at the level of the second and third molars caused an inocclusion that was compensated by teeth overeruption after 3 months of wearing. Once the new occlusion was confirmed, mock-up impressions were made to prepare a personalized acrylic resin incisor table (Pattern Resin LS; GC Corporation, Tokyo Japan) on the articulator (Fig. a). In this temporary time, patient involved a regular oral health check-up to certify the good dental and temporomandibular health.
The second step started by restoring the posterior teeth, notably by the left mandibular quadrant. After endodontic treatment, an inlay-core was cemented on the mandibular left first molar. The mandibular left second premolar was restored with resin composite filling (Tetric EvoCeram; Ivoclar Vivadent AG, Schaan, Liechtenstein). Then, the final preparation of the molar was performed and impressions were made with a two-step putty-wash technique using addition silicone impression material (Express 2 PentaPutti and Express 2 Ultra-light BodyQuick; 3 M Espe™, St Paul, Minnesota USA) and casts were poured using type IV hard stone (Fujirock EP; GC Corporation, Tokyo Japan). The provisional crown was produced using a silicone key made from an intra-oral mock-up impression. The working casts were articulated and definitive restoration was completed on the model from a base metal alloy (Colado CC; Ivoclar Vivadent AG, Schaan, Liechtenstein) and cosmetic porcelain (VITA VM 13; VITA Zahnfabrik, Bad Sackingen, Germany). Then, resin composite restorations were performed on the right mandibular first and second molars. The two maxillary quadrants were produced in the same way, meaning that the onlay preparation impression was made after preparing the cavity and the resin composite on the adjacent teeth. The ceramic onlay (Emax CAD; Ivoclar Vivadent AG, Schaan, Liechtenstein) was bonded on the left maxillary first premolar (Fig. b) and the right maxillary first molar (Fig. c) (Multilink Automix; Ivoclar Vivadent AG, Schaan, Liechtenstein). Previously, immediate dentin sealing had been placed on the prepared dental surfaces, and the resin composite fillings were placed on the right maxillary first and second premolars and the left maxillary second premolar. The set of adhesive restorations was carried out using a dental dam to ensure the best conditions of isolation (Nic Tone rubber dam; SC expert Tech Solutions SRL, Romania).
The third step consisted of functional prosthetic rehabilitation with palatal veneers and aesthetic restoration of the incisor edge with aesthetic resin composite. The preparations for palatal veneers consisted of simple conditioning of the dental tissue left by erosion. The working casts were articulated, and the incisor table resulted in the manufacture of the palatal restorations with CAD/CAM resin-composite blocks (Lava Ultimate; 3 M Espe™, St Paul, Minnesota USA) and a CAD/CAM system (Fig. ). The restoration design presented a portion resting on the incisor edge to guide correct positioning during bonding (Multilink Automix; Ivoclar Vivadent AG, Schaan, Liechtenstein). This part was then removed to make room for the aesthetic composite (Estelite Asteria; Tokuyama Dental Corporation, Tokyo, Japan) (Fig. ). After these treatments, the patient was asked to wear a lower jaw gutter to protect the restorations. The patient acknowledged having improved function and aesthetics and was pleased with the results. Routine clinical assessments were made at 4 months and then at 6 months with visual, photographic and radiographic examinations. |
pmc-6587290-1 | A 52-year-old woman was admitted to our hospital, suffering from chronic lumbar pain for ten years and aggravating after physical labour. 4 months ago, she experienced violent low back pain and numbness of left leg, radiating from left hip to the low extremity with no obvious predisposing cause. Physical examination showed tenderness of interspinal areas in the low lumbar and hypesthesia to pinprick in the L5 and S1 dermatomal distribution, as well as positive straight leg raising sign. Routine spinal X-rays showed L4 spondylolisthesis and spondylolysis in both sides. CT scan reconfirmed L4 spondylolisthesis and spondylolysis in both sides and showed evidence of disc protrusion and lumbar stenosis at L4/5 and L5/S1, compressing left L5 and S1 root (Fig. ).
The patient subsequently took a posterior lumbar decompression surgery at L4-S1, internal fixation and intervertebral fusion at L4/5 and L5/S1. Satisfied outcome was achieved and the patient got out of our hospital at the 6th day. Routine X-ray test was taken before her discharge and showed internal fixations were in good places, but with a black shadow at the surgical place. 10 days later, the patient had an uncontrolled lumbar sprain and began to have dysfunction of excretion. She was readmitted to our hospital. CT and MRI were taken and gas pseudocyst was detected, which compressed the dura sac (Fig. ). Laboratory studies before the revision surgery were taken to rule out the possibility of infection. Debridement surgery was taken. During the surgical procedure, a sound of rushing air was heard when the deep fascia and muscle were opened through the previous route. A thin, blister-like membranous structure surrounded with little clot organization were seen in the surgical field, significant compressing the dural sac with no sign of infection. The membranous structure and clot organization were removed until dura sac were decompressed and returned to throb. Isotonic saline and diluted iodophor solution were used to irrigate surgical field and intradiscal space at the last stage of the operation to prevent persistence of air and infection in spinal canal. Her symptoms disappeared soon after the surgery. Routine X-rays were repeated 2 weeks after debridement, and gas gap disappeared with no signs of infection (Fig. ). |
pmc-6587381-1 | A 70-year-old white male with past medical history significant for cirrhosis secondary to nonalcoholic steatohepatitis, portal hypertension status post transjugular intrahepatic portosystemic shunt, portal vein thrombosis on warfarin, hypertension, and hypothyroidism presented to the hospital with chief complaint of hematuria. On admission, his blood pressure was 125/57 mm Hg, heart rate was 55 beats per minute, and temperature was 37.5°C. Laboratory studies demonstrated a blood urea nitrogen/creatinine ratio of 41/4.49 (baseline ~1.38), hemoglobin 10.9 g/dL, white blood cell count 6 K/UL, platelets 120 K/UL, and INR 8.7. Urinalysis was remarkable for too numerous to count RBCs, 20 to 50 white blood cells, large leukocyte esterase, and 3+ protein. A review of the sediment showed numerous RBCs and hyaline casts without any dysmorphic RBCs. Renal ultrasound revealed right kidney measuring 12.2 cm and left kidney measuring 12.6 cm with normal cortical thickness, no evidence of hydronephrosis, and mildly increased echogenicity bilaterally. Given the supratherapeutic INR and ongoing hematuria, warfarin was held and treatment was initiated with intravenous fluids and antibiotics for possible urinary tract infection. Urine cultures were pending.
Secondary workup for the etiology of AKI revealed 2.8 g of proteinuria on a 24-hour sample and a positive antinuclear antibody at 1:80 dilution. Acute hepatitis panel, double-stranded deoxyribonucleic acid, rapid plasma reagin, rheumatoid factor, complement levels (C3 and C4), and antineutrophil cytoplasmic antibody profile were all negative. Serum protein electrophoresis and serum immunofixation demonstrated a polyclonal IgA. Serum-free light chains ratio was 1:2. Urine cultures were consistent with pan-sensitive Escherichia coli. Despite conservative measures, the patient’s renal function continued to worsen with peak creatinine of 8.6 mg/dL, and a renal biopsy was performed. Light microscopy demonstrated sclerotic glomeruli with mesangial expansion and accumulation of red cells within Bowman’s space. Tubular cross section demonstrated RBC casts. There was no necrosis or crescent formation (). Electron microscopy (EM) demonstrated scattered mesangial and subendothelial immune complex deposits staining positive for IgA. The patient was diagnosed with ARN superimposed on sclerosing IgA nephropathy. He required 5 sessions of hemodialysis prior to discharge. Later, his creatinine stabilized to 2.5 mg/dL and proteinuria improved from 2.8 g to 1.1 g at a follow-up visit in the clinic.
Up to 5 glomeruli, 2 of which are globally sclerotic, are identified per level. The glomeruli show mesangial expansion. One glomerulus shows segmental sclerosis. One intact glomerulus shows accumulation of red cells within Bowman’s space. No necrosis or crescent formation is identified. There is mild interstitial fibrosis. Occasional tubular cross sections show red cell casts while other tubules show granular casts. Limited sampling of the vasculature did not reveal any abnormalities. The biopsy examined via immunofluorescence consists of 2 tissue fragments containing renal cortex. Up to 19 glomeruli with no globally sclerotic glomeruli are identified per level. IgA and C3 are identified within the glomeruli as listed below. By immunofluorescence, the immune complexes appear to be mesangial and along the capillary loops. Ultrastructural evaluation reveals scattered mesangial and occasional subendothelial immune complex-type dense deposits ().
The glomeruli show mesangial expansion with segmental sclerosis. Ultrastructural evaluation reveals scattered mesangial immune complex-type dense deposits and mesangial expansion. There are occasional subendothelial immune complex-type dense deposits. The podocytes are hypertrophic with diffuse foot process effacement. |
pmc-6587385-1 | A 63-year-old man with chronic genotype-1A HCV infection complicated by compensated cirrhosis (Child-Turcotte-Pugh Score A [CTP-A], Model for End-Stage Liver Disease [MELD] 6) with radiographic and laboratory evidence of portal hypertension was evaluated for HCV treatment. He previously had been treated with multiple courses of interferon and ribavirin with end-of-treatment responses but subsequent relapses. He also had been treated with interferon, ribavirin, and a protease inhibitor, but discontinued the medications prematurely due to adverse drug effects. The patient had RYGB surgery in early 2000.
In 2014, he was treated with a combination of sofosbuvir and simeprevir. Prior to therapy, his liver enzyme levels were normal and his HCV RNA level was 29 964 IU/mL. After the first 4 weeks of treatment, HCV RNA was undetectable but it became detectable again by the 16th week of treatment. In 2015, he was treated with sofosbuvir and ledipasvir for 24 weeks. He had no detectable HCV RNA 12 weeks after completion of treatment (SVR-12) but relapsed 24 weeks later. |
pmc-6587385-2 | A 57-year-old woman with chronic genotype-1A HCV infection complicated by cirrhosis (CTP-A, MELD 6) with a history of grade 1 hepatic encephalopathy was evaluated for HCV treatment. She previously had been treated unsuccessfully with interferon and ribavirin. She had RYGB surgery in the 1990s.
In 2014, she had elevated liver enzyme levels (aspartate aminotransferase = 88 U/L, alanine aminotransferase = 76 U/L) and an HCV RNA level of 4 136 276 IU/mL. She was treated with a combination of sofosbuvir and simeprevir for 12 weeks. HCV RNA level during treatment is unknown; however, 8 weeks after completing therapy, her HCV RNA level was 3 132 997 IU/mL. Subsequently, she was treated with a combination sofosbuvir and ledipasvir, but this therapy was stopped after 11 weeks due to lack of virologic response. |
pmc-6587475-1 | A 30-year-old man presented with a 7-month history of mild lower back pain. He had no history of trauma, morning stiffness, oral ulcers, fever, hair loss, erythrasma, or weight loss. He had been treated by local doctors, but they had failed to make a diagnosis. Treatment had initially provided pain relief, but his lumbosacral pain had recurred.
A month before he was seen at the rheumatology outpatient clinic in our hospital, his lumbosacral pain had become more severe, and he had developed pain in both his legs and his knees, which was more severe on the right. The pain had been severe enough to restrict his daily activities and was present at night, disturbing his sleep. His other symptoms included a dry mouth. He was treated by local doctors again, but this time he did not respond to treatment. A localized computerized tomography (CT) scan revealed mild bone hyperplasia of the lumbar vertebrae and disc herniations in L3–S1, prompting referral to the rheumatology department of our hospital. On physical examination, he was found to have percussion pain over his lumbosacral region and restriction of movement when doing squats. Examination of other systems revealed no abnormalities. The rheumatologists made a provisional diagnosis of spinal arthritis.
A peripheral blood test found a white blood cell count of 3.48 × 109/L (normal range, 3.5–9.5), red blood cell count of 3.87 × 109/L (normal range, 4.3–5.8), hemoglobin level of 11.4 g/dL (normal range, 13–17.5), platelet count of 215 × 109/L (normal range, 125–350), and 48.3% lymphocytes (normal range, 20–50). His C-reactive protein level was 2.29 mg/dL (normal range, <0.3) and his rheumatoid factor level was 12 U/mL (normal range, <25). Tests for antinuclear antibody, anti-cyclic citrullinated peptide, antikeratin antibody, and antineutrophil cytoplasmic antibodies (A-ANCA, C-ANCA, and P-ANCA) were all negative. His chest X-ray was normal. Magnetic resonance imaging of his sacroiliac area revealed inverse transformation of the bone marrow, prompting his clinicians to do a bone marrow biopsy. This revealed 84% blast cells and B-cell lymphoblastic leukemia (Fig. ).
