- Clinical and Hormonal Analysis of Five New Cases and Eight Previously Reported Cases of Isolated ACTCH Deficiency in Korea.
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Eun Young Oh, Byoung Joon Kim, Yun Jae Chung, Dong Joon Kim, Jong Ryul Hahm, Jae Hoon Chung, Yong Ki Min, Myung Shik Lee, Moon Kyu Lee, Kwang Won Kim
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J Korean Endocr Soc. 1999;14(3):568-577. Published online January 1, 2001
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 Abstract
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- Isolated ACTH deficiency is an uncommon disorder, which is defined by low cortisol production with low or normal plasma ACTH levels and no other pituitary abnormalities. We report five new cases of this disorder, and summarize the clinical and hormonal features of 8 previously reported cases in Korea plus 5 new cases. 1) The clinical manifestations of isolated ACTH deficiency are variable, non-specific and similar to those seen in adrenocortical insufficiency of any cause, the age of patients ranged from 21 to 66 years old with an average age of 46 years, and the male to female ratio was 10:3. 2) Hyponatremia and hypoglycemia were commmon laboratory findings, so the presence of unexplained hyponatremia or hypoglycemia should always warrant consideration of the diagnosis of isolated ACTH deficiency. 3) 3 of 13 patients accompanied by empty sella suggesting selective destruction of pituitary ACTH producing cells. 4) ACTH response to exogenous CRH or vasopressin was not elicited in all tested cases, suggesting pituitary disorders. 5) Most patients showed dramatic response with oral predinisone. In conclusion, when there are unexplained general weakness, fatigue, weight loss, nausea, vomiting, hypoglycemia, or hyponatremia, isolated ACTH deficiency should be excluded. Immunologic and pathologic studies, and hormonal evolution with glucocorticoid treatment are needed to understand the pathogenesis of isolated ACTH deficiency.
- A Case of Diabetes Mellitus Caused by Calcitonin and Somatostatin Secreting Pancreatic Islet Tumor.
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Jae Hoon Chung, Kwang Won Kim, Byoung Joon Kim, Sung Hoon Kim, Kyung Ah Kim, Myung Sik Lee, Moon Gyu Lee, Yong Ki Min, Jong Ryol Ham, Dong Joon Kim, Hoe Jung Lee, Young Ryoon Oh
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J Korean Endocr Soc. 1999;14(2):425-431. Published online January 1, 2001
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- A case of 39-year-old diabetic patient with a calcitonin and somatostatin secreting pancreatic islet tumor is presented. He had suffered from chronic diarrhea and dyspepsia for 10 years and was diagnosed with diabetes 2 years ago. Abdominal CT revealed a huge abdominal mass which was considered as a neuroendocrine tumor after US-guided needle biopsy. A distal pancreatectomy and splenectomy were performed. Histologically, tumor cells, amanged in solid sheets, showed small nuclei without significant atypia and granular eosinophilic cytoplasm. Tumor cells showed strong immunoreacitivity for calcitonin and somatostatin. The serum clacitonin was markedly elevated (268.7 pmol/L, normal range; 0.9-7.6 pmol/L). After resection of the tumor, diarrhea and dyspepsia diappeared, and oral glucose tolerance test showed normal glucose tolerance with normalization of calcitonin.
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