Although rare, agranulocytosis is the most serious, potentially fatal side effect of antithyroid drug. We experienced a 13-year-old girl who developed methimazole-induced agranulocytosis at 1 month after the initiation of treatment. Her granulocyte count recovered after discontinuation of methimazole and treatment with broad spectrum-antibiotics, G-CSF, and methylprednisolone. After recovery from agranulocytosis she was treated with radioiodine ablation therapy. Early detection and proper management of antithyroid drug-induced agranulocytosis is very important.
Adolescent ; Agranulocytosis* ; Female ; Granulocyte Colony-Stimulating Factor* ; Granulocytes* ; Humans ; Methimazole ; Methylprednisolone*

Patients with chronic renal failure, generally, sufferred from normocytic normochromic anemia caused by decreased level of erythropoietin. But, secondary erythrocytosis has been reported in patients with several renal diseases; renal artery stenosis or throm- bosis, polycystic kidney disease, bilateral hydronephrosis, etc. We report one case of chronic renal failure combined with polycythemia vera. The case was 32 year-old man whose chief complaints were dyspnea, back pain, itching sensation, headache. 6 month ago, the laboratory examination showed only proteinuria and hematuria without deterioration of renal function. The renal function was aggravated with an accelerated course, and bone marrow examination revealed hypercellularity (erythroid predominance), and renal biopsy showed the finding of the end stage of renal disease which may be originated from IgA nephropathy.
Adult ; Anemia ; Back Pain ; Biopsy ; Bone Marrow Examination ; Dyspnea ; Erythropoietin ; Glomerulonephritis, IGA* ; Headache ; Hematuria ; Humans ; Hydronephrosis ; Immunoglobulin A* ; Kidney Failure, Chronic* ; Polycystic Kidney Diseases ; Polycythemia Vera* ; Polycythemia* ; Proteinuria ; Pruritus ; Renal Artery Obstruction ; Sensation

Post-traumatic pisotriquetral osteoarthritis is an unusual condition and its diagnosis is not easy to make. This disease must be distinguished from other conditions that cause ulnar side wrist pain. We experienced a case of post-traumatic pisotriquetral osteoarthritis treated by pisiformectomy.
Diagnosis ; Osteoarthritis* ; Wrist

Despite the well-established nature of bromate-induced ototoxicity, cochlear implantation after bromate intoxication has been rarely documented. We hereby present a case of a 51-year-old female deafened completely after bromate ingestion. Her hearing was not restored by systemic steroid treatment and hearing aids were of no use. A cochlear implantation was performed on her right ear 3 months after the bromate ingestion. In bromate intoxication cases, early monitoring of hearing level is necessary and other drugs with potential ototoxicity should be avoided. The outcome of cochlear implantation was excellent in this case of bromate-induced deafness.
Cochlear Implantation* ; Cochlear Implants* ; Deafness ; Ear ; Eating ; Female ; Hearing ; Hearing Aids ; Hearing Loss, Sudden* ; Humans ; Middle Aged

Osteoma of the temporal bone most commonly occurs in the external ear. Osteomas in the middle ear are not common, and only 25 relevant cases (18 papers) have been reported in the English literature. With only 5 cases reported to date, osteoma of the promontory in the middle ear is rare. This study reports a case of asymptomatic osteoma of the promontory in the middle ear mimicking a congenital cholesteatoma in a 4-year-old girl.
Child, Preschool ; Cholesteatoma* ; Ear, External ; Ear, Middle ; Female ; Humans ; Osteoma* ; Temporal Bone

PURPOSE: To find the applicability of adjuvant radiotherapy for extrahepatic bile duct cancer (EBDC), we analyzed the pattern of failure and evaluate prognostic factors of locoregional failure after curative resection without adjuvant treatment. MATERIALS AND METHODS: In 97 patients with resected EBDC, the location of tumor was classified as proximal (n = 26) and distal (n = 71), using the junction of the cystic duct and common hepatic duct as the dividing point. Locoregional failure sites were categorized as follows: the hepatoduodenal ligament and tumor bed, the celiac artery and superior mesenteric artery, and other sites. RESULTS: The median follow-up time was 29 months for surviving patients. Three-year locoregional progression-free survival, progression-free survival, and overall survival rates were 50%, 42%, and 52%, respectively. Regarding initial failures, 79% and 81% were locoregional failures in proximal and distal EBDC patients, respectively. The most common site was the hepatoduodenal ligament and tumor bed. In the multivariate analysis, perineural invasion was associated with poor locoregional progression-free survival (p = 0.023) and progression-free survival (p = 0.012); and elevated postoperative CA19-9 (> or =37 U/mL) did with poor locoregional progression-free survival (p = 0.002), progression-free survival (p < 0.001) and overall survival (p < 0.001). CONCLUSION: Both proximal and distal EBDC showed remarkable proportion of locoregional failure. Perineural invasion and elevated postoperative CA19-9 were risk factors of locoregional failure. In these patients with high risk of locoregional failure, adjuvant radiotherapy could be considered to improve locoregional control.
Bile Duct Neoplasms ; Bile Ducts, Extrahepatic* ; Celiac Artery ; Cystic Duct ; Disease-Free Survival ; Follow-Up Studies ; Hepatic Duct, Common ; Humans ; Ligaments ; Mesenteric Artery, Superior ; Multivariate Analysis ; Prognosis ; Radiotherapy, Adjuvant* ; Recurrence ; Risk Factors ; Survival Analysis ; Survival Rate