After providing informed consent to referral, he was referred to our hematology department for further investigations. Immunophenotypic analysis, performed on bone marrow aspirate samples, confirmed the previous findings. The diagnosis of ALL was confirmed by demonstration of expression of the B-cell markers: CD19, HLA-DR, CD58, CD79a, TDT, CD123, and CD38 (Fig. ). Traditional cytogenetic analysis was performed on interphase cells prepared from bone marrow samples cultured for 24 hours without mitogens, using standard technology.[ The result was reported as an abnormal karyotype 47,XY,t(4;11)(q21;q24),+6(4)/46,XY(16) (Fig. ). Fluorescence in situ hybridization analysis was performed to test for common abnormalities associated with ALL, according to the manufacturer's instructions. The results were positive for MLL-AF4 (Fig. ), which is present in 6% of adults with ALL, and indicates a poor prognosis.[ He was started on an adult ALL chemotherapy protocol. His back pain resolved within a week of starting chemotherapy. At his most recent assessment, 10 months after diagnosis, he was on maintenance chemotherapy and in remission (Table ). |
pmc-6587639-1 | A 15-year-old girl visited the orthopedic service because of severe knee pain after a sudden fall while heading downstairs, and she was unable to stretch her leg. The patient had a history of dislocation of the patella 2 weeks ago. Physical examination showed tenderness on palpation over the upper pole of the patella and absence of active movement of the knee accompanied by swelling and joint effusion. A plain radiograph showed an avulsed fragment of the superior pole of the patella (Fig. ). Magnetic resonance imaging (MRI) confirmed a superior pole patellar avulsion fracture and dysfunction of the knee extensor mechanism (Fig. ). There were no signs of damage to the articular cartilage, meniscus, and cruciate ligament caused by previous dislocation.
Under general anesthesia, the patient underwent open surgery for reduction of the patellar fracture and reconstruction of the knee extension apparatus through an anterior approach. All of the above-mentioned imaging findings were further confirmed during surgery. Through a midline incision above the quadriceps tendon, careful dissection was performed to expose the injury site. Intraoperatively, a sleeve-like piece of bone was detected above the patella, and the proximal quadriceps tendon was attached to the bone piece (Fig. ). Then, complete removal of the blood clot and tissue debris was performed in the joint cavity and around the torn ends of the quadriceps tendon. Patellar retinaculum and quadriceps tendon with the patellar fracture piece was meticulously repaired and secured to the main patellar body by 2 Bio Composite suture anchors (Arthrex, Naples, FL) using a modified Mason–Allen stitch and was augmented by performing the figure-of-8 technique (Fig. ).
Postoperatively, the patient's knee was maintained in full extension using a knee brace, and weight-bearing was prohibited until 6 weeks after surgery. The passive range of motion exercise of the knee was started on the first day after surgery, and it was gradually progressed to full-scale motion as the tolerance improved. During the first 6 weeks of surgery, the patient's main activities included patellar mobility exercises, knee passive range of motion exercises, and quadriceps activation exercises. After 6 weeks, the patient was referred to the rehabilitation department for restoration of complete knee activity and initiation of muscle strength training. The patient was allowed to gradually resume sports when the normal strength, stability, and knee range of motion were comparable to those on the contralateral side.
Six months after the operation, the knee function was fully restored, there was absence of pain and swelling, and the patient was able to return to sports. |
pmc-6587640-1 | A 66-year old Chinese woman, born in Beijing, was admitted with complaints of cough, expectoration since for 3 months. She denied fever, night sweats, loss of weight, chest pain, or hemoptysis. Her past history was unremarkable with no history of tuberculosis or smoking. Physical examination was normal. Clinically, electrocardiography and routine laboratory tests were normal. Contrast-enhanced computerized tomography of the chest showed 35 × 22 mm irregular cystic lesion in right lower lobe, wall thickness of 0.5 mm to 3.5 mm, smooth inner margin, septum was seen within the cyst. The lesion also showed mild distortion of surrounding lung tissue with no evidence of abnormal arterial blood supply (Fig. A–C). The result of brush test showed cells with heteromorphic nucleus by bronchoscopy. Right lower lobe thoracoscopic wedge resection was done. The size of the lung tissue removed was 10.0 × 2.5 × 1.5 cm. Histopathology (Fig. ) showed cystic tumor of 4.5 × 2.0 × 1.5 cm, composed of multiple varying sized cysts lined by pseudostratified ciliated columnar epithelium with few areas of necrosis. All these features were consistent with the diagnosis of type 1 CPAM. On follow up 1 year later, the patient is asymptomatic. |
pmc-6587655-1 | A 68-year-old Taiwanese man with a history of gouty arthritis, hyperlipidemia, and adrenal insufficiency presented in our ED after 1 month of dyspnea and low back pain. He had no history of recent surgery or illicit substances use; however, previous frequent intravenous and/or intramuscular drug injections over shoulders and buttocks for gout attacks by local clinicians were mentioned. The patient denied smoking history and had no other notable medical record except low dose prednisolone prescribed. He also denied any recent invasive procedures or surgeries. The patient had consulted another hospital for similar symptoms before this visit. He was admitted and given serial examinations—an abdominal computed tomography (CT) scan and a TTE—but only a sternal fracture with a closed perifocal hematoma was found. During admission, the patient was diagnosed with Oxacillin-sensitive S aureus (OSSA) bacteremia, but no vegetation was detected using cardiac imaging. He was discharged with an uneventful course on his first admission.
The patient's persistent dyspnea and low back pain worsened, and he returned to our ED again 2 weeks later. On presentation, the patient was afebrile and had a pulse rate of 132 beats/min, a respiratory rate of 20 breaths/min, and a blood pressure of 117/87 mm Hg. A physical examination showed bilateral crackles, a right-side wheeze on chest auscultation, and a palpable fluctuant, non-tender mass (3 × 3 cm) over the anterior chest wall. There was no heart murmur and no skin rash over the chest wall. The patient's abdomen was soft without hepatosplenomegaly. His lower legs were not swollen, and they had no skin rash. No visible erythematous changes over hand (Janeway erythema) nor painful spots over fingers (Osler's nodes) being detected. The patient's laboratory data revealed the following: White blood cell count = 17.2 × 109/L (normal: 3.4–9.1 × 109/L) with 76% polys and 16% bands; platelet count = 32 × 109/L (normal: 138–353 × 109/L); prothrombin time = 14.3 s (control: 9.4–12.5 s); C-reactive protein = 270.0 mg/L (normal: 0–8 mg/L); blood glucose = 259 mg/dL (normal: <140 mg/dL); blood urea nitrogen = 62 mg/dL (normal: 7–21 mg/dL); serum creatinine = 1.8 mg/dL (normal: 0.7–1.5 mg/dL); alanine aminotransferase = 58 U/L (normal: 0–54 U/L); lactate = 7.2 mmol/L (normal: 0.7–2.1 mmol/L); D-dimers (fibrin degradation product) > 3500 ng/mL; fibrinogen = 546.8 mg/dL (normal: 30–400 mg/dL). Arterial blood gas measured in the ED revealed severe metabolic acidosis (pH, 7.28; PaCO2, 19.2; PaO2, 226; HCO3−, 8.9; BE, −18, SaO2, 100%) under an FiO2 1.0 concentration (non-rebreathing mask). No pyuria or hematuria was detected. An initial chest radiograph in the ED revealed no significant findings, and the electrocardiogram showed sinus tachycardia. A blood specimen was obtained for culturing and intravenous antibiotic agents (piperacillin and levofloxacin) were empirically initiated.
Because the patient's hemodynamic status and oxygen saturation rapidly deteriorated during observation in the ED, computed tomography of both chest and abdomen was arranged to detect a possible infection focus. A hypodense lesion with a peripherally enhanced wall and bone involvement was found in his sternal region (Fig. A), and multiple septic emboli lesions were found in his bilateral lung fields (Fig. B). Moreover, there was a paraspinal hypodense lesion with a peripherally enhanced wall and air-trapping over the T11 level. There was also one linear fracture line on T11 (Fig. A), and an L2 compressed fracture with a small anterior paraspinal abscess with gas density (Fig. B). CT-guided percutaneous drainage from the T11-T12 paraspinal space was done on day 2, and only minimal pus-like fluid was obtained for culture.
Because of the septic pulmonary emboli, TEE was done on day 3; it showed an oscillating mass (1.56 cm in size) over the anterior and septal leaflets of the tricuspid valve and moderate tricuspid regurgitation (Fig. ). The mitral, aortic, and pulmonary valves were intact, and there were no intracardiac shunts, either ASD (atrial septal defect) or PFO (perforation of foramen ovale) being found. Cardiovascular and chest surgeons were consulted immediately for emergency surgical intervention, but the family did not wish to pursue aggressive interventions and the patient eventually expired on day 4. OSSA was isolated from the blood culture and pus-like aspirate. The diagnosis was right-sided IE complicated with sternal abscesses, septic pulmonary emboli, and lumbar spondylodiscitis. |
pmc-6587658-1 | A 54-year-old male (body weight 75 Kg) was admitted to our hospital with acute chest tightness, asthma and shortness of breath after unexpectedly sliding down 3 steps 1 week ago. The patient had a history of HA with FVIII activity of 0.8% when he was first diagnosed. After the diagnosis, he received replacement therapy (13.3 IU/kg of FVIII each time) twice a week, which maintained an FVIII activity ranging from 2% to 80%. And he underwent bilateral knee replacement 5 years ago due to spontaneous hemorrhage. He has a 30-year history of smoking and drinking without withdrawal. Physical examination revealed a grade IV/VI holo-systolic murmur heard best at the apex and wet rales in both lungs. Transthoracic echocardiography (TTE) showed a posterior leaflet prolapse of mitral valve (P2 mainly) with moderate to severe regurgitation due to rupture of tendinae, and a left ventricular diastolic diameter of 56 mm and an ejection fraction of 70% (Fig. A). Coronary computed tomography angiography (CTA) demonstrated calcification and moderate stenosis of the left anterior descending artery (LAD) (Fig. B). Further examination of coronary angiography showed a 60% stenosis in the proximal LAD (Fig. C). Thus an operative plan of CABG and MVP was made.
The perioperative coagulation management strategy for this patient was based on the recommendations of anesthesiologist, surgeons, clinical pharmacists and perfusionists. Our goal was to achieve an initial FVIII level ≥70% for more than 2 weeks. Meanwhile, psychologists were counseled because patient was very anxious about excessive bleeding during surgery.
Two hours before surgery, a bolus of 1000 IU (13.3 IU/kg) of FVIII (Recombinant Coagulation FVIII for Injection, Bayer HealthCare LLC) was injected, which obtained an FVIII of 80%. After induction, a 3750 IU (50 IU/kg) bolus of FVIII was injected and subsequent FVIII level reached 135%. A tranexamic acid infusion of 1 g/h (lasting 10 h) was then started before incision. An intravenous introducer sheath (9F) was placed in the right internal jugular vein, and 400 mL of autologous blood was harvested. A dose of heparin (375 IU/kg) for CPB resulted in an activated clotting time (ACT) ≥300 seconds before the left internal mammary artery (LIMA) was severed. Surgery was performed with LIMA anastomosed to LAD after cardioplegic arrest, mitral valvuloplasty involving a triangular resection of the posterior leaflet, creation of an artificial chordae (Gortex CV-4) and insertion of an annuloplasty ring (28#, Sorin). The CPB lasted around 95 minutes, and a transesophageal echocardiography examination was performed (Fig. D). Finally, heparin was neutralized with 220 mg of protamine, causing ACT to return to 130 seconds, and a repeat FVIII activity level was 50.6%. 400 mL of autologous blood and 700 ml of CPB machine blood was returned to the patient as well as 4 units of fresh frozen plasma with an additional bolus of 1000 IU (13.3 IU/kg) FVIII. The patient was transferred to the surgical intensive care unit and extubated within 2 hours postoperatively. 100 mg aspirin per day was given from that day on. A bolus of 2000 IU (26.7 IU/kg) FVIII was administered in the evening of the day of surgery, and FVIII supplementation was continued every 12 hours (2500 IU each time on days 1–4, 2000 IU on days 5–9 and 1000 IU on days 10–14) until postoperative day 14.
The total postoperative drainage from mediastinal, chest and pericardial drainage tubes was 350 mL on day 1 and 150 mL on day 2, so the tubes were removed on the 3rd postoperative day. The patient recovered uneventfully and was discharged home on the 8th postoperative day with a residual FVIII activity of 54%. One-year follow-up showed the patient had no bleeding or thrombosis complications, and TTE showed no evidence of mitral regurgitation. |
pmc-6587661-1 | A 31-year-old Asian woman was found to have twin pregnancy during her first gravida. She initially presented frequent vaginal bleeding, and her obstetrician prescribed oral progesterone at a dosage of 300 to 400 mg per day (Utrogestan) and intramuscular progesterone at a dosage of 125 mg/amp per week (Progeston Depot-s) since her 8th week of gestation. However, because of early uterine contraction and cervical incontinence, she started to take additional ritodrine at 10 mg 6 times daily (Yutopar) and rectal indomethacin since her 18th week of gestation. After Mcdonald cerclage was performed at the 21st week of gestation, she restricted her daily activities at home and rested in bed most of the time, following her obstetrician's advice. A cesarean section was then arranged at the 32nd week due to frequent uterine contractions. However, progressive erythematous swelling and local heat of the left lower limb were found on the 14th day after delivery. In addition, she denied dyspnea, chest pain, fever, chills, cough, and hemoptysis.
Venous duplex ultrasound examination showed thrombus formation in the left femoral vein and popliteal vein with reduced compressibility. Under the impression of DVT with VTE, a regimen of rivaroxaban (15 mg) twice daily was prescribed after discussion by mode of shared decision making with families and patient herself.
After oral anticoagulation therapy with additional medical compression stocking, her leg swelling was ameliorated gradually 3 days later. On the basis of current clinical trial and treatment guidelines, we switched the regimen to rivaroxaban (20 mg) once daily after 21 days.
