A usefulness of urinary K/Cr ratio as differential diagnosis of acute hypokalemic paralysis / 대한내과학회지

BACKGROUND: Acute hypokalemic paralysis, a clinical syndrome characterized by acute systemic weakness and low serum potassium, is a rare but treatable cause of acute weakness. The aim of our study is to analyze the cause of hypokalemic paralysis and to define clinical characteristics of hypokalemic paralysis. METHODS: Hypokalemia was defined as a serum potassium concentration less than 3.0 mEq/L in this study. A total 31 patients with hypokalemic paralysis (male 71%, mean age; 35.7 +/- 14.3 years) have been studied retrospectively from June 1994 to March 2004 for the evaluation of clinical characteristics. According to the pathophysiology of hypokalemia, patients were divided into the potassium renal loss group (n=9) and the potassium shift group (n=14). We calculate the UK/UCr (mmol/mmol) ratio, Transtubular K+ concentration gradient (TTKG) and compared the results between two groups. RESULTS: The cause of hypokalemic paralysis was hyperthyroidism (45.2%), medication (19.4%), renal disease (6.5%), hyperaldosteronism (3.2%), poor oral intake (3.2%), vomiting (3.2%), and unknown origin (19.4%). There was a significant difference of UK/UCr ratio (p=0.046) but no significant difference of TTKG (p=0.116) between the potassium loss and shift groups. CONCLUSION: The most common cause of acute hypokalemic paralysis is hyperthyroidism. The UK/UCr ratio is more useful measure than TTKG in differentiation of potassium loss and potassium shift as a cause of hypokalemia.

A Case of Familial Medullary Thyroid Carcinoma with a E768D Mutation in RET Proto-Oncogene / 대한내분비학회지

A medullary thyroid carcinoma, a neoplasm of parafollicular C cell origin, occurs as a sporadic or hereditary disease. A hereditary medullary thyroid carcinoma is an autosomal dominantly inherited disease, which is composed of multiple endocrine neoplasia 2A and 2B, with a familial medullary thyroid carcinoma. Germline mutations of the RET gene are the underlying cause of the majority of hereditary medullary carcinomas. Here, the case of a 42 years-old man with a familial medullary thyroid carcinoma, confirmed by the detection of a RET proto-oncogene mutation at exon 13 on codon 768 from a GAG(Glu) to a GAT(Asp), is described. The patient underwent a total thyroidectomy and modified radical neck dissection. His sister was found to have the same mutant gene.

A Case Report of von Hippel-Lindau Disease Manifested in a Monozygous Twin / 대한내분비학회지

Von Hippel-Lindau (VHL) disease is an autosomal dominant disease, which forms hypervascular tumors in multiple organs, such as hemangioblastomas in the retina and central nervous system, renal cell carcinomas, pheochromocytomas and cysts in various organs. Recent advances in gene testing have made it possible to screen family members for VHL disease. We experienced a 28 year-old male, who was diagnosed with bilateral pheochromocytomas through a family screening test when his elder monozygous twin brother was diagnosed with a pheochromocytoma. He received no treatment until December, 2004, when he visited the Emergency room due to a headache. A hemangioma of the cerebellum was seen in the brain MR study, leading to the diagnosis of type 2A VHL disease. An abdominal CT scan revealed no lesions of the pancreas or kidney. There was no evidence of a hemangioma in the retinal scan. The subsequent gene testing showed a germline mutation in exon 3 codon 167 of the VHL gene. The mother of the patient was revealed to have the same mutation of the VHL gene, but the elder brother of the patient did not.

Retrospective Analysis of Treatment Outcome and Prognostic Factors in Multiple Myeloma / 대한혈액학회지

BACKGROUND: The prognostic outlook for multiple myeloma has markedly improved in recent decades, which is probably related to the introduction of chemotherapy and the development of supportive care for various complications. In the present work we analysed retrospectively the therapeutic outcomes of newly diagnosed patients with multiple myeloma treated at Korea Cancer Center Hospital (KCCH). And we studied to identify prognostic factors influencing the therapeutic outcome of the disease. METHODS: Between January 1987 and December 1998, eighty three patients were diagnosed as multiple myeloma by the criteria of Southwestern Oncology Group at KCCH. Of these patients, clinical analysis was performed retrospectively for sixty-one patients who were treated with combination chemotherapy. RESULTS: The median age at diagnosis was 55 years of age, which was lower than that of western countries and 48% of patients were in their 4th decade. Male to female ratio was 1: 1.1. The main chief complaint at diagnosis was bone pain. Ninety-one percent of the patients were clinical stage III. Serum immuno-electrophoresis revealed M-protein as IgG in 41%. The ratio of micro to lambda light chain was 1.5:1. The response rate to initial chemotherapy was 75% (95% C. I.=63.4~86.6%) and median progression free interval was 20.9 months. The median overall survival of total 61 patients was 28.5 months. The patients' age at diagnosis and the response to initial chemotherapy were statistically significant prognostic factors influencing the overall survival of patients. CONCLUSION: This study showed that we could get relatively high response rate with conventional chemotherapy for the patients with multiple myeloma, but, that most patients eventually progressed and the cure was nearly impossible. To obtain cure or to have much longer survival time, we need more delicate strategies including more intensive chemotherapy with stem cell transplantation for the treatment of multiple myeloma patients.

Clinical Characteristics of Female Hepatocellular Carcinoma / 대한간학회지

BACKGROUND/AIMS: The incidence of hepatocellular carcinoma has been universally lower in female than in male. The aims of our study are to define whether there are any difference between female and male patients with hepatocellular carcinoma in terms of clinical characteristics and results of treatment. METHODS: Retrospective analyses of medical history, physical findings, laboratory results, etiological factors, characteristics of tumor, and therapeutic results were performed in 80 female patients with hepatocellular carcinoma compared to 160 male patients. RESULTS: Asymptomatic presentation and family history of liver disease were found more frequently in female patients than in male patients. A history of smoking and alcohol drinking were found less frequently in female patients than in male patients. The detection rate of spider angioma was significantly lower in female patients than in male patients. There was no difference in laboratory results, characteristics of tumor, and therapeutic results between female and male patients. CONCLUSIONS: Environmental factors such as smoking and alcohol drinking could contribute the sexual difference of hepatocarcinogenesis. However, clinical characteristics at the time of diagnosis and therapeutic results were not significantly different between female and male patients with hepatocellular carcinoma.

Supraumbilical Skin Rash as a Rare Complication of Transcatheter Arterial Chemoembolization in Patients with Hepatocellular Carcinoma / 대한간학회지

Transcatheter arterial chemoembolization (TACE) is a therapeutic option for unresectable hepatocellular carcinoma. Supraumbilical skin rash is a rare complication of TACE caused by patent hepatic falciform artery. We report herein two cases of supraumbilical skin rash developed after TACE for hepatocellular carcinoma, with discussion on the pathogenesis, prophylaxis, and treatment.