Fifteen percent of patients with Cushing's syndrome could be accompanied with nonpituitary tumors that secrets ACTH-the ectopic ACTH syndrome. Among them, bronchial carcinoid is one of the rare causes of the ectopic ACTH syndrome. We experienced a 60-year-old woman who showed conditions of weight gain, generalized edema, hypokalemia, diabetes mellitus, and right middle lung mass. Histologically, the tumor was diagnosed with bronchial carcinoid and was confirmed as an ectopic ACTH syndrome through immunohistochemical staining of ACTH. After resection of the lung mass, blood sugar, electrolytes, generalized weakness, and blood pressure improved.
ACTH Syndrome, Ectopic* ; Adrenocorticotropic Hormone ; Blood Glucose ; Blood Pressure ; Carcinoid Tumor* ; Cushing Syndrome ; Diabetes Mellitus ; Edema ; Electrolytes ; Female ; Humans ; Hypokalemia ; Lung ; Middle Aged ; Weight Gain

Situs inversus totalis is a rare condition with a genetic predisposition that is autosomal recessive. We report an elderly patient with situs inversus, multiple choledocholithiasis, and he was treated via endoscopic retrograde cholangiopancreatography (ERCP) with sphincterotomy, and then his multiple CBD stones were removed with basket and balloon. This paper further expands the application of these techniques and shows that they can be safely and effectively applied in the setting of situs inversus, although attention must be paid to the details of left-right reversal.
Aged ; Cholangiopancreatography, Endoscopic Retrograde ; Choledocholithiasis* ; Genetic Predisposition to Disease ; Humans ; Situs Inversus*

A primary thyroid lymphoma is rare among all types of thyroid malignancy. Usually, a thyroid lymphoma is associated with underlying chronic autoimmune thyroiditis. Recently, we experienced a primary thyroid mucosa-associated lymphoid tissue(MALT) lymphoma, with an incidental micropapillary thyroid carcinoma, but lacking evidence of autoimmune thyroiditis. A female patient visited our hospital for further evaluation of a rapidly enlarging, painless thyroid mass which had been stable for 8 years. Lymphocytic thyroiditis or a lymphoma was suspected from fine needle aspiration performed at another hospital. The thyroid function test and other routine laboratory tests were normal. The histopathological findings after a total thyroidectomy revealed a MALT lymphoma with a micropapillary thyroid carcinoma. There was no evidence of chronic autoimmune thyroiditis. This is, to the best of our knowledge, the first case report of a MALT lymphoma arising from the thyroid gland without evidence of chronic autoimmune thyroiditis in Korea
Biopsy, Fine-Needle ; Female ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Thyroid Function Tests ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroidectomy ; Thyroiditis, Autoimmune*

A primary thyroid lymphoma is rare among all types of thyroid malignancy. Usually, a thyroid lymphoma is associated with underlying chronic autoimmune thyroiditis. Recently, we experienced a primary thyroid mucosa-associated lymphoid tissue(MALT) lymphoma, with an incidental micropapillary thyroid carcinoma, but lacking evidence of autoimmune thyroiditis. A female patient visited our hospital for further evaluation of a rapidly enlarging, painless thyroid mass which had been stable for 8 years. Lymphocytic thyroiditis or a lymphoma was suspected from fine needle aspiration performed at another hospital. The thyroid function test and other routine laboratory tests were normal. The histopathological findings after a total thyroidectomy revealed a MALT lymphoma with a micropapillary thyroid carcinoma. There was no evidence of chronic autoimmune thyroiditis. This is, to the best of our knowledge, the first case report of a MALT lymphoma arising from the thyroid gland without evidence of chronic autoimmune thyroiditis in Korea
Biopsy, Fine-Needle ; Female ; Humans ; Korea ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Thyroid Function Tests ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroidectomy ; Thyroiditis, Autoimmune*