After 3 months of anticoagulation therapy, we observed only partial improvement of the symptoms, and the condition deteriorated after her ordinary activities. Repeated venous duplex ultrasound examination revealed residual thrombus in the left femoral vein. For further evaluation and management, we performed invasive venous angiography. |
pmc-6588116-1 | A 54-year-old woman with history of hypertension and newly diagnosed type II diabetes mellitus developed facial flushing, muscle weakness, labile blood pressures, lower extremity edema, and uncontrollable hyperglycemia despite maximal medical therapy. She then developed acute heart failure requiring inpatient admission. On admission, physical examination showed classic stigmata of Cushing's syndrome, including easy bruisability, moon facies, a buffalo hump, and abdominal striae (). Brain magnetic resonance imaging showed no sellar or suprasellar mass. Random cortisol level was 61 μg/dL (normal 6–23 μg/dL), random ACTH level was 223 pg/mL (6–58 pg/mL), and 24-h urinary cortisol secretion was elevated. She underwent inferior petrosal sinus sampling with no significant central to peripheral ACTH concentration gradient, leading to a diagnosis of ectopic ACTH syndrome. Abdominal imaging, including ultrasonography, computed tomography (CT), and ultimately positron emission tomography (PET)-CT, identified a 3.4 × 2.9 cm hypermetabolic mass in the pancreatic tail with abnormal peripancreatic lymph nodes and a hepatic lesion concerning for metastatic disease (). Endoscopic ultrasonography with fine needle aspiration of the pancreatic mass revealed a pNEN with immunohistochemical stains positive for ACTH, synaptophysin, and chromogranin.
With the diagnosis of an ACTH-producing pNEN confirmed, the patient underwent laparoscopic distal pancreatectomy and splenectomy with hepatic metastasectomy. All gross disease was resected. Histological examination revealed a high-grade neuroendocrine carcinoma without features of small or large cell involving pancreas, liver, and 6 of 16 lymph nodes (). The patient's ACTH and cortisol levels normalized within 24 h. Her postoperative course was complicated by Ogilvie's syndrome requiring right hemicolectomy. She subsequently recovered without other complications. She was started on lanreotide postoperatively, which she continued at the time of discharge. Imaging revealed progressive hepatic metastases 3 months after resection. She was treated with capecitabine at another institution at this time, which she continued intermittently for the next 6 months. Unfortunately, she developed recurrent symptoms of Cushing's syndrome and heart failure at ∼9 months postoperatively and died of complications related to her disease 1 year after the operation. |
pmc-6588662-1 | A 40-year-old female presenting with mild hepatic dysfunction was referred to our hospital. She did not smoke but had a drinking habit. At age 28 years, she had presented with elevated platelet counts (> 100 × 104/μL); ET had been diagnosed based on bone marrow biopsy results. She was prescribed aspirin (100 mg/day) and anagrelide (2.5 mg/day). She had also been prescribed ebastine for itching a while ago.
On admission to our hospital, laboratory examination revealed slightly elevated alanine aminotransferase (ALT) levels (82 IU/L), although the patient’s ALT level had improved from that recorded previously. Her platelet count was slightly elevated (62.4 × 104/μL). Prothrombin time and activated partial thromboplastin time were normal. Abdominal ultrasonography revealed a cecal tumor. Colonoscopy revealed advanced cecal cancer (Fig. ). Computed tomography (CT) indicated cecal wall thickening (Fig. ).
The patient recovered from liver dysfunction without treatment. She stopped taking oral aspirin 1 week prior to surgery but continued anagrelide until the day before surgery. To prevent thrombosis, she wore elastic stockings; furthermore, intermittent pneumatic compression was performed during surgery. Laparoscopic-assisted ileocecal resection was performed. We used a soft coagulation system to achieve complete hemostasis. The operative duration was 202 min; blood loss was 34 mL.
From the first postoperative day, the patient started walking, drinking water, and resumed oral anagrelide intake. She resumed oral aspirin intake on the fifth postoperative day. Her perioperative platelet count was controlled to approximately 40–60 × 104/μL (Fig. ). Prothrombin time and activated partial thromboplastin time also did not show abnormal values during the perioperative period. The postoperative course was uneventful and she was discharged on the seventh postoperative day.
The tumor pathological stage was T3N1M0 (Stage IIIB). The patient received intravenous oxaliplatin plus oral capecitabine (CapeOX) as postoperative adjuvant chemotherapy (oxaliplatin 130 mg/m2, capecitabine 1000 mg/m2). However, after one course, she again experienced liver dysfunction (aspartate aminotransferase [AST] level, 388 IU/L; ALT level, 531 IU/L); because of anagrelide, her platelet count decreased to 17.8 × 104/μL. Therefore, we asked her to discontinue anagrelide and aspirin that day onwards; 5 days later, her platelet count recovered to 50 × 104/μL. Subsequently, she resumed taking anagrelide and aspirin; however, she refused to resume any adjuvant chemotherapy after this incident. Her liver function normalized gradually in 4 months. There were no clinical signs of thrombosis, and there was no appearance of a new thrombus on contrast-enhanced CT 6 months after the operation. One-year post operation, she is well without tumor recurrence or new metastasis. |
pmc-6588891-1 | A 47-year-old male developed heart failure due to dilated cardiomyopathy 12 years ago. A cardiac resynchronization therapy-defibrillator (CRT-D; Medtronic® Viva XT CRT-D; AAI 60) was implanted due to VF 7 years ago, and as a bridge to transplantation, a HeartMate II® LVAD was implanted 4 years ago. No arrhythmia developed immediately after LVAD implantation; thus, his CRT-D shock therapy was turned off immediately after LVAD implantation. At the time of LVAD implantation, his transthoracic echocardiographic study showed a significant decrease in the left ventricular (LV) contractility (ejection fraction; 13%), dilation of left ventricle (51 mm in diastole) and trivial aortic regurgitation (AR) without opening of aortic valve but right ventricular (RV) contraction had maintained well relatively (RV fractional area change; 33%).
Eight months after LVAD implantation, the patient developed palpitations and was admitted to our hospital due to repeated VAs necessitating electrical defibrillation. Echocardiography showed the left ventricle diameter did not change, whereas right ventricle volume was slightly enlarged. The repeated VAs were also refractory to various anti-arrhythmic agents, including amiodarone, nifekalant, mexiletine and lidocain, with eventual progression to sustained VF. The hemodynamic compromise due to sustained VF resulted in liver congestion, which was alleviated with a phosphodiesterase type 5 inhibitor, diuretics, and rotation speed optimization (from 8800 to 9600 rpm). These interventions reduced organ dysfunction, suggesting that minimum-required perfusion to vital organs was maintained even under sustained VF. The patient was followed up on an outpatient basis thereafter.
Approximately 2 years after the development of sustained VF, paroxysmal AF was detected on the monitoring records of CRT-D, with a gradually increasing frequency. After 3 years of sustained VF, the patient was readmitted to our hospital due to worsening of symptoms associated with right heart failure and liver congestion (total bilirubin, 3.9 mg/dl). Although his electrocardiogram remained sustained VF (Fig. ), the CRT-D revealed conversion of the sinus or atrial pacing rhythm to persistent AF. Transthoracic echocardiography revealed that fibrillation of the atrium resulted in the disappearance of not only the mitral flow but also the RV outflow tract doppler flow by the atrial kick (Fig. , Additional file 1, 2, 3, 4, 5 and 6). Under sustained VF, RV cardiac output is greatly dependent on atrial kick in which the contribution of atrial kick extraordinary enhanced. The hemodynamic study indicated that the pressure wave from the right atrium (RA) to the right ventricle was significantly flattened resulting that the pulmonary artery pulsatility index, which is defined as the ratio of pulmonary artery pulse pressure to right atrial pressure, was markedly decreased. It suggested a marked reduction in blood flow induced by RA contraction in persistent AF as opposed to sinus rhythm (Fig. ). As the incremental rotation speed lead to an increase in RA pressure (RAP) from 19 mmHg to 22 mmHg, the rotation speed was set to 9600 rpm and the right heart congestion was treated with additional diuretics insufficiently and the enhanced level of total bilirubin was prolonged. Until four months later, heart failure was gradually improved and the level of total bilirubin decreased below 2 mg/dl. The monitoring records of CRT-D revealed the recovery of sinus rhythm during previous four months (Fig. ). Although he had remained sustained VF, the recovery of sinus rhythm finely corresponded to the improvement of heart failure and the level of his total bilirubin decreased to 1.2 mg/dl. It intensely corroborated the contribution of persistent AF on the worsening of right heart failure. |
pmc-6588903-1 | A 32-year-old Caucasian woman was recruited during her first pregnancy for a longitudinal breastfeeding research study (UWA Human Research Ethics, reference RA/4/20/4023). Timing of samples and measurements collected are outlined in Table .
Prior to conception the woman had a healthy BMI of 19.8 and Crohn’s disease, medicated with Adalimumab. During pregnancy she was diagnosed with GDM and was otherwise healthy with no other reported cardiovascular or metabolic diseases. Overall, the woman reported having a healthy balanced diet, having no allergies, and being a non-smoker. Her infant was fed colostrum and she experienced breast fullness on day 4, coinciding with secretory activation. The mother was advised to pump as frequently as possible, in addition to breastfeeding, and had breastfeeding support at home, although she did not have breastfeeding support from healthcare providers until her infant was 4 months old. It is of interest that the mother herself was born SGA, 2100 g at 40 weeks.
The male infant was delivered vaginally at 38 weeks and 3 days, after induction due to diagnosed placental insufficiency resulting in fetal growth restriction. The infant birth weight was 2390 g, and below the 3rd percentile. For the first 3.5 months of life, the exclusively breastfed infant was tracking along the 3rd percentile for growth, as shown in Fig. a. After 3.5 months, formula supplementation was initiated yet the infant growth continued along the 3rd percentile. In contrast, the infant was born with a head circumference of 34 cm (Fig. b), just below the 50th percentile, indicating head sparing as is characteristic of SGA infants. He was otherwise healthy, with no tongue tie or other oral abnormalities, and averaged 12 feeds per day (within the population range). Solids were introduced at 5 months of age. At 1 year, the infant weighed 9604 g with a head circumference of 47.8 cm, corresponding to the 50th and 85th percentile respectively.
As part of the longitudinal study, maternal milk production was measured at 3 months post-partum by weighing the infant before and after each feed for 24 h using an electronic scale []. A volume of 414 mL/day was produced, below the reference range (478–1298 mL/day). Infant milk intake for that day was 419 mL, of both breastfed and expressed milk. The breastfeeding profile falls within range for the number of breastfeeds and duration of breastfeed, but not for the average breastfeed volume from the left breast nor the milk volume that was breastfed and expressed (Table ). A total daily dose of 60 mg Domperidone was initiated at 6 weeks post-partum, presumably to address issues of low milk supply, along with regular pumping after and between feeds, and continued up until 7 months of lactation, however she reported that her dosage decreased over this time.
As part of the longitudinal study, monthly pre-feed samples were collected from birth to 6 months post-partum, and additional samples were also collected for every feed or expression during the 24-h milk production at 3 months []. Samples were collected by hand expression into 5 mL plastic vials and were stored frozen at − 20 °C for up to 48 h then transferred to − 80 °C laboratory freezer until analysis. The total fat concentration of the whole milk was measured using the creamatocrit method []. The protein concentration of skimmed milk was measured using the Bradford Protein Assay with human milk protein standards. The lactose concentration of skimmed milk was measured using an enzymatic spectrophotometric method []. Replicate analyses were carried out for all samples.
The median fat concentration measured in 1, 5 and 6 months post-partum (25.8, 34.3 and 26.6 g/L, respectively) were below the reference range (36-50 g/L; Fig. a). All samples from 2 to 6 months post-partum (ranging from 4 to 8 g/L) had median protein concentrations below the reference range (9–12 g/L; Fig. b). The median lactose concentrations were within the reference range (60-100 g/L) at each month (Fig. c). References ranges were taken from those previously established in milk samples of healthy mothers breastfeeding healthy term infants [].
