Tanaka T, Hibi I, Shimizu N, Imura H, Tanaka K, Fukata J, Fujieda K, Ichimura T, Kuribayashi T, Ito K
Endocrine Research Laboratory, National Children's Hospital, Tokyo, Japan.
Endocr J. 1993 Oct;40(5):581-9. doi: 10.1507/endocrj.40.581.
A dose of 1.5 micrograms/kg of MCI-028, human corticotropin-releasing hormone (hCRH), was administered intravenously to 38 children with non-endocrine short stature with normal function in the hypothalamo-pituitary-adrenocortical axis and to 71 children with a disorder in the same axis. Blood levels of adrenocorticotropic hormone (ACTH) and cortisol were determined to evaluate the axis. The 95% confidence limits of peak responses of ACTH and cortisol in non-endocrine short stature were between 17.2 and 135.3 pg/ml, and between 13.1 and 35.6 micrograms/dl, respectively, and were used as standards for children. When compared with these standards, the hormonal responses in children with various disorders in the hypothalamo-pituitary-adrenocortical axis were as follows: in two children with Cushing's syndrome caused by adrenal tumor, ACTH values were decreased and were not responsive to hCRH, while cortisol values, though within the normal limit, were not responsive; in children with primary adrenal insufficiency or congenital adrenal hyperplasia, cortisol values were decreased and not responsive, whereas ACTH values tended to be increased and ACTH response high except for 21 alpha-hydroxylase deficiency of congenital adrenal hyperplasia. In two cases of pituitary dwarfism complicated with ACTH deficiency, both ACTH and cortisol values were decreased and poorly responsive; and in children who were receiving glucocorticoid, both ACTH and cortisol values tended to be decreased and to respond poorly to hCRH. As for side effects, hot flushing was observed among 8.0% of the subjects after administration of hCRH. But this symptom was not severe and no other side effects of clinical importance were observed.(ABSTRACT TRUNCATED AT 250 WORDS)
对38名下丘脑 - 垂体 - 肾上腺皮质轴功能正常的非内分泌性身材矮小儿童以及71名该轴存在紊乱的儿童静脉注射1.5微克/千克的人促肾上腺皮质激素释放激素(hCRH)MCI - 028。测定促肾上腺皮质激素(ACTH)和皮质醇的血药浓度以评估该轴功能。非内分泌性身材矮小儿童中ACTH和皮质醇峰值反应的95%置信区间分别在17.2至135.3皮克/毫升以及13.1至35.6微克/分升之间,并用作儿童的标准值。与这些标准值相比,下丘脑 - 垂体 - 肾上腺皮质轴存在各种紊乱的儿童的激素反应如下:两名肾上腺肿瘤所致库欣综合征患儿的ACTH值降低且对hCRH无反应,而皮质醇值虽在正常范围内但也无反应;原发性肾上腺皮质功能减退或先天性肾上腺皮质增生患儿的皮质醇值降低且无反应,而ACTH值往往升高且ACTH反应较高,但先天性肾上腺皮质增生的21α - 羟化酶缺乏症患儿除外。两例合并ACTH缺乏的垂体性侏儒症患儿的ACTH和皮质醇值均降低且反应较差;正在接受糖皮质激素治疗的儿童的ACTH和皮质醇值往往降低且对hCRH反应较差。至于副作用,注射hCRH后8.0%的受试者出现潮热。但该症状不严重,未观察到其他具有临床意义的副作用。(摘要截短于250字)