No abstract available.
Cranial Nerve Diseases ; Granuloma, Plasma Cell* ; Polychondritis, Relapsing*

Micronutrient deficiencies in Crohn's disease (CD) patients are not uncommon and usually result in a combination of reduced dietary intake, disease-related malabsorption, and a catabolic state. Decreased serum thiamine levels are often reported in patients with CD. Wernicke's encephalopathy (WE) is a severe form of thiamine deficiency that can cause serious neurologic complications. Although WE is known to occur frequently in alcoholics, a number of non-alcoholic causes have also been reported. Here, we report two cases of non-alcoholic WE that developed in two severely malnourished CD patients who were supported by prolonged total parenteral nutrition without thiamine supplementation. These patients complained of sudden-onset ophthalmopathy, cerebellar dysfunction, and confusion. Magnetic resonance imaging allowed definitive diagnosis for WE despite poor sensitivity. The intravenous administration of thiamine alleviated the symptoms of WE dramatically. We emphasize the importance of thiamine supplementation for malnourished patients even if they are not alcoholics, especially in those with CD.
Administration, Intravenous ; Alcoholics ; Cerebellar Diseases ; Crohn Disease* ; Diagnosis ; Humans ; Magnetic Resonance Imaging ; Micronutrients ; Parenteral Nutrition, Total* ; Thiamine ; Thiamine Deficiency ; Wernicke Encephalopathy*

A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses.
Adenoma ; Adrenalectomy ; Adrenocortical Adenoma ; Adult ; Aldosterone* ; Blood Pressure ; Cushing Syndrome ; Dexamethasone ; Diagnosis ; Female ; Humans ; Hydrocortisone* ; Hyperaldosteronism ; Hypertension ; Hypokalemia ; Korea ; Plasma ; Veins

Left-sided portal hypertension and consequent gastric varices can occur in patients with isolated splenic vein thrombosis. It is a rare but clinically significant and curable cause of gastrointestinal hemorrhage. Our patient, a 20-year-old woman, with left flank pain was diagnosed with having idiopathic splenic vein thrombosis with resultant splenic infarction. Thorough workups for the possible etiologies of splenic vein thrombosis were all negative. After six months of anticoagulation, follow-up computed tomography revealed formation of gastric varices; one month following the discovery, she developed gastrointestinal bleeding. Splenectomy was performed, resulting in the resolution of gastric varices.
Esophageal and Gastric Varices* ; Female ; Flank Pain ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; Hemorrhage ; Humans ; Hypertension, Portal ; Splenectomy ; Splenic Infarction* ; Splenic Vein* ; Thrombosis* ; Young Adult

Systemic lupus erythematosus (SLE) is a multisystemic inflammatory autoimmune disease mediated by autoantibodies and immune complexes. In SLE, a splenectomy to control the thrombocytopenia does not increase the total risk of thrombosis, but tends to increase arterial events. We experienced a patient with lupus- anticoagulant positive SLE who developed a venous thrombosis after a splenectomy for the control of thrombocytopenia, which was a very rare case.
Antigen-Antibody Complex ; Autoantibodies ; Autoimmune Diseases ; Humans ; Lupus Erythematosus, Systemic ; Splenectomy ; Thrombocytopenia ; Thrombosis ; Venous Thrombosis

BACKGROUND: Gallstone is the most common disease of the biliary system. Most cases of gallstone are asymptomatic and we incidentally found gallstones during a community health survey. Our study was designed to determine the prevalence and risk factors for gallstone in a population who underwent health screening. METHODS: We enrolled a total of 36,314 persons who visited the health promotion center in Kyungpook National University Hospital in Korea from 1998 to 2004. They had their gallbladder or biliary system examined with using ultrasonography. The body mass index and biochemical parameters from liver function tests, the lipid profiles and the fasting blood sugar were all measured. All the subjects were divided into several groups and the particular prevalence for gallstone disease for each group was calculated. Chi-square tests and logistic regression analysis assessed the risk factors for gallstone disease. RESULTS: Among 36,314 persons, (19,345 males (53.3%) and 16,969 females (46.7%)), gallstone was found in 735 persons (2.0%). On univariate analysis, the risk factors for gallstone disease were age, body mass index, fasting blood sugar, total cholesterol, LDL-cholesterol, triglyceride and serum aspartate aminotransferase. On multivariate logistic regression analysis, the the risk factors were high body mass index, older age and high fasting blood sugar. CONCLUSIONS: The risk factors seem to show a common pathogenesis for gallstone disease. Although the relation between gallstone disease and metabolic syndrome has not been established, it appears to be associated with metabolic syndrome, but further study on a general population is required.
Aspartate Aminotransferases ; Biliary Tract ; Blood Glucose ; Body Mass Index ; Cholesterol ; Fasting ; Female ; Gallbladder ; Gallstones* ; Gyeongsangbuk-do ; Health Promotion ; Health Surveys ; Humans ; Korea ; Liver Function Tests ; Logistic Models ; Male ; Mass Screening ; Prevalence* ; Risk Factors* ; Triglycerides ; Ultrasonography