Infant total energy intake (both breastfed and expressed milk intake) at 3 months was calculated using infant milk volume intake and energy per gram of fat (37.7 kJ/g), protein (16.7 kJ/g) and lactose (16.7 kJ/g). Infant daily energy intake from fat, protein and lactose was approximately 595 kJ, 46 kJ and 537 kJ, respectively. The infant total daily energy intake was 1178 kJ/day, amounting to a low total energy dose from fat, protein and lactose per day (Table ). |
pmc-6588915-1 | A 46-year old woman initially presented with progressive orthostatic headache (sudden onset) at the emergency unit since 1 month. There was no history of trauma and the neurological exam was normal. Analgesia and bedrest, as prescribed from the patient’s family doctor, had only minor clinical effect. Initial cranial computed tomography (CT) scan revealed small bifrontal hygroma. Pressure measurement by lumbar puncture was considered not reliable due to pressure values equaling to zero. Further workup by cranial and whole-spine magnetic resonance imaging (MRI; fluid-sensitive and thin-sliced) revealed cranial dural contrast-enhancement (with small bifrontal hygroma, Fig. a) as well as extradural fluid collection at the level of the thoracic spine suggestive of a dural leak causing CSF loss and thus SIH (Fig. b) [, ]. Three consecutive (unspecific) lumbar epidural blood patches (serially performed at 3 day intervals) were conducted without sustainable clinical amelioration. Finally, a longspine CT-myelography (dynamic myelography with postmyelography spine CT imaging) identified a possible dural tear due to ventral extradural contrast leakage at the level of the thoracic vertebrae 11/12 and thoracic vertebrae 12 (Th12) / lumbar vertebrae 1 (L1) with high suspicion of a trans−/intradural lesion (Fig. c and d). Consequently, surgical exploration of the described levels was performed by a translaminar and transdural approach at the level of Th12 (Fig. a-d). Intraoperatively, a ventral dural slit was identified with CSF leakage to the extradural space at the level of Th12/L1 due to an intradural disc sequester (Fig. b). Subsequent resection of the disc sequester (by use of sensory/motor evoked potentials, SEPs/MEPs, of lower extremities and anal sphincter) with tight ventral/dorsal dural closure by suture (Fig. c) and laminoplasty of Th12 (Fig. d) was successfully conducted (refer to the intraoperative video animation in the “Additional file 1”). Histological workup of the resected lesion confirmed non-calcified intervertebral disc material. The postoperative clinical course was uneventful. The patient underwent stepwise and cautious mobilisation to avoid relapse of CSF leakage. Immediate cessation of the orthostatic symptoms was observed postoperatively and at a 3 months follow-up and the patient was able to return to work. Conventional radiography of the thoracolumbar junction showed no signs of dislocation of the artificially reattached lamina of Th12. Written informed consent was obtained from the patient to report and publish individual patient data. |
pmc-6588922-1 | In June 2011, a 56-year-old male was referred to our department by head and neck surgeon in order to improve his upper denture retention and stability. The patient was diagnosed with a squamous cell carcinoma of the maxillary gingiva (T4N0M0) in May 2005 and underwent an extended left maxillectomy, an anterior and middle cranial base resection, a left ophthalmectomy, and a flap reconstruction using the rectus abdominis muscle were performed. On physical examination, a recessed deformation on the left side of his face could be seen because of the left ophthalmectomy. The function of the left levator palpebrae muscle was eliminated to the level of a slight elevation by using the frontal muscle. A metal plate was anchored to the inferior wall of orbit. The left ethmoid bone, inferior nasal turbinate, the maxilla, alisphenoid, medial and lateral pterygoid muscle were already excised during the mesh titanium plate reconstruction of the anterior wall from the maxillary orbital region. Intraorally, the left maxilla had been excised from the midline, with the rectus abdominis muscle flap sutured directly to the soft palate. The peripheral mucous membrane around the left upper lip was already scarred, without the oral vestibule, and the flap margin had moved along with the surrounding soft tissue. The 70 × 50 mm flap was sagging from its weight and was in contact with the mandibular molars, reducing the volume of the oral cavity unless dentures were worn. The maxilla was removed from the midline to the maxillary tuberosity, while the mandible was removed from the anterior border of the ramus to the coronoid process. Dead space was eliminated because the abdominal rectus muscle was placed from the anterior cranial base to the oral cavity during reconstruction (Fig. ). No expiratory leakage or food reflux was observed, and the rhinopharyngeal closure was maintained. Prior to performing surgery, there was no tumor recurrence or metastasis. The patient had a mouth opening of 43 mm, which we judged operable and then conducted the flap reduction and elevation under general anesthesia in Dec 2008. Informed consent was obtained from the patient’s parents prior to study initiation, and all procedures were performed in accordance with the Declaration of Helsinki.
Surgical reconstruction was performed as follows:An incision was made from the buccal side of the sutured edge (scar) in the abdominal rectus muscle flap (Fig. ). We can conduct a vestibular extension at the same time by incising this position. The adipose tissue was peeled from the buccal side to slightly beyond the skin flap center while maintaining approximately 5 mm thickness. The adipose tissue was reduced using a radio knife (8 g) (Fig. ). When we reduce fat tissue, we must avoid perforating of the skin. The skin was incised directly above the zygomatic bone, with tissue separation (avoiding exposure of the plate) to enable easy visibility of the zygomatic bone. Subsequently, the subcutaneous tissue was peeled from the zygomatic bone to the oral cavity for tunneling. Three mini QUICKANCHOR® (Depuy Mitek Surgical Products, Inc. Raynham, MA, USA) anchors were placed in the zygomatic bone, and anchor sutures were drawn through the subcutaneous tissue to lift the skin flap. A modeling compound was used to shape the margin of the celluloid splint (Fig. ). The advantage of flap suspension using Mitek anchors is the simple operability, less anchor positioning limitation, and easier length adjustment of the thread for suspension, which lead to easier fixation of soft tissue without slackness as well as clinically sufficient strength for fixation of ligament and tissue. On the other hand, less than 4 mm thickness of the cortical bone for suture anchor fixation causes insufficient fixation, therefore, determining placing position on the bone for fixation is necessary. Consequently, due to the versatility, the position that is considered optimal for stronger fixation and more efficient suspension can be selected as the anchor placing position, while the periosteum, corium, and scar tissue that are thought the most suitable for maintaining the strength can be chosen for the suture thread. Regarding the anchor placing position in this case, we determined 3 positions on the zygomatic bone and sutured flap corium taking into consideration a complete maxillectomy had been completed, which resulted in being able to lift the flap outward and upward.
Postoperatively, the color of the skin flap was normal without congestion or necrosis. The celluloid splint was removed 10 days after the surgery with no infection or necrosis observed in the skin flap. We can find only fat, scar tissue, not carcinoma in the reduced fat tissure. At 3 months postoperatively, epithelialization and scarring were observed on the border of the skin flap and buccal mucosa, with no wound opening. Next, a denture that was stabilized to the right residual teeth with a clasp made. This prosthesis had two double Akers cast clasps unilaterally to retain the prosthesis by the four remaining molars. The major connector used anteria paratal plate. The patient was quite satisfied to be able to masticate, form an alimentary bolus, and swallow without any teeth falling out. No re-sagging of the skin flap or wound infection was observed at 3 years postoperatively. Patient follow-up will be continued at our department (Fig. ). |
pmc-6588931-1 | A 43-year-old Japanese woman presented to our hospital with a 7-year history of progressive headaches and intermittent claudication. She was diagnosed as having SLE according to the 1997 American College of Rheumatology revised classification criteria (oral ulcers, discoid rashes, positive anti-nuclear antibody, leukopenia, and lymphopenia) [] and histological findings of the skin (interface dermatitis and positive lupus band test) 11 years previously, all of which improved with low-dose prednisolone. She had no atherosclerotic risk factors such as cigarette smoking, dyslipidemia, diabetes mellitus, or hypertension. She drank alcohol only on social occasions and had no significant other past medical, social, environmental, obstetrical, gynecological, or employment history. Her mother had autoimmune hepatitis and died at the age of 60 of subarachnoid hemorrhage. On hospitalization, her temperature was 36.0 °C, her blood pressure was 124/82 mmHg, and her pulse was 78 per minute, regular and of normal tension. A physical examination revealed the absence of pulsation in the bilateral posterior tibial arteries and the left dorsalis pedis artery. She had no adenopathy, aphthous ulcers, or rash. Her heart sounds were clear and regular without audible murmurs and her lungs were clear. Her abdomen was flat and soft, and bowel sounds were normal without murmurs. A neurological examination showed that her cranial nerves were intact, her muscle strength was normal, her deep tendon reflexes were symmetrical without Babinski sign, and no sensory abnormalities were noted. Laboratory data revealed mild leukopenia (3300/μL) and slightly elevated erythrocyte sedimentation rate (24 mm/hour). C-reactive protein level, complement level, and urine sediment were normal. Anti-double-stranded deoxyribonucleic acid (dsDNA), anti-Smith, anti-cardiolipin, anti-neutrophil cytoplasmic antibodies, and lupus anticoagulant were all negative. Vascular ultrasonography revealed occlusion of her bilateral posterior tibial arteries, bilateral peroneal arteries, and left anterior tibial artery with collateral circulation and wall thickening of her right radial artery. Luminal narrowing in her right temporal artery and concentric hypoechoic mural thickening (the halo sign) in her left temporal artery were detected, although no abnormalities in her carotid arteries were found (Fig. A).
Enhanced computed tomography showed vascular occlusion of bilateral lower limbs and her right ophthalmic artery (Fig. B, C). Enhanced magnetic resonance imaging and positron emission tomography revealed no inflammation in the wall of the large vessels. The histological findings of the left temporal artery revealed narrowing of the lumen caused by intimal thickening without inflammatory cells, and the deposition of immunoglobulin G (IgG), complement component 3 (C3), and fibrinogen in the wall of the intima (Fig. ). Atherosclerotic changes, such as fibrous plaque, foam cells, or Monckeberg’s calcification, were not found. Active or healed arteritis, including giant cells within the intima, fragmentation, loss of internal elastic lamina, or neovascularization of the media, was not detected.
We made a diagnosis of large vessel vasculopathy associated with SLE based on the histopathological findings. Vascular involvements were only observed in the peripheral large vessel according to the Chapel Hill Consensus Conference nomenclature system []. We initiated 120 μg/day of beraprost sodium and 200 mg/day of cilostazol therapy orally. At 6 months after discharge, re-examination by ultrasonography revealed recanalization in our patient’s left posterior tibial artery. |
pmc-6589168-1 | A 35-year-old Hispanic man presented to our emergency department and reported 1 week of generalized malaise, abdominal pain, fatigue, subjective fever, sore throat, joint pain, watery diarrhea, intolerance to oral intake, and petechial skin rash (Fig. ). The skin rash was described as blisters, which progressed to erythematous macules that were non-confluent and were associated with burning and itching. The rash started on his palms and soles and then subsequently spread centripetally to his arms, chest, and trunk. Diarrhea occurred at a frequency of 2–3 episodes a day but had resolved by the time of evaluation. His past medical history was only significant for chronic back pain, for which he had been taking 100 mg of diclofenac potassium twice daily for 1 week before admission. Prior to admission, he was not taking any medications due to lack of a past medical history. The only medication taken was diclofenac potassium, as mentioned above. He denied the use of any other medication, tobacco smoking, alcohol intake, or drug use. His family history and psychosocial history were unremarkable. He denied any prior surgical intervention. He reported self-employment as an independent taxi driver.
At initial assessment on admission the physical examination was remarkable for a blood pressure of 132 systolic and 77 diastolic mmHg, a temperature of 39.6 °C (103.2 °F), pulse of 132, and respiratory rate of 18. He was well developed, well groomed, with skin remarkable for non-confluent, non-blanching erythematous macules. He had an atraumatic head and his eyes had reactive pupils that were symmetric with clear conjunctiva. He had a supple neck with no signs of jugular vein distention (JVD) or thyromegaly. His thorax was symmetric with non-labored respirations, lungs were clear to auscultation bilaterally, his heart rate was regular with regular rhythm, pulses were palpable and normal in his bilateral upper and lower extremities; no rub or murmur was noted. His abdomen was soft and non-tender with no distention and no palpable masses; bowel sounds were present. He was alert and oriented, and followed commands; cranial nerves 2–12 were preserved, deep tendon reflexes were intact, strength was 5/5 bilaterally in both his upper and lower extremities.
The laboratory results were remarkable for anemia, thrombocytopenia, elevated C-reactive protein (CRP) 21.9 mg/L (reference value ≤ 5 mg/L), and mild transaminitis, with no deranged bilirubin or alkaline phosphatases. No acute findings were reported from chest X-ray.
He was initially diagnosed as having acute viral gastroenteritis and was managed with supportive therapy. Due to a persistent fever, he was transferred to the intensive care unit (ICU) and started on broad-spectrum antibiotics and antiviral therapy, which included vancomycin, aztreonam, azithromycin, and oseltamivir. Because his blood cultures and urine culture were negative, vancomycin was discontinued.
His febrile episodes persisted and he had repeated negative blood and urine cultures, negative vasculitis, and unremarkable autoimmune work-ups. Subsequently, he developed acute encephalopathy and experienced a seizure. He was then intubated for airway protection and placed on mechanical ventilation. A lumbar puncture was deferred, given the presence of profound thrombocytopenia and the risk of bleeding. Computed tomography of his head reported no acute signs of intracranial hemorrhage or infarction. Other remarkable findings were a platelet count of 10 k/μl, hemoglobin of 6.2 g/dl, hematocrit of 18.2%, reticulocyte count of 10%, plasma prothrombin time (PT) > 11.3 seconds (reference range, 9–12 seconds), activated partial thromboplastin time (PTT) > 27.1 seconds (reference range, 26.1–33.8 seconds), serum creatinine 0.8 mg/dl (reference range, 0.5–1.5 mg/dl) alanine aminotransferase serum of 55 unit/L (reference range, 5–40 unit/L), aspartate transaminase serum of 35 unit/L (reference range, 9–48 unit/L), total bilirubin serum of 1.4 mg/dL (reference range, 0.2–1.2 mg/dL), direct serum bilirubin of 0.6 mg/dL (reference range, 0.0–0.3 mg/dL), lactate dehydrogenase (LDH) serum of 1172 unit/L (reference range, 100–190 unit/L), haptoglobin of 0 mg/dL (reference range, 30–200 mg/dL), a peripheral smear with schistocytes (Fig. ), total hemolytic complement assay less than 14 (reference range, 31–60 U/mL), and a positive direct Coombs test (Table ). All viral serologic tests were negative (Table ). Given these test results and a significantly worsening neurological status, high doses of intravenously administered steroids were initiated, along with intravenously administered gamma immunoglobulin. This was indicated for suspected immune-mediated hemolysis versus an acute vasculitis process. A skin biopsy was also performed, and a formalin-fixed skin biopsy showed patchy granular deposition of C5b-9 along superficial dermal vessels, dermal epidermal junction (Fig. ).
The findings of microangiopathic hemolytic anemia, thrombocytopenia, and neurological derangement were enough to suspect a diagnosis of TTP. Thus, serum ADAMTS13 activity and inhibitor assays were conducted. Serum ADAMTS13 activity was < 3% (reference range, 68–163%), and the serum inhibitor level was 1.5 (normal range, < 0.5). Specific identification of the inhibitor could not be obtained. He was then empirically treated by transfusing fresh frozen plasma and he underwent plasmapheresis sessions.