BACKGROUND: The sodium/iodide symporter (NIS) has an important role in the diagnosis and treatment of well differentiated thyroid carcinoma. The relationship between the uptake of thallium- 201 scan (201Tl scan) and the expression of sodium/iodide symporter (hNIS) was studied in thyroid nodules. METHODS: Patients that had undergone operations for thyroid nodules, and who received a 201Tl scan before their operations, were investigated. Anti-NIS antibodies were used to analyze the presence and distribution of the hNIS protein by immunohistochemical staining of their thyroid tissues. RESULTS: Forty-four patients (papillary carcinoma; 18, follicular adenoma; 11, adenomatous goiter; 14, nonspecific thyroiditis; 1) 30 with no immunoreactivity and 14 with a positive reaction to the anti-NIS antibody, were included. The NIS negative patients (12/30) had no 201Tl uptake, but all others were positive on 201Tl scan, and the NIS-positive patients (13/14) had positive 201Tl uptake, with 1 negative on 201Tl scan, with significant difference (p=0.035). Of the 18 patients with a papillary thyroid carcinoma, the NIS negative patients (2/10) had no 201Tl uptake and the others were positive on 201Tl scan, but without significant difference. NIS positive patients (1/8) with a papillary thyroid carcinoma had no 201Tl uptake, and the others were positive on 201Tl scan, but without significant difference. Whether the results of NIS staining and 201Tl scan were positive or not did not affect the responses of radioactive iodine therapy in our study. CONCLUSION: These results suggest that thallium-201 uptake may be correlated with hNIS expression in thyroid nodules
Adenoma ; Antibodies ; Diagnosis ; Goiter ; Humans ; Immunohistochemistry ; Iodine ; Ion Transport* ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroid Nodule* ; Thyroiditis

BACKGROUND: This study was performed to identify the incidence and prognostic significance of chromosomal abnormalities as well as clinical significance of immuno phenotype in patients with acute myelogenous leukemia (AML). METHODS: The chromosomal abnormalities, immunophenotype and their hematologic/clinical correlations were studied in 68 patients with de novo AML admitted to Pusan National University Hospital between January 1996 and December 2000. 47 of 68 patients had received induction chemotherapy and we analysed the response of treatment according to the karyotype pattern and immunophenotype. RESULTS: The karyotypic patterns were divided into three groups; favorable (t (8;21), t (15;17) and inv (16); n=19, 28%), poor (-5, del (5q), -7, der (1;7), abn (3q) and complex karyotypes; n=11, 16%) and intermediate group (other abnormalities or normal karyotype; n=38, 56%). The incidence of chromosomal abnormalities was 56% (38/68) and overall complete remission (CR) rate of 47 evaluable patients was 64%. The CR rates of favorable, intermediate and poor groups were 88%, 59% and 44%, respectively (p=0.021). The median survival time of all patients was 7 months, those of poor and intermediate groups being 2 months and 6 months. The median survival time of favorable group was not reached (p=0.008). The overall 5 year survival rate was 38% and those of favorable, intermediate and poor groups were 68%, 31% and 9%, respectively (p=0.009). Expression of CD7, CD14, CD33, CD34 and terminal deoxynucleotidyl transferase had no impact on CR rate and overall survival. In multivariate analysis, both age and chromosomal abnormalities influence significantly on prognosis. CONCLUSION: Cytogenetic study is important in predicting the outcome of patients with AML. And the treatment must be tailored according to the result of cytogenetics such as this study.
Adult* ; Busan ; Chromosome Aberrations* ; Cytogenetics ; DNA Nucleotidylexotransferase ; Humans ; Immunophenotyping ; Incidence ; Induction Chemotherapy ; Karyotype ; Leukemia ; Leukemia, Myeloid, Acute* ; Multivariate Analysis ; Phenotype ; Prognosis ; Survival Rate

We experienced a Korean patient with complete form of pachydermoperiostosis. He presented with typical features consisting of clubbing with spade like enlargement of the hand and feet, thickening of facial skin and scalp, irregular thickening of cortex with periosteal new bone formation involving radii, ulnae, tibiae, and fibulae. Classically, pachydermoperiostosis is characterized by a triad of finger clubbing, periostitis, skin and soft tissue changes, giving an acromegaloid look. A variety of associated abnormalities have been described such as cranial suture defects, female escutcheon, bone marrow failure. There are a few reports documenting gastric ulcer, hypertrophic gastropathy and Crohn's disease as accompanying diseases. The patient had upper gastrointestinal bleeding caused by hemorrhagic gastritis and duodenal ulcer. A case is herein reported of pachydermoperiostosis accompanied by peptic ulcer disease.
Acromegaly ; Bone Marrow ; Cranial Sutures ; Crohn Disease ; Duodenal Ulcer ; Female ; Fibula ; Fingers ; Foot ; Gastritis ; Hand ; Hemorrhage ; Humans ; Osteoarthropathy, Primary Hypertrophic* ; Osteogenesis ; Peptic Ulcer* ; Periostitis ; Scalp ; Skin ; Stomach Ulcer ; Tibia ; Ulna