BACKGROUND: Bronchial anthracofibrosis (BAF) is a dark black or brown pigmentation of multiple large bronchi associated with a fibrotic stenosis or obliteration that is incidentally found during a diagnostic bronchoscopy. Some reporters have suggested endobronchial tuberculosis or tuberculous lymphadenitis as a possible cause of BAF. However, some BAF patients do not have any medical history of tuberculosis. The aim of this study was to elucidate the clinical features of simple BAF patients, which were not associated with tuberculosis. METHODS: We reviewed the patients' charts retrospectively and interviewed all BAF patients who were followed up for 1 year or more. Among the 114 BAF patients, 43 patents (38 %) had no associated tuberculosis, cancer and pneumoconiosis. The clinical characteristics, radiological findings and associated pulmonary diseases of these patients were evaluated. RESULTS: Most patients were non-smokers, old aged, housewifes who resided in a farming village. The common respiratory symptoms were dyspnea, cough and hemoptysis. The predominant X-ray findings were a multiple bronchial wall thickening(89%), bronchial narrowing or atelectasis (76%) and a mediastinal lymph node enlargement with/without calcification (78%). Pulmonary function test usually showed mild obstructive ventilatory abnormalities but no patient showed a restrictive ventilatory pattern and the patients were frequently affected with chronic bronchitis(51%), post-obstructive pneumonia(40%) and chronic asthma(4%). CONCLUSION: Because BAF is frequently associated with chronic bronchitis and obstructive pneumonia as well as tuberculosis, a careful clinical evaluation and accurate differential diagnosis is more essential than empirical anti-tuberculous medication.
Diagnosis, Differential ; Pneumoconiosis

17 -Hydroxylase deficiency is a rare form of congenital adrenal hyperplasia that is characterized by primary amenorrhea, absence of secondary sex characteristics, hypertension, and a hypokalemic alkalosis that has resulted resulting from increased production of deoxycorticosterone and corticosterone by the adrenal. The diagnosis of this enzyme deficiency can be recognized by the increasing serum concentrations of steroid precursors, DOC and corticosterone and the decreasing concentrations of cortisol, and adrenal androgens. We diagnosed this in a 19 year old female who presented with primary amenorrhea. We report this case with a review of the literatures.
Adrenal Hyperplasia, Congenital ; Alkalosis ; Amenorrhea* ; Androgens ; Corticosterone ; Desoxycorticosterone ; Diagnosis ; Female ; Humans ; Hydrocortisone ; Hypertension ; Sex Characteristics ; Young Adult

Malignant fibrous histiocytoma is a pleomorphic sarcoma in adults, which occurs principally as a mass on an extremity or in the abdominal cavity or retroperitoneum. It typically involved deep fascia or skeletal muscle and only rarely was confined to the subcutis without fascial involvement. Malignant fibrous histiocytomas developed in the intraabdominal organs are very rare and only few cases have been reported until now. Here, we report a case of malignant fibrous histiocytomar developed in the stomach of a 46-year old male who showed clinical and histologic features of malignant fibrous histiocytoma without any identified etiologic factors. The patient was treated successfully with surgery, and has had no recurrence since, during the ensuring one and a half yars.
Abdominal Cavity ; Adult ; Extremities ; Fascia ; Histiocytoma, Malignant Fibrous* ; Humans ; Male ; Middle Aged ; Muscle, Skeletal ; Recurrence ; Sarcoma ; Stomach*

Although a pathogenic mechanism of hemolytic anemia complicated with viral hepatitis is unknown, it is suggested that there are four mechanisms; 1) In the individual who has predisposition to hemolytic anemia, viral infection accelerates the red cell destruction & hemolysis become obvious. 2) Directly, virus itself injures to the red cell membrane. 3) The serious liver failure & hypersplenism induce the hemolysis. 4) Autoimmune hemolytic anemia because of immunological abnormality caused by viral infection. We experienced a case of autoimmune hemolytic anemia in 33-year-old male patient who was diagnosed as chronic lobular hepatitis B with biopsy. Diagnosis was estabilished by clinical features, blood cell count, routine urinalysis, direct & indirect Coombs test, liver function test, immunoglobulin quantitations, hepatitis B marker, bone marrow aspiration, and liver biopsy. This case was treated with corticosteroid and transfusion. During follow-up, he has been well tolerated.
Adult ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; Biopsy ; Blood Cell Count ; Bone Marrow ; Cell Membrane ; Coombs Test ; Diagnosis ; Follow-Up Studies ; Hemolysis ; Hepatitis B* ; Hepatitis* ; Humans ; Hypersplenism ; Immunoglobulins ; Liver ; Liver Failure ; Liver Function Tests ; Male ; Urinalysis