He received five sessions of plasmapheresis, which improved his clinical status and allowed for successful liberation from mechanical ventilation. Antibiotics were de-escalated based on culture reports, and intravenous steroid dosages were tapered down. Laboratory assessments showed increases in levels of platelets, haptoglobin, LDH, and hemoglobin. Levels of important compounds throughout the course of treatment are presented in Table . He was subsequently discharged from the ICU and hospital. The post-plasmapheresis level of serum ADAMTS13 activity was 126%, thus confirming the diagnosis of acquired TTP.
Further follow-ups were done in our hematology clinic at 3 months and 6 months following hospital discharge. As mentioned in this case report, he was found to be asymptomatic, including a lack of rash and no further neurological symptoms. In addition, all laboratory tests were reported to be within normal limits, including serum haptoglobin, LDH, hemoglobin, and platelets, as were renal function tests, liver function tests, and serum ADAMTS 13 activity. No further intervention was recommended from a hematology point of view and our patient returned to work without further complications.
Our patient gave us informed consent to publish this case, including images. |
pmc-6589176-1 | We present the case of a 42-year-old Caucasian non-smoking female who was admitted in the Endocrinology Department for right unilateral exophthalmia and epiphora in the prior 8 months (Fig. a). Her family clinical history was positive for breast cancer (mother) and high blood pressure and type 2 diabetes (father) but negative for endocrine conditions. Since menarche at age 12, she has had regular menses and a childbirth. |
pmc-6589226-1 | A 64-year-old Caucasian man with a lifelong history of thrombocytopenia was found with elevated total protein of 10.3 g/dl, serum monoclonal spike of 3.5 g/dl, immunoglobulin G (IgG) of 5371 mg/dl, and free lambda light chains of 703 mg/dl with free kappa/lambda ratio of 0.08. Calcium and renal function were within normal limits. Hemoglobin was 16.6 g/dL, and platelet count was 33 × 103/μL. Skeletal survey was negative for any lytic lesions. Bone marrow examination showed adequate megakaryocytes with normal platelet morphology, and no dysmegakaryopoiesis or micromegakaryocytes were seen on the bone marrow. Erythrocytes and granulocytes were adequate in number without any significant abnormality.
The past medical history included essential hypertension and hypogonadism, but without neuropathy or dermatologic conditions. The diagnosis of thrombocytopenia stemmed from childhood and was accompanied by a penetrant family history of thrombocytopenia seen in his mother, maternal aunt, brother, and son. No genetic cause of the thrombocytopenia had been established, and questioning found no personal or family history of physical deformity, vision, hearing, or bleeding disorders. Laboratory review of his platelet count history ranged from 30 to 50 × 103/μL.
Repeat testing of hemoglobin was 17.9 g/dl with hematocrit of 55.6%. Peripheral blood examination showed normal appearing red and white blood cells, with erythrocytosis, and morphologically normal platelets that were decreased in number. Erythropoietin level was normal (12.2 mIU/mL), and molecular testing found no mutations in JAK2 V617F or BCR/ABL1 (p190 and p210). The erythrocytosis was attributed to use of testosterone supplementation for hypogonadism. A limited congenital thrombocytopenia testing panel was ordered and resulted negative for mutations in myeloproliferative leukemia protein (MPL), runt-related transcription factor 1 (RUNX1), myosin heavy chain 9 (MYH9), and Wiskott–Aldrich syndrome (WAS) gene mutations. Flow cytometry for platelet associated autoantibodies. Anti-glycoprotein IIb/IIIa antibodies, anti-glycoprotein Ib/Ix antibodies, and anti-glycoprotein CD36 antibodies were also negative.
After a period of monitoring, his serum IgG increased to 7063 mg/dl and monoclonal-spike to 5.4 g/dl. Albumin level was 2.7 g/dl, and beta-2 microglobulin level was 2.52 mg/dl. Repeat bone marrow biopsy showed 70% plasma cells with adequate megakaryocytes and normal platelet morphology, and he was diagnosed with International Staging System (ISS) stage II IgG lambda multiple myeloma. Treatment was now indicated, however complicated given his marked thrombocytopenia.
He started treatment with pulse doses of dexamethasone (40 mg daily for 4 days) with subsequent addition of bortezomib at 0.4 mg/m2 with slow escalation to full dose (1.3 mg/m2) by week 3. Oral cyclophosphamide (600 mg/weekly) was added to this regimen during cycle 2. After 5 cycles of CyBorD (cyclophosphamide, bortezomib, and dexamethasone), the patient had normalization of IgG levels and decrease in M-spike to 0.62 g/dl consistent with a partial response. He was referred for consolidative melphalan-conditioned autologous stem cell transplant.
Stem cell mobilization with granulocyte colony-stimulating factor (10 mcg/kg × 5 doses and plerixafor 24 mg × 1 dose) resulted in peak white blood cell count of 151 × 103/μL and stem cell yield of 105.6 × 106 CD34+/kg in a single session (the highest recorded at our institution that year). The patient proceeded to high-dose melphalan (200 mg/m2) followed by infusion of 45 × 106 CD34+/kg autologous stem cells. Despite a robust and quick neutrophil engraftment on day +9, the patient encountered marked nausea and a severe pruritic skin rash that started after engraftment and lasted for 1-2 months, resembling a graft versus host reaction. After engraftment, his platelet count peaked as high as 84 × 103/μL on D + 21 before slowly declining back to his baseline of about 30 × 103/cells 6 weeks after transplant. He continued to have stable disease after autologous transplant and started lenalidomide maintenance with aspirin prophylaxis.
At a subsequent follow-up visit after autologous transplant, a more comprehensive 21-gene-inherited thrombocytopenia panel was sent, and a heterogeneous mutation of ANKRD26 c.-128G>A in the 5′ untranslated region (5′ UTR) of chromosome 10 was identified. This was consistent with a diagnosis of ANKRD26-RT. The patient continues to have persistent thrombocytopenia in the range of 30–50 × 103 platelets. Given his significant family penetrance of thrombocytopenia, the patient and family were referred to genetic counseling for further evaluation. |
pmc-6589228-1 | A 28-year-old male semiprofessional basketball player presented to our orthopaedic practice with pain proximal and lateral to the left knee joint. He was treated for a tibial shaft stress fracture with tibial intramedullary nail (IMN) fixation 1 year prior at an outside hospital. He first noticed pain proximal and lateral to the left knee 2 weeks after surgery, and it persisted for the entire year. He had no complaints of pain in other joints and he denied any history of trauma since surgery. X-ray images taken during initial evaluation at our clinic showed no evidence of fractures, appropriate position of hardware, and presence of heterotopic bone at the proximal TFJ (). We ordered a CT scan to further evaluate the heterotopic bone and rule out implant problems including screw breakage, loosening, or prominent position. The CT scan demonstrated that the implant was properly positioned with no protruding or loosening screws (Figures –). We posited that the implant likely had little to no impact on the patient's pain. The scan also displayed no acute fracture consistent with the patient's reported level of pain (Figures –). However, it showed that the drill for the proximal locking screw may have penetrated through the tibia and into the fibula (). It also revealed proximal tibiofibular synostosis immediately distal to the proximal TFJ joint (Figures –) aligned with the bone reaming along the drill path (). Following a literature review and a discussion with our patient, we decided to proceed with nonoperative management. The patient declined a steroid injection. The patient's knee pain resolved without intervention, and he was able to return to playing semiprofessional basketball. At 13 months postoperation, he reported intermittent, mild pain on the medial side of the knee while playing basketball, but this did not limit his participation. At the final follow-up 16 months postoperation, he reported no pain. |
pmc-6589238-1 | A 14-year-old male with multiple psychiatric conditions, including bipolar disorder, attention deficit hyperactivity disorder, oppositional defiant disorder, posttraumatic stress disorder, and poor impulse control, presented to the primary care physician (PCP) with urinary frequency and nocturia of a month's duration. His medications included clonidine 0.1 mg three times daily, guanfacine 4 mg daily, bupropion sustained-release 150 mg daily, quetiapine 200 mg twice daily, and lithium 450 mg three times daily. Lithium was started when he was 5 years old. A year prior to his presentation, the lithium level was in the range of 0.9–1.1 mmol/L and serum creatinine was 0.78 mg/dL (estimated glomerular filtration rate or eGFR 86 ml/min/1.73 m2 based on the bedside Schwartz formula) []. Physical examination was unremarkable. Urinalysis revealed a specific gravity of 1.006, a pH of 6.0, but no hematuria or proteinuria. Blood tests showed a lithium level of 1.4 mmol/L, a TSH of 5.09 uIU/mL (normal 0.55–4.78 uIU/mL), a calcium of 11.1 mg/dL (normal 8.6–10.6 mg/dL), and a creatinine of 1.15 mg/dL. The PCP reduced the lithium dose to 450 mg twice daily and initiated levothyroxine for hypothyroidism. One week later, the lithium level reduced to 1.1 mmol/L. Over the next couple of weeks, the creatinine increased to 1.3 mg/dL. The PCP discontinued the lithium by tapering the dose over the next 3 weeks. Despite a very low lithium level of <0.1 mmol/L four weeks after discontinuation, the serum creatinine continued to increase, prompting a nephrology referral. At presentation to the nephrology clinic, physical examination was unremarkable. The creatinine was 1.46 mg/dL (), calcium 13 mg/dL, ionized calcium 7.1 mg/dL (normal 4.6 to 5.3 mg/dL), phosphorus 3.3 mg/dL, PTH 3 pg/mL (normal 9–52 pg/mL), 25 hydroxy vitamin D 18 ng/mL (normal 30–100 ng/mL), alkaline phosphatase 110 IU/L, sodium 135 meq/L, potassium 4.8 meq/L, chloride 103 meq/L, bicarbonate 24 meq/L, BUN 20 mg/dL, albumin 4.5 g/dL, and TSH 0.42 uIU/mL. Urinalysis showed a specific gravity of 1.008, a pH of 6.5, but no hematuria or proteinuria. Spot urine protein to creatinine ratio was 183 mg/g Cr (normal <200 mg/g Cr), and calcium to creatinine ratio was 530 mg/g Cr (normal <200 mg/g Cr). A renal ultrasound () showed bilateral punctate hyperechogenic foci, small cysts, and extensive hyperechogenicities in both cortex and medulla.
The patient was managed conservatively with hydration and avoidance of nephrotoxic agents. His serum creatinine peaked at 1.6 mg/dL (eGFR 38 ml/min/1.73 m2) followed by a gradual return to baseline of 0.78 mg/dL (eGFR 86 ml/min/1.73 m2) at one year. Hypercalcemia and hypercalciuria also resolved with a serum calcium of 9.6 mg/dL, a PTH of 32 pg/mL, and a urine calcium to creatinine ratio of 55 mg/g Cr. Polyuria and nocturia persisted. |
pmc-6589246-1 | A 74-year-old woman without relevant medical history presented with intermittent fever for more than one month. She also mentioned body weight loss about 18 kilograms and dry cough in recent half year. She did not travel in recent one year. She was admitted to local hospital and was transferred to our hospital for survey.
On physical examination at admission, her temperature was 37.3°C, heart rate 101 beats/min, respiratory rate 16 breaths/min, and blood pressure 142/54 mmHg. Pulmonary examination revealed accessory muscles use and bilaterally diffuse rales. Abdominal examination showed palpable spleen contour while palpation without muscles guarding or rebounding pain. The rest of the physical examination was unremarkable.
Laboratory examination revealed a total leukocyte count of 19,900 cells per μL and a platelet count of 133,000 per μL. Her alkaline phosphate was 481 U/L, serum total bilirubin 3.2 mg/dL, and lactate dehydrogenase 311 U/L. Her serum transaminases, internal normalized ratio (INR), and the activated partial thromboplastin time (aPTT) were all within normal range on initial presentation. Initial acid-fast stain of sputum, stool, and urine was negative. Chest X-ray showed multiple tiny nodules over bilateral lung field with alveolar infiltration over bilateral upper lobes, suspected as pulmonary tuberculosis with miliary tuberculosis (). Computed tomography from local hospital revealed multiple tiny nodules over bilateral lung fields, hepatosplenomegaly, and lymphadenopathy over omentum plus mediastinum, which indicated disseminated tuberculosis (Figures and ).
