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증례 : 혈액종양 ; 부신피질자극호르몬을 분비하는 췌장의 악성 신경내분비 종양 1예
송은미 ( Eun Mi Song ),이경은 ( Kyoung Eun Lee ),조정연 ( Jung Youn Jo ),구혜수 ( Hea Soo Koo ),최문영 ( Moon Young Choi ),성주명 ( Chu Myong Seong ),이순남 ( Soon Nam Lee ) 대한내과학회 2011 대한내과학회지 Vol.80 No.2
췌장에서 기원한 부신피질자극호르몬을 분비하는 신경내분비 종양은 처음부터 간 전이 등이 흔한 악성 종양이다. 대개 Cushing 증후군의 임상양상을 보이며, 진단은 방사선학적 검사, 조직검사 및 혈청학적 검사를 기반으로 한다. 저자들은 somatostatin analogue 약물에 불응성이고, 급격한 임상적 악화를 보이면서 감염으로 사망하게 된, 다발성 간 전이를 동반한 부신피질자극호르몬을 분비하는 췌장의 신경 내분비 종양을 경험하였기에 보고하는 바이다. Pancreatic endocrine tumors are low-to-intermediate-grade neoplasms arising from the pancreatic islets that produce various hormones. These tumors account for a minority of pancreatic tumors and are generally considered functional if they are associated with a hormonal syndrome. Adrenocorticotropic hormone-secreting tumors (ACTHomas) are very rare and require special clinical consideration. Patients with ACTH-secreting tumors usually present with Cushing`s syndrome, due to ectopic ACTH production. ACTHomas have a poor prognosis because of early metastasis and difficulty controlling corticosteroid production. We report a 62-year-old male with an ACTH-secreting metastatic pancreatic neuroendocrine tumor, who did not respond to somatostatin analogs and died. (Korean J Med 2011;80:238-242)
o,p'-DDD가 흰쥐 뇌하수체 전엽의 ACTH분비의 활성도에 미치는 영향에 관한 면역조직화학적 연구
기근홍,최종범,임성철,박동수,이병래,전호종,서재홍,박규호 朝鮮大學校 附設 醫學硏究所 1989 The Medical Journal of Chosun University Vol.14 No.2
It has been well known that the toxicity of o,p'-DDD on the zona fasciculata in adrenal gland. The toxicity revealed adrenostatic effect on the cortisol biosynthesis by mitochodrial destruction. So, o,p'-DDD was used for unoperable adrenocortical carcinoma and its metastasis. The secretory activity of cortisol in adrenal cortex depended on ACTH secretion in the adenohypophysis. o,p'-DDD was dissolved in corn oil and administered orally for 28 days by 75 ㎎ / ㎏, 150㎎ / ㎏ and 300 ㎎ / ㎏ to investigate the adrenostatic effect on the cortisol biosynthesis of o,p'-DDD by assesment of ACTH secretory activity in anterior lobe of pituitary gland of rats. The results obtained were that the secretory activity of ACTH and the number of ACTH secretory cells were increased according to the dosage of o,p'-DDD, especially 300㎎ / ㎏ administrated group. Therefore, the suppression of cortisol biosynthesis of o,p'-DDD was confirmed by increased secretory activity of ACTH and the number of ACTH secretory cells.
부신갈색종에 의한 Ectopic ACTH Syndrome 증례 보고
홍석준,김원배<SUP>1<.SUP>,Suck Joon Hong,M.D. and Won Bae Kim,M.D.<SUP>1<.SUP> 대한갑상선-내분비외과학회 2002 The Koreran journal of Endocrine Surgery Vol.2 No.2
A 28-year old female was diagnosed preoperatively to have a pheochromocytoma producing ACTH at other hospital. However the operation was delayed due to poor general condition and abnormal liver function. She revealed severe hypercortisolism, hypokalemic metabolic alkalosis, glucose intolerance and showed rapid downhill course. She was transferred to our hospital for operation but septic complications were strarted already. She was not recovered in spite of desperate operation and died just after moribund discharge. ACTH producing pheochromocytoma is uncommon and literature review indicated high mortality rates due to improper preoperative diagnosis. Proper preoperative recognition and early surgical management is mandatory for better results. (Korean J Endocrine Surg 2002;2:116-119)