Branchio-oto-renal (BOR) syndrome is a clinically heterogeneous autosomal dominant form of syndromic hearing loss characterized by variable hearing impairment, malformations of the pinnae, the presence of branchial arch remnants, and various renal abnormalities. BOR syndrome is caused by mutations in EYA1 and SIX1, which are critical to organogenesis and are expressed together in developing otic, branchial, and renal tissue. Branchio-otic (BO) syndrome comprises branchial fistulas and preauricular pits, but lacks renal anomalies. We present a case of BO syndrome in 30year-old man with a review of the literature.
Branchial Region ; Branchio-Oto-Renal Syndrome ; Branchioma ; Fistula ; Hearing Loss ; Organogenesis

To investigate the underlying mechanisms of C18 fatty acids (stearic acid, oleic acid, linoleic acid and alpha-linolenic acid) on mast cells, we measured the effect of C18 fatty acids on intracellular Ca2+ mobilization and histamine release in RBL-2H3 mast cells. Stearic acid rapidly increased initial peak of intracellular Ca2+ mobilization, whereas linoleic acid and alpha-linolenic acid gradually increased this mobilization. In the absence of extracellular Ca2+, stearic acid (100 microM) did not cause any increase of intracellular Ca2+ mobilization. Both linoleic acid and alpha-linolenic acid increased intracellular Ca2+ mobilization, but the increase was smaller than that in the presence of extracellular Ca2+. These results suggest that C18 fatty acid-induced intracellular Ca2+ mobilization is mainly dependent on extracellular Ca2+ influx. Verapamil dose-dependently inhibited stearic acid-induced intracellular Ca2+ mobilization, but did not affect both linoleic acid and alpha-linolenic acid-induced intracellular Ca2+ mobilization. These data suggest that the underlying mechanism of stearic acid, linoleic acid and alpha-linolenic acid on intracellular Ca2+ mobilization may differ. Linoleic acid and alpha-linolenic acid significantly increased histamine release. Linoleic acid (C18:2: omega-6)-induced intracellular Ca2+ mobilization and histamine release were more prominent than alpha-linolenic acid (C18:3: omega-3). These data support the view that the intake of more alpha-linolenic acid than linoleic acid is useful in preventing inflammation.
alpha-Linolenic Acid ; Fatty Acids* ; Histamine Release* ; Inflammation ; Linoleic Acid ; Mast Cells ; Oleic Acid ; Verapamil

Human sparganosis is caused by the larval tapeworm of genus Spirometra. This parasite commonly invades subcutaneous tissues and muscles. However, infection in the pleural cavity is rare. A 65-year-old male patient, who had undergone surgical excision of subcutaneous masses due to a parasite infection (presumed to have been sparganosis) approximately 10 years ago, showed pleural effusion and peripheral eosinophilia. The anti-sparganum specific IgG antibody levels in the serum and pleural fluid were significantly higher than the normal control levels. Three consecutive doses of praziquantel (75 mg/kg/day) were administered for control of pleural effusion and peripheral eosinophilia. In this patient, sparganosis was suspected, and the probable cause of the infection was ingestion of raw snakes and frogs. Immunoserologic tests using ELISA can be helpful in diagnosis of pleural sparganosis and praziquantel is suggested as an alternative treatment for surgically unresectable cases.
Cestoda ; Eating ; Enzyme-Linked Immunosorbent Assay ; Eosinophilia ; Eosinophils ; Humans ; Immunoglobulin G ; Male ; Muscles ; Parasites ; Pleural Cavity ; Pleural Effusion ; Pleurisy ; Praziquantel ; Snakes ; Sparganosis ; Sparganum ; Spirometra ; Subcutaneous Tissue

BACKGROUND AND OBJECTIVES: Sudden sensorineural hearing loss (SSNHL) is a medical emergency when an individual experiences at least 30 dB (decibels) of sudden SSNHL, occurring over a time period of three days or less. Pure tone audiometry (PTA) is by far the most widely used to diagnose SSNHL. Besides PTA, auditory steady-state response (ASSR) is also generally accepted to predict the hearing of SSNHL patients objectively and effectively. We analyzed correlation between ASSR and PTA in SSNHL patients, compared that with the corresponding data in non-SSNHL patients, and studied the usefulness of correlation between ASSR and PTA to forecast prognosis of SSNHL in clinical applications. SUBJECTS AND METHOD: We retrospectively analyzed the charts of SSNHL patients. We analyzed the correlation between the thresholds of PTA, ASSR, and audiotory brainstem response (ABR) in SSNHL, compared that with corresponding data in non-SSNHL patients. RESULTS: There was a very strong positive linear correlation between the average hearing threshold of ASSR and PTA among the non-SSNHL patients. Furthermore, there was relatively strong positive linear correlation between the average hearing threshold of ASSR and PTA among SSNHL patients. Also there was a normal positive linear correlation between the average hearing threshold of ABR and PTA among SSNHL patients. The average hearing threshold difference between ASSR and PTA among SSNHL patients was smaller compared with non-SSNHL patients, and this was statistically significant. CONCLUSION: There was a strong correlation between the average hearing threshold of ASSR and PTA among non-SSNHL & SSNHL patients. Compared to ABR, ASSR showed greater correlation with PTA in the SSNHL patients. We concluded that ASSR could be a useful diagnostic tool in SSNHL.
Audiometry ; Brain Stem ; Emergencies ; Evoked Potentials, Auditory, Brain Stem ; Hearing ; Hearing Loss, Sensorineural* ; Hearing Loss, Sudden ; Humans ; Prognosis ; Retrospective Studies