On the 3rd day of hospitalization, progressive orthopnea developed with paradoxical respiration (). Oxyhemoglobin saturation by pulse oximetry (SpO2) was 83% in room air. Emergent intubation was performed, and she was transferred to the respiratory intensive care unit (RICU) for resuscitation. Serial laboratory examination revealed leukocytosis with left shifting, INR > 10 ratio, aPTT > 100 seconds, fibrinogen 137.2 mg/dL, ferritin 980 ng/mL, triglyceride 110 mg/dL, sIL-2 receptor > 5000 pg/mL, and PaO2/FiO2 of arterial blood gas 55.1. Chest X-ray revealed newly developed symmetrically alveolar infiltration over bilateral lung field. Severe septic shock with acute respiratory distress syndrome was diagnosed. HLH was suspected due to splenomegaly, decreased fibrinogen, elevated ferritin, and sIL-2 receptor. Bone marrow biopsy was needed for definite diagnosis but held because her condition was unstable, and her family also hesitated due to high risk. Early goal-directed therapy was initialized with empirical antibiotics of Tazocin. Besides, ventilator setting was lung protective strategy. In addition, serum markers were negative for HIV-1, HIV-2, hepatitis B surface antigen, hepatitis C virus IgG antibody, influenza virus type A antigen, influenza virus type B antigen, IgG and IgM antibodies to cytomegalovirus, and herpes simplex virus. The Epstein-Barr virus (EBV) capsid antigen IgG antibodies were positive while EBV early antigen and nuclear antigen IgG antibodies and EBV IgM antibodies were all negative, indicating prior infection. We did not check EBV DNA from the patient's serum by polymerase chain reaction (PCR). On the 4th day, we arranged bronchoscopy for bronchoalveolar lavage fluid, and the result showed positive acid-fast stain 4+ and no bacteria were found. PCR for DNA of tuberculosis was also positive. We shifted antibiotics to antituberculosis medication of HERZ (isoniazid, ethambutol, rifampin, and pyrazinamide) plus levofloxacin immediately. On the 12th day, definite culture showed Mycobacterium tuberculosis which was sensitive to all antituberculosis medication. Bone marrow aspiration on this day confirmed diagnosis of HLH, and blood smear showed phagocytosis of macrophages without granular formation and negative acid-fast stain. We diagnosed HLH by fulfilling 5 out of 8 criteria of HLH in 2004. On the 31st day, she was extubated and finally she was discharged from our hospital after following for 73 days. |
pmc-6589247-1 | A 67-year-old woman presented (10/2017) with right side breast discomfort. Ultrasound demonstrated a 6.1 × 4.0 × 5.8 cm mass with no adenopathy, and biopsy showed an ER-negative, PR-negative, HER2-negative IDC. Staging computerized tomography (CT) showed bilateral pulmonary nodules (all < 3 mm), a 5 cm lower pole left kidney mass, renal vein thrombus, and innumerable sclerotic bone lesions (). Bone marrow biopsy confirmed metastatic breast cancer. In 12/2017, she started taking capecitabine. While follow-up evaluations revealed breast softening, CT after 3 cycles of capecitabine showed no response in distant sites. She developed two new pulmonary nodules, enlarging renal mass, new retroperitoneal lymphadenopathy, and worsening osteosclerotic metastasis; capecitabine was discontinued.
For a clinical trial, breast tissue underwent genomic testing and an activating SQSTM1-RET fusion mutation was revealed. Renal biopsy (to exclude a second primary malignancy) showed clear cell RCC. The genomic analysis of the renal biopsy did not yield mutations. Ipilimumab and nivolumab was started in 5/2018. She developed treatment-related rash, which was resolved with steroids. After 4 cycles of ipilimumab/nivolumab (7/2018), CT showed partial response with a resolution of lung nodules and shrinkage of the RCC primary tumor, enlarging adenopathy and worsening bony metastasis. A clinical breast exam was normal. Given mixed response, nivolumab maintenance was implemented and nab-paclitaxel was added. CT (10/2018) showed partial response with improved adenopathy, stable renal lesion, and stable bony lesions. The patient currently remains on this combination of nivolumab and nab-paclitaxel with her last CT () showing stable disease (current duration of therapy: 10 months). |
pmc-6589272-1 | A 64-year-old female presented to the emergency department secondary to shortness of breath, cough, and associated fever. She had a past history of chronic obstructive pulmonary disease, left upper lobe cavitary lung lesion, and microcytic anemia. The patient was on daily oral steroids for the last several years due to poorly controlled COPD and had recently been released from the hospital two weeks before for a left lower lobe pneumonia. At the time of her prior admission, she was started on vancomycin and aztreonam for her pneumonia and was ultimately discharged on levofloxacin for ten days.
During her emergency department course, her chest radiograph revealed a worsening left lower lobe infiltrate which was later confirmed on computed tomography of the chest. The patient had worsening hypoxia during her course and was eventually placed on BIPAP therapy, and she was started on intravenous vancomycin, levofloxacin, and fluconazole for a presumed hospital-acquired or fungal pneumonia given her recent hospital admission and cavitary lung lesion. Prior to admission, she had no physical exam findings to suggest a fungal infection. She was admitted to the hospitalist service for further evaluation and management.
While hospitalized, the patient continued antibiotic and antifungal therapy, and on day two, aztreonam was added due to a worsening clinical picture. The following day, the patient underwent consultations from infectious disease, pulmonology, and cardiothoracic surgery due to her worsening clinical status and pneumonia with associated cavitary lung lesion. Following consultations, the patient underwent a fiberoptic flexible bronchoscopy with bronchoalveolar lavage which showed a large mucous plug obstructing the left main bronchus but no associated lesions. Cultures from the bronchoalveolar lavage eventually grew Nonomuraea solani, Candida glabrata, and Candida dubliniensis.
Following a protracted hospital course of nine days, the patient was discharged home with cefpodoxime 400 mg twice a day for ten more days and was instructed to follow up with infectious disease within the next two weeks. However, the patient eventually presented to the emergency department one month later with a left-sided empyema status after wedge resection of the cavitary lesion that grew Corynebacterium amycolatum and Staphylococcus hominis spp. on cultures. |
pmc-6589283-1 | A 52-year-old female, with an unremarkable medical history, with the exception of lumbar herniation, was subjected, under general anesthesia and in a genupectoral position, to L4-L5 discectomy. During the annulus resection, the no. 11 scalpel blade broke and it was retained within the disc space. Attempts to remove the foreign body were performed under fluoroscopy. However, the broken blade migrated towards the abdominal cavity and viscera. Immediately, a CT angiography scan was performed, in order to locate the broken instrument (). CT angiography revealed that the main vessels were intact, while the broken surgical knife was located anterior to the lumbar spine at the L4/L5 level and to the left of the aorta.
The patient remained during this procedure stable, at all times. Urgently, the patient was placed in a supine position and a robot-assisted laparoscopy was initiated. Under general endotracheal anesthesia, the da Vinci® platform was brought to the operating table between the patient's legs. The camera port was inserted inferior and to the right of the umbilicus using the Hasson technique. Under direct vision, two additional robotic arm trocars were inserted at the right and left iliac fossa, respectively. Once the robot was docked, an exploration of the peritoneal cavity was performed. With the patient tilted to the right, the small intestine was transferred to the right abdominal cavity so that the retroperitoneum below the level of the left kidney could be exposed. The retroperitoneum was carefully dissected using bipolar Cadier forceps on the left arm and monopolar scissor and hook on the second arm. After the access to the abdominal aorta was gained, the broken scalpel was identified in close distance to the aorta and the left common iliac artery with no signs of active bleeding. The scalpel was slowly removed with extreme care not to traumatize the vessels (). A final abdominal exploration was performed before the platform was removed (). The patient was deintubated without complications and was transferred to the recovery room.
The patient had an uneventful hospitalization and was discharged at the 3rd postoperative day. At follow-up, 2 years after the operation, she remains without any signs or symptoms of disease. |
pmc-6589302-1 | One female patient, aged 49 at the time of the first visit, without any systemic disease (ASA-1 following the classification proposed by the American Society of Anesthesiologists) presented referring an episode of acute abscess in the region of 1.1, 1.2, and 1.3 that was treated with systemic administration of Amoxicillin 875 mg+Clavulanate 125 mg twice a day for six days.
The clinical examination revealed the presence of one isolated periodontal pocket (measuring 13 mm) in correspondence of the palatal aspect of 1.2, in the presence of one enamel alteration (PRG) (). Mild pain was associated with percussion of 1.2, and the element was vital, without any caries. Full-mouth clinical evaluation excluded the presence of generalized periodontitis. Adjacent sites did not show the presence of any pathological periodontal pocket, with probing depth less than 4 mm in all sites. Radiographic examination showed a deep intrabony defect distal to 1.2 ().
Differential diagnosis reasonably excluded the presence of a vertical root fracture (VRF) since the tooth was vital and no history of trauma was reported. The treatment option was to elevate a palatal flap to allow the debridement of the pocket, to remove physically the etiologic factor (the PRG) maintaining tooth vitality, and to stimulate periodontal regeneration by using enamel matrix derivative (EMD).
After local anesthesia on the vestibular and palatal side with Articaine 4%+epinephrine 1 : 100.000, a horizontal incision was performed on the palatal aspect preserving interdental papilla between 1.1 and 1.2 and between 1.3 and 1.4 (). The papilla between 1.2 and 1.3 was separated from the vestibular portion and reflected in order to directly visualize the PRG and the defect (). After the debridement of the defect, by removing the granulation tissue, the PRG was smoothed by the use of a diamond bur under abundant irrigation of sterile saline solution (Figures and ). Following the instructions provided by the manufacturer, tooth surface was conditioned for two minutes with 24% EDTA gel (Straumann® PrefGel®, Straumann AG, Basel, Switzerland) and, after its removal through copious irrigation with sterile saline solution, EMD (Straumann® Emdogain®, Straumann AG, Basel, Switzerland) was placed in the defect (). The flap was then sutured with single 5/0 and 6/0 sutures (ETHILON®, Ethicon Inc., Johnson & Johnson, Piscataway, NJ, United States) (). The patient was advised to avoid any trauma or traction in the region of surgery and not to consume hard food during the first five days. Ibuprofen 400 mg was prescribed twice a day for three days for inflammation control and pain relief. Healing completed without any adverse event.
Clinical and radiographic examination 12 months after surgical intervention revealed a complete healing with bone regeneration in the site of the defect (Figures and ). |
pmc-6589302-2 | One female patient, aged 36 at the time of the first visit, without any systemic disease (ASA-1 following the classification proposed by the American Society of Anesthesiologists) presented referring mild pain sensation while touching with the tongue the palatal-gingival margin of 1.2.
The clinical examination revealed the presence of one deep narrow isolated periodontal pocket (PD = 10 mm) in correspondence of the palatal aspect of 1.2, in the presence of bleeding on probing, no plaque accumulation, and one PRG (). Full-mouth clinical evaluation excluded the presence of periodontitis. Radiographic examination showed no evidence of one visible intrabony defect (). The tooth was endodontically treated and did not show signs and symptoms of a lesion of endodontic origin.
The treatment was by a regenerative approach, using EMD, on the palatal aspect, removing mechanically the PRG from the root (Figures and ), following the technique described in detail before.
After one year from surgical intervention, clinical examination showed a physiological probing depth (less than 4 mm) in the site of the surgery and the absence of symptoms (Figures and ). |
pmc-6589305-1 | A 38-year-old nonsmoking female presented with left-sided chest pain and dyspnea for three consecutive days. The patient was admitted to Masih Daneshvari Hospital on September 19, 2016. She had no history of fever, weight loss, and arthralgia or skin rash. Her blood pressure was 110/70 mmHg, pulse rate was 85, temperature was 37.5°C, and respiratory rate was 28. The breath sound was decreased in the left lower zone of the lung, and other physical examinations were unremarkable.
Chest radiograph manifested two pulmonary nodules and basal atelectasis in the left lower lobe with pneumothorax (). Chest CT scan exhibited collapsed consolidation of the left lower lobe with pneumothorax and the presence of relatively two separated small-sized cystic lesions within the collapsed segment ().
The laboratory tests revealed the increase in WBC count with predominantly neutrophils; the erythrocyte sedimentation rate and C-reactive protein were elevated. The nitroblue tetrazolium (NTB) blood test was at the 99% accuracy level. Anti-nuclear antibodies (ANAs), anti-neutrophil cytoplasmic antibodies (C-ANCA and P-ANCA), and anti-double-stranded DNA (anti-dsDNA) were negative; in addition, the anti-HIV antibody test was negative. Transparently, the pulmonary function tests showed moderately restriction.
Lung lesion resection for two intraparenchymal pulmonary nodules and cavitary lung lesion in the left lower lobe was performed; furthermore, the histological examination showed some epithelioid and giant cell granulomas with necrotizing arteritis in conjunction of a large area of necrosis (Figures and ). Acid-fast staining, periodic acid-Schiff (PAS) staining, and Gomori methenamine-silver (GMS) staining were negative; therefore, the ultimate diagnosis was necrotizing sarcoid granulomatosis. The prognosis of NSG is favorable, and medical treatment is usually not required. As a matter of fact, in our case report, the patient recovery conspicuously occurred; nevertheless, in some cases, treatment with corticosteroids is quite essential. |
pmc-6589315-1 | A 17-year-old young female from Kathmandu, Nepal, presented to the emergency department of the Institute of Medicine (IOM) with a generalized painful skin rash along with extensive blistering with mucosal involvement for one day (Figures –). She had history of cough, sore throat, and fever few days prior to the appearance of rash for which she had taken azithromycin orally. On the 3rd day of oral medication, she developed rash which was nonpruritic and painless. There was eruption of bumps starting from the trunk and spreading all over the body. Her eyelids and lip were swollen, and this was later associated with blistering and crusting. Around 20% skin detachment of body surface area was involved. The patient was clinically diagnosed as Stevens–Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap with tracheobronchitis. To find out the cause of SJS-TEN overlap, azithromycin was stopped. After 2 days of stopping azithromycin, her clinical symptoms did not improve, rather new lesions were seen. Then, the bedside cold agglutination test was done by cooling the blood taken in EDTA vial at 4°C. Before cooling, the blood formed smooth coating of the tube. After incubation at 4°C for 3 minutes, macroscopic hemagglutination was observed as cell clumping in thin film of blood that clinged to the tube (). The clumping disappeared when the tube was warmed at 35.8°C and reappeared at 4°C (). So, infective cause of SJS-TEN overlap was suspected, the most common cause being Mycoplasma pneumoniae. Blood was sent for the investigation of mycoplasma IgM and IgG antibodies. Also, serial dilutions of the patient's serum were mixed with an equal volume of 0.2% washed human O group erythrocytes, and clumping was observed till titer of 1 : 128 dilution after leaving at 4°C overnight (). The clumping is dissociated at 37°C ().