Vasoactive intestinal peptide(VIP) is a 28-amino acid peptide, which may cause secretory diarrhea by stimulating the production of adenylate cyclase. Neuroendocrine tumors, secreting vasoactive intestinal peptide (VIP), are almost always of a pancreatic in origin. However, a pheochromocytoma may produce several neuropeptides, containing VIP, as they are considered to be neuroendocrine tumors. A 57-year-old woman, who presented with chronic watery diarrhea, hypokalemia, weight loss and a left adrenal mass, is described. Histologically, the tumor was diagnosed as a pheoch-romocytoma, with ganglioneuronal differentiation, and was histochemically confirmed to produce a vasoactive intestinal polypeptide. A left adrenal VIP-producing pheochromocytoma was successfully resected. After surgery, her diarrhea subsided and the electrolytes, affected neuroendocrine hormone levels, blood pressure and blood sugar level were normalized.
Adenylyl Cyclases ; Blood Glucose ; Blood Pressure ; Diarrhea ; Electrolytes ; Female ; Humans ; Hypokalemia ; Middle Aged ; Neuroendocrine Tumors ; Neuropeptides ; Pheochromocytoma* ; Vasoactive Intestinal Peptide* ; Weight Loss

Congenital hepatic fibrosis (CHF) is a rare developemental abnormality, which is characterized pathologically by periportal fibrosis with irregularly shaped proliferating bile ducts. In most, if not all, cases CHF is associated with autosomal recessive polycystic kidney disease. Recently, we experienced two cases, confirmed by percutaneous needle liver biopsy, of CHF with polycystic kidney disease. The first patient was a 19-year-old man and presented with hematemesis and hepatosplenomegaly. Esophageal varix was noted by an endoscopic examination and an endoscopic variceal ligation was performed. Abdominal CT scanning revealed innumerable cysts of both kidneys. The pateint also had cystic dilation of subarchnoid space in the basal cistern and posterior fossa detected through brain MRI. The second patient was a 24-year-old man admitted for an evaluation of splenomegaly. He had no esophageal varix but, splenic varix and splenorenal shunt were detected through an abdominal CT scanning. Innumerable renal cysts were also present. The diagnosis of CHF was confirmed in both cases by its typical histologic features. We report these cases with a review of the relevant literatures.
Bile Ducts ; Biopsy ; Brain ; Diagnosis ; Esophageal and Gastric Varices ; Fibrosis* ; Hematemesis ; Humans ; Hypertension, Portal ; Kidney ; Ligation ; Liver ; Magnetic Resonance Imaging ; Needles ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Recessive ; Splenomegaly ; Splenorenal Shunt, Surgical ; Tomography, X-Ray Computed ; Varicose Veins ; Young Adult

Systemic lupus erythematosus (SLE) is autoimmune disease that often develops antiphopholipid syndrome (APS). Lupus anticoagulant and anticardiolipin antibodies are the hallmarks of APS. The hypercoagulable state in APS may lead to adrenal vein thrombosis, and subsquently lead to hemorrhagic necrosis of the adrenal gland. Adrenal hemorrhage is a very rare complication of APS. Although there have been some reports about adrenal hemorrhage associated with primary APS, adrenal hemorrhage associated secondary APS in SLE has not yet been reported. We describe the adrenal hemorrhage associated with secondary APS in SLE, and the patient which complained of general weakeness, nausea, vomiting and diffuse abdominal pain. Abdominal magnetic resonance imaging (MRI) showed hemorrhage, of both renal glands, and clinical features and immunological studies were consistent with APS in SLE. The acute adrenal insufficiency was much improved after the patient was treated with glucocorticoids
Abdominal Pain ; Adrenal Glands ; Adrenal Insufficiency* ; Antibodies, Anticardiolipin ; Autoimmune Diseases ; Glucocorticoids ; Hemorrhage ; Humans ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Magnetic Resonance Imaging ; Nausea ; Necrosis ; Thrombosis ; Veins ; Vomiting