Takamura T, Ohsawa K, Nishimura Y, Yamagishi S, Komatsu Y, Iwata A, Osada S, Nagai Y, Miyakoshi H, Kobayashi K
Department of Internal Medicine, Kanazawa Red Cross Hospital, Japan.
Endocr J. 1995 Feb;42(1):83-8. doi: 10.1507/endocrj.42.83.
A case of ACTH deficiency and partial GH deficiency associated with neurohypophyseal ectopy is described. A 42-year-old woman of short stature was admitted for hypoglycemic coma. The patient had hypocortisolemia, an increase in urinary 17-OHCS after consecutive injections of ACTH-Z, and a low plasma ACTH level which showed no response to corticotropin-releasing factor. This indicated the presence of ACTH deficiency. The plasma GH level showed a blunted response to insulin-induced hypoglycemia, but its response to GRF was preserved. Other hypothalamo-pituitary axes were intact. T1-weighted magnetic resonance imaging demonstrated ectopic neurohypophyseal tissue and a tiny anterior pituitary remnant. ACTH deficiency and partial GH deficiency might have developed as a consequence of pituitary stalk injury and inadequate regeneration of the anterior lobe.
本文描述了一例与神经垂体异位相关的促肾上腺皮质激素（ACTH）缺乏和部分生长激素（GH）缺乏的病例。一名身材矮小的42岁女性因低血糖昏迷入院。患者存在皮质醇血症降低，连续注射ACTH-Z后尿17-羟皮质类固醇（17-OHCS）增加，且血浆ACTH水平较低，对促肾上腺皮质激素释放因子无反应。这表明存在ACTH缺乏。血浆GH水平对胰岛素诱导的低血糖反应迟钝，但其对生长激素释放因子（GRF）的反应保留。其他下丘脑-垂体轴完整。T1加权磁共振成像显示神经垂体组织异位和微小的垂体前叶残余。ACTH缺乏和部分GH缺乏可能是垂体柄损伤和前叶再生不足的结果。