The complete blood count revealed total leukocyte count of 8000/μl with lymphocytes of 60% with elevated ESR (70 mm/hour). Her liver enzymes and serum creatinine were normal. Test for syphilis (RPR and TPHA), human immunodeficiency virus (HIV), hepatitis B and C viruses, herpes simplex virus, Epstein–Barr virus, influenza A/B, and Chlamydia pneumoniae were negative.
She was restarted with drug azithromycin and added hydrocortisone, paracetamol, betadine gargle, mupirocin, and ciprofloxacin ointment. Punch biopsy of her skin demonstrated subepidermal inflammation with necrotizing infundibular epithelium and necrotic keratinocytes consistent with SJS. Mycoplasma IgM antibody report was positive (2550 U/ml), which suggested the current infection and confirmed our diagnosis. The same treatment was continued and her clinical symptoms improved (). |
pmc-6589433-1 | An 81-year-old female patient visited the dental clinic with a foreign body sensation under the tongue and dry mouth on February, 2018. Papillary thyroid carcinoma was diagnosed, and a total thyroidectomy was performed in June 2003. Other underlying diseases were hypertension and chronic kidney disease. A sialolith was found in the right mandibular submandibular duct in the examination, and a sialolithotomy with sialendoscope was planned (Fig. ). Preoperative and postoperative clinical symptoms of the surgical site were recorded.
Extraoral and intraoral disinfection was done with a betadine-soaked cotton ball. After disinfection, the oral cavity was thoroughly washed with sterile saline. Local anaesthesia was performed on the tongue and mouth floor with 2% 1:100,000 lidocaine HCl-epinephrine, and the tongue was sutured using 3-0 silk to elevate it and secure the field of view of the surgical site. Through a microscope, the entrance of the right submandibular gland’s duct was confirmed, and a #0000 probe was carefully and sequentially inserted (Fig. ) along the duct and expanded to a #3 probe. To relieve the stenosis of the duct orifice and allow access to the endoscope inside the duct, the orifice was expanded using a dilator. Sialendoscopy was done under saline irrigation, and the stone was confirmed. Using a three-wire basket, the stone was removed from the duct (Fig. ). The size of the ductal orifice was smaller than that of the stone; a 0.1-cm incision was made at the entrance of the duct. After removal of the stone, the endoscope was reinserted to confirm that all the in situ stones were removed (Fig. ). Then, the duct system was washed with a steroid solution. There was no postoperative bleeding or abnormality such as oedema and pain. Daily dressing and clinical examination to check for complications, such as infection, was performed; there were no unusual side effects after the operation. |
pmc-6589433-2 | A 66-year-old man visited the dental clinic because of the blockage of salivary duct of the right mandibular submandibular gland (Fig. ) on June, 2018. From 7 to 8 years ago, there was a swelling and pain in the lower neck while eating food. Sialolithotomy was performed in other hospitals 4 to 5 years ago, but failed because of unstable movement of the stone during surgery. There was no systemic abnormality.
Extraoral and intraoral disinfection was done with a betadine-soaked cotton ball. After disinfection, the oral cavity was thoroughly washed with sterile saline. The patient underwent general anaesthesia through a nasotracheal intubation, and the tongue was sutured using 3-0 silk. The operation procedure was the same as the previous case (Fig. ). There was no postoperative bleeding or abnormality such as oedema and pain. Daily dressing and clinical examination to check for complications, such as infection, was performed; the patient was discharged the next day without any discomfort. |
pmc-6589876-1 | A 54-year-old black South African woman was admitted to a local hospital in Scotland following several months of episodic night sweats, feverishness, and unexplained weight loss. She then developed frequent episodes of right-sided facial and arm “twitching” with preserved consciousness. These episodes progressively became constant and were exacerbated when she tried to talk. They persisted through sleep. She also complained of progressive breathlessness at rest. She recalled being treated for TB over 20 years previously, in the late 1980s, when she had been living in South Africa. Further details of this were unavailable. Her past medical history also included autoimmune neutropenia and mild anemia that had required no intervention. She had no significant family history. She worked in healthcare and did not smoke tobacco or drink alcohol. Although she lived in the United Kingdom, she visited South Africa each year.
Clinical examination revealed a persistent, low-grade pyrexia and a purpuric rash. She was hemodynamically stable, alert, and able to follow commands. She was tachypneic, and on occasion she required inhaled oxygen support to maintain oxygen saturation at 92–94%. She had continuous, semirhythmic contractions in the muscles on the right side of her face. These movements extended from her mouth to her eyebrows (Additional file 1: Video S1). She had no other neurological deficits, and the remainder of her general examination was unremarkable. A summary of various investigations carried out and the differential diagnoses that were considered is shown in Table .
An in-depth timeline of the patient’s inpatient course is provided in Additional file : Table S1. She was initially suspected of having meningococcal septicemia, owing to her purpuric rash and pyrexia, and she was commenced on 2 g daily of intravenous (IV) ceftriaxone. Her condition then deteriorated rapidly into acute respiratory failure with new bilateral perihilar opacification evident on chest imaging (Fig. ), requiring a short period of intensive care for noninvasive ventilation. Ceftriaxone was stopped following negative blood culture results and suspicion that marked thrombocytopenia (lowest platelet count 3 × 109/L) explained her purpura. IV co-trimoxazole 120 mg/kg daily was commenced to treat Pneumocystis pneumonia (PCP), and she also received 2 g of meropenem four times daily for 7 days for ongoing pyrexia. She had blood, urine, sputum, and cerebrospinal fluid cultures for mycobacteria. These each isolated Mycobacterium tuberculosis. Her pancytopenia and hyperferritinemia prompted bone marrow investigation. A marrow aspirate revealed macrophage activation and hemophagocytosis, supporting a diagnosis of HLH (Fig. ). A trephine biopsy identified numerous caseating epithelioid granulomata, supporting a diagnosis of TB. Her twitching was investigated with magnetic resonance imaging (MRI) of the brain (with and without contrast). This revealed four small contrast-enhancing foci within the parenchyma of the brain. These were interpreted as likely tuberculomas. One of the lesions was located on the motor strip of her left hemisphere (Fig. ). This location corresponds somatotopically with her right-sided abnormal facial movements. A clinical diagnosis of EPC was made. Electroencephalography (EEG) revealed muscle artefact but was otherwise normal (Fig. ). Given the widespread evidence of tuberculous disease, a diagnosis of disseminated TB was made.
10 mg boluses of IV diazepam were initially given and failed to reduce her continuous focal seizure activity. IV levetiracetam 750 mg daily was then added but demonstrated minimal effect. She was therefore given 10 mg boluses of buccal midazolam and loaded with IV phenytoin at a dose of 17 mg/kg/day. When these were unsuccessful in stopping the seizures, IV levetiracetam was uptitrated to 2500 mg daily, and the IV phenytoin was uptitrated to 550 mg daily. She was also commenced on 1 mg of regular oral clonazepam daily. Her seizures stopped while she was receiving these three medications. She had experienced 7 days of unbroken focal seizure activity in total. A residual right-sided Todd’s paresis was noted, which soon resolved. She required high doses of oral antiepileptic drug (AED) polytherapy to maintain seizure freedom (clobazam 10 mg twice daily, levetiracetam 1500 mg twice daily, and phenytoin 350 mg once daily). Reducing or changing her AEDs proved difficult, with attempts to do so resulting in the reemergence of EPC seizures. Interval MRI of the brain with contrast demonstrated an increased number of enhancing parenchymal foci in keeping with tuberculomata scattered throughout her brain (Fig. ).
The patient was commenced on a 12-month regimen of oral anti-TB therapy as 6 months of once-daily isoniazid 300 mg, rifampicin 600 mg, pyrazinamide 2 mg, and ethambutol 1000 mg, then 10 months of the isoniazid and rifampicin. Eight weeks of IV etoposide 150 mg/m2 were given for HLH (twice weekly for the first 2 weeks, then once weekly for weeks 3–8). Long-term oral steroids were given alongside etoposide as a dexamethasone taper from 18 mg once daily over 8 weeks. Following this, prednisolone was commenced at a 20-mg-daily dose for the duration of anti-TB therapy. Overall, the patient had remained hospitalized for 6 months due to ongoing episodes of unwellness with fevers, respiratory distress (requiring a second brief admission to intensive care, for invasive ventilation), physical mobility problems, and difficulties with memory and speech. During this period, repeated sets of cultures, including blood, urine, sputum, viral throat swabs, blood-borne viruses, and repeat imaging, including of the chest, abdomen, pelvis, and brain, failed to identify a cause of her ongoing pyrexia. A repeat bone marrow aspiration, however, demonstrated ongoing hemophagocytosis, and her ferritin levels remained markedly elevated. Her pyrexia was therefore believed to be a result of ongoing active HLH. She was commenced on long-term oral cyclosporine 250 mg twice daily with a target of normalization of the ferritin levels, which took 12 months. Prednisolone was tapered slowly during this period. The result of her immunologic screening for primary HLH with a granule release assay and perforin expression studies was negative, and she therefore did not undergo further HLH genetic study in light of these results and the increased likelihood that she had HLH secondary to TB. To investigate the possibility of an underlying susceptibility to disseminated TB, the patient had a cytokine profile (including interferon-γ, interleukin 12, and tumor necrosis factor) and GATA2 gene mutation screening, which returned normal results. A biopsy of the lesions in the brain was considered in order to help further characterize the pathological etiology of her seizures, but the risks were believed to outweigh the benefits because she had responded to AEDs and was receiving treatment for both TB and HLH.
Upon discharge, she was seizure-free and experienced a gradual improvement in function, although she remained unable to work and required intensive outpatient functional rehabilitation. She had negative repeat fluid culture results for M. tuberculosis, and she was no longer pyrexic or pancytopenic, although her ferritin remained elevated. Her first 12 months of outpatient recovery, during which she completed oral anti-TB treatment, were complicated by five brief readmissions to the hospital with relapses of EPC seizures. Interval MRI scans of the brain during this period demonstrated a gradual reduction and resolution in the number of tuberculomata and progressive atrophy of the left temporal, parietal, and occipital lobes. Her seizure frequency gradually improved over the subsequent 3 years to one or two events per year, and she tolerated stopping phenytoin and remaining on clobazam 10 mg twice daily and levetiracetam 1500 mg twice daily. The patient’s care required frequent collaboration between neurology, infectious disease, and hematology specialists. |
pmc-6589960-1 | The case is of an 18-year-old Filipino male resident of San Joaquin Valley with history of fever, body ache, fatigue, and dry cough 4 month prior to admission (PTA). The patient was a field worker who was previously healthy. These symptoms persisted for 3 weeks with new-onset generalized maculopapular rash on his hands, forearms, and shins. A primary care visit resulted in a diagnosis of viral infection and he received antihistamines. His rashes resolved 3 months PTA, with his cough and fatigue persisting.
The patient started practicing karate in order to improve his energy level. Approximately 2 months PTA, he traumatized both of his elbows during practice, without any apparent integumentary disruption. He noted painful edematous elbows over the next 2 weeks without any improvement. He was seen in the emergency department diagnosed with bilateral elbow bursitis and sent home on oral antibiotics. On the day of admission, without any improvement he noticed skin breaks over both elbows with purulent discharge bilaterally ( and ). Initial imaging revealed bilateral osteomyelitis of both olecranon ( and ). This was confirmed with whole body scan during his hospitalization. He was also found to have a large left lower lobe infiltrate ( and ). Culture of incision and drainage of both elbows demonstrated Coccidioides immitis. Subsequently, histopathology showed spherules with endosporulation. Coccidioidal immunodiffusion, IgM, and IgG were positive with complement fixation titer of 1:256.
He was started on oral 1000 mg fluconazole daily. The treatment was switched to amphoteric B liposomal complex (ABL). His elbow underwent additional osseous debridement and skin grafting. ABL was continued for 4 more weeks and transitioned to oral 1000 mg fluconazole daily. The patient was lost to follow-up, last seen 2 years after admission with well-healed wound and full function of bilateral elbows, cleared chest X-ray with coccidioidal serology titers of 1/32. |
pmc-6589960-2 | A 43-year-old previously healthy Hispanic male resident of Bakersfield with a subacute cough and no other described symptoms suffered a right anterior tibial injury secondary to a fall from a forklift. At an urgent care, there was no evidence of bony injury or a break in the integument; however, swelling, erythema, and pain were appreciated at the site of injury.
Subsequent persistence of swelling, erythema, and pain resulted in a visit to the Kern Medical Emergency Department 30 days after initial trauma. The patient was noted to have cough and subjective fever, as well as pretibial swelling and erythema with no break in the epidermis. Imaging demonstrated right lower lobe pneumonic infiltrate and a lytic lesion in the right tibia ( and ). Operative management included saucerization and debridement. Coccidioidal serology was negative for immunodiffusion IgM and positive for immunodiffusion IgG with a complement fixation titer of 1:32. Intraoperative cultures were positive for Coccidioides species. The patient was started on oral 800 mg fluconazole daily. The patient continues to follow-up in clinic with full resolution of the tibial wound and last complement fixation titer of 1:8. |
pmc-6590094-1 | A 71-year-old never-smoker man was diagnosed by cervical lymph-node dissection with advanced lung adenocarcinoma with more than five metastases, including brain, bone, and lymph nodes (cT1cN3M1c based on the 8th edition of the TNM staging system) at 62 years of age (Fig. ). His performance status (PS) was 0. He received whole-brain radiotherapy (WBRT) and one cycle of chemotherapy consisting of cisplatin and vinorelbine (Fig. ). He was referred to our hospital and received six cycles of carboplatin and pemetrexed with good partial response sustained for 1.5 years. He developed first local progression of primary lung cancer of the left lower lobe and again received four cycles of carboplatin and pemetrexed with stable disease for more than 1 year. After developing a second local regrowth, he was enrolled into the industry-initiated phase II clinical trial (CDLK378A2203) with ceritinib as a cervical lymph node specimen was positive for ALK rearrangement by fluorescence in situ hybridization (FISH). Eight months later, he developed a third local progression and then received 12 cycles of pemetrexed with partial response for 10 months, followed by treatment with alectinib because of its approval in Japan. Five months later, he developed a fourth local regrowth and received an additional six cycles of pemetrexed with minor response for 5 months. As magnetic resonance imaging (MRI) of the brain showed no abnormality, positron emission tomography (PET)-computed tomography (CT) only showed hypermetabolic activity of the enlarged primary lung cancer, and he demonstrated easy fatigability associated with long-term treatments, he decided to undergo sublobar resection and resection of accessible left hilar and left main bronchus lymph nodes. The pathological stage was ypT1aN0M0, stage IA with therapy effect grade 2a. RNAs extracted from a resected tumour showed a 75-bp insertion between exons 24 and 25 besides EML4-ALK (Echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase) rearrangement. Despite no further treatment, he is still alive for more than 3 years without recurrence. |
pmc-6590132-1 | This patient was a 52-year-old woman with AN-BP, who had been involved in self-induced vomiting and laxative abuse of more than 200 tablets per day for 20 years. She had been administered supportive psychotherapy and symptomatic treatment by psychiatrists including hospitalization at 13 instances. She visited us for evaluation of prominent ascites and treatment of AN-BP. On physical examination, epigastric vein dilatation and striae cutis distensae of the swelling abdomen, which was filled with ascites, were seen. Blood test results indicated hypoalbuminemia, increased hepatic and biliary enzyme levels, an increased creatinine level, hypokalemia, and anemia (Table ). The abdominal ultrasonogram showed findings of chronic hepatitis: an irregular liver surface, a dull margin, and heterogeneity of the parenchyma. On the computed tomography scan, a gastro-renal shunt was found, although no varices were detected. Hence, portal hypertension due to liver cirrhosis was suspected. Therefore, we performed a liver biopsy. However, only slight pericellular fibrosis was observed without evidence of liver cirrhosis (Figure a,b). Liver fibrosis was also proven by the increase in liver fibrosis makers of type IV collagen 7S (8.5 ng/mL) and hyaluronic acid (400 ng/mL). On psychological testing, depression and anxiety were remarkable (Table ). During inpatient care, we provided nutritional treatment and diuretic drugs to control ascites. After her general state improved, an inpatient cognitive behavioral treatment was initiated. She was discharged 2 months after admission because the ascites almost disappeared and her body weight increased by approximately 3 kg. However, serum levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (γ-GTP), and creatinine were not significantly improved. During our 3-month follow-up, her ascites were well controlled with diuretic drugs, and we instructed her to limit her daily activities. As for the aforementioned serum levels, no changes were observed. |
pmc-6590132-2 | This patient was a 38-year-old woman with AN-BP for 15 years. In the first 14 years, her purging behavior only included binging and self-induced vomiting. Laxative abuse started over the last year, and she consumed 100 tablets or more daily. Before our intervention, she had been hospitalized repeatedly by physicians and surgeons for infusion therapy in order to improve dehydration and electrolyte imbalance. She was admitted to our department because of severe malaise, systemic edema, and liver malfunction. High levels of ALP and γ-GTP were remarkable on the blood test (Table ). ALP-2, an ALP isozyme derived from the liver was remarkably raised to 72%. Hypoalbuminemia, increased AST and ALT levels, high creatinine level, and anemia were also indicated. An abdominal ultrasonogram confirmed ascites and a chronic hepatitis pattern. On endoscopic examination, we observed esophageal varices, the gross appearance of which was categorized as located on the lower third of the esophagus (Li), straight form (F1), blue color (Cb), and slight red cherry sign (RC1), according to the Japanese Research Society for Portal Hypertension (JRSPH) classification. No abnormalities were detected on computed tomography. Liver biopsy results revealed slight pericellular fibrosis, but neither bridging fibrosis nor liver cirrhosis (Figure c,d). The increase in liver fibrosis markers M2BPGi (1.34 COI), type IV collagen 7S (530 ng/mL), and hyaluronic acid (721 mg/dL) was observed. As for inpatient care, diuretic drugs (spironolactone, 25 mg/day and tolvaptan, 7.5 mg/day) were administered in order to control systemic edema and ascites. After 2 weeks of hospitalization, she was discharged with decreased edema and ascites, but there was no change in AST, ALT, ALP, and γ-GTP levels. Four months later, neither the recurrence of edema and ascites nor the improvement in hepatic enzyme level was seen. Coexistence of a borderline personality disorder complicated her treatment. We performed psychotherapy based on motivational interviews. |
pmc-6590132-3 | This patient was a 29-year-old woman with AN-BP, who had been involved in self-induced vomiting and laxative abuse of more than 200 tablets per day for approximately 10 years. We provided her with supportive psychotherapy and psychoeducation at our outpatient clinic, along with impatient care as needed. Anxiety, depression, and difficulties in self-identity and expression were distinctive on her psychological test (Table ). Her condition was also complicated with chronic kidney disease; her serum creatinine level was generally 1.5–2.0 mg/dL. She had a medical history of ruptured esophageal varices, for which endoscopic variceal ligation was conducted. The varices ruptured again, and inpatient care was given in the Gastrointestinal Department. On physical examination, epigastric vein dilatation and hemorrhage were observed. The blood test results on admission are presented in Table . The enhanced computed tomography scan revealed gastroesophageal varices and a gastro-renal shunt. The endoscopic examination showed gastric varices, classified as gastro-renal shunt accompanied with gastric varices (Lg-f), enlarged and tortuous (F2), white color (Cw), and no red color sign (RC0), and esophageal varices categorized as Li, F1, Cb, and RC1 according to the JRSPH classification. On liver biopsy, there was only slight pericellular fibrosis and again no finding of liver cirrhosis (Figure e,f). A high level of M2BPGi (1.56 COI) was also confirmed. On admission, 4 U of red blood cells were infused, and endoscopic variceal ligation was performed. Her hemoglobin level recovered from 5.6 to 10.4 mg/dL, and hemostasis was confirmed on second-look endoscopy. The serum creatinine level decreased from 2.47 to 1.02 mg/dL with hydration. Hyperkalemia also improved in accordance with the recovery of renal function, and consequently, returned to hypokalemia of 2–3 mmoL/L, which was the usual level for her. Psychosomatic doctors provided supportive psychological interviews, and her AN-BP state remained stable. She was discharged after 11 days of hospitalization. Subsequently, she underwent balloon-occluded transfemoral obliteration for the gastric varices. Eleven months later, dissipation of the gastric and esophageal varices was revealed on endoscopic follow-up. |
pmc-6590484-1 | Our case is that of a 43-year-old primiparous woman. She was diagnosed with Behçet disease at the age of 34 years; she presented with recurrent oral ulceration, recurrent genital ulceration, and pseudofolliculitis with characteristic acneiform nodules. She had no eye lesions. Her condition was well controlled with prednisolone (PSL) 8 mg/day oral dose. She fell pregnant spontaneously and visited our perinatal center for her prenatal care. As she was detected with hypertension at her first visit, we recommended that she check her blood pressure regularly at home. She developed fever, oral ulcers, and arthralgia at 16 weeks of gestation, due to which a relapse of Behçet disease was suspected. Because her condition was controlled well with PSL, the daily oral dose of PSL was increased from 8 mg to 10 mg. At this time, ulceration and pain of the vulva were not observed. She visited our outpatient clinic for a regular prenatal examination at 18 weeks and 4 days of gestation, which was when an ulcer localized in the vagina was incidentally observed (). The ulcer was painless and the patient had no symptoms in the vulva either (). Chlamydial and gonococcal infection were not detected, and cytology of the vaginal wall showed no findings suggesting malignancy. As the other symptoms of Behçet disease were resolved after the dose of PSL had been increased, the same dose was maintained. At the next prenatal care visit, at 22 weeks and 4 days of gestation, the vaginal ulcer had disappeared and other symptoms of Behçet disease were not seen. At 28 weeks and 0 days of gestation, atypical genital bleeding and vaginal ulcer recurrence were observed (). At the same time, recurrence of the oral ulcer and arthralgia as well as a mild increase in the fever were observed; based on these symptoms, she was diagnosed with a relapse of Behçet disease and the daily dose of PSL was increased from 10 mg to 15 mg. The vaginal ulcer disappeared at 29 weeks and 2 days of gestation (). Thereafter, there was no relapse of the symptoms of Behçet disease.
At 33 weeks and 0 days of gestation, her blood pressure had increased, due to which she was admitted to the hospital. She was diagnosed with severe preeclampsia superimposed. Induction of labor was conducted but it was not effective, and we performed cesarean section at 37 weeks and 6 days of gestation. She delivered a male infant weighing 2002 g. There were no complications in the postoperative course, and she was discharged with her baby at 7 days after cesarean section. No recurrence of the vaginal ulcer and other symptoms of Behçet disease were seen on subsequent follow-up examination at 1 month after delivery.
The patient was explained about the possibility of publishing this case as a case report and the accompanying images. She provided consent. |
pmc-6590497-1 | A 39-year-old woman with a history of borderline personality disorder, bipolar disorder, and depression presented to the emergency department (ED) intubated after a suicide attempt by hanging. Her partner at the bedside estimated the patient had been hanging for roughly ten minutes prior to being discovered and also suspected concurrent overdose on home alprazolam. Admission vital signs and exam were remarkable for HR 112 bpm, BP 90/60 mmHg, pupils 6 mm and reactive to light, GCS 3, and neck erythema.
The initial laboratory workup was notable for white blood cell (WBC) count 18.6 K/μL, troponin 0.24 ng/mL (peak of 1.78 ng/mL), and arterial blood gas (ABG) on FiO2 100% demonstrating pH 7.201, pCO2 36 mmHg, pO2 231.6 mmHg, bicarbonate 13.8 mmol/L, anion gap 8 mmol/L, and lactate 0.86 mmol/L. Electrocardiogram demonstrated sinus tachycardia with no ST-segment or T-wave changes. Trauma imaging in the ED included contrasted tomography (CT) of the head without contrast, CT cervical spine, and CT angiography of the neck, which were negative for cervical spine injury, cervical artery dissection, or brain hemorrhage or infarct. However, chest X-ray (CXR) demonstrated diffuse, bilateral pulmonary edema (). The patient was also found to be hypotensive and required vasopressor support. She was transferred to the intensive care unit (ICU) for further management of NPPE complicated by acute hypoxic respiratory failure, severe nonanion gap metabolic acidosis, and presumed type II non-ST-segment elevation myocardial infarction (NSTEMI).
Shortly after arrival to the ICU, transthoracic echocardiogram (TTE) was obtained which demonstrated a left ventricular (LV) ejection fraction (LVEF) 40-45% and LV wall-motion abnormality consistent with midventricular form of Takotsubo syndrome (TTS, ). Intravenous diuresis with furosemide was administered with satisfactory response. Additionally, the patient's metabolic derangements corrected with supportive measures, sedation and vasopressor support were weaned, and she was successfully extubated within 48 hours of admission without any adverse neurologic sequelae. She was successfully started on metoprolol tartrate 25 mg twice daily with goal conversion to succinate formulation and uptitration to therapeutic effect as hemodynamically tolerated. The patient was transferred for continued inpatient psychiatric evaluation and management. Follow-up echocardiogram three months after initial TTE demonstrated full recovery of LV function. |
pmc-6590497-2 | A 64-year-old man without history of cardiac disease was admitted for debridement of anterior mandibular osteomyelitis. Preoperative cardiopulmonary assessment was unremarkable, and the patient tolerated the procedure well. Shortly after extubation, he unexpectedly demonstrated labored breathing with inspiratory stridor. Laryngospasm was visualized by direct laryngoscopy, and the patient ultimately required reintubation. Appropriate endotracheal tube placement was confirmed by chest rise and CO2 monitor, yet the patient was found to have oxygen saturation of 90% on 100% FiO2. He subsequently became severely hypotensive requiring vasopressor support and ICU admission.
Upon arrival, pulmonary auscultation revealed bilateral rales, and frothy secretions were suctioned via the endotracheal tube. The initial workup was most significant for troponin elevated to 1.76 ng/mL (normal < 0.021 ng/mL), electrocardiogram with new, diffuse, deep T-wave inversions, CXR demonstrating flash pulmonary edema, and bedside TTE demonstrating severely reduced LVEF of 20% with diffuse akinesis involving the mid-to-apical segments of the LV with basal sparing. Right ventricular function was normal. Coronary angiogram was negative for acute coronary syndrome or any significant obstructive coronary artery disease.
These findings suggested TTS as the etiology of patient's acute cardiogenic shock. He responded well to intravenous diuretic therapy and was successfully extubated and weaned from all inotropic/vasopressor support within five days of ICU admission. This clinical improvement corresponded with radiographic resolution of his pulmonary edema. Seven days after initial presentation, repeat TTE demonstrated EF improvement to greater than 40% with distal anteroseptal periapical and distal inferoseptal hypokinesis and akinesis. At ambulatory follow-up appointment two months after hospital discharge, he was found to be asymptomatic with ECG normalization. |
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.