1例成人神经垂体异位病例，表现为促肾上腺皮质激素缺乏和部分生长激素缺乏。

An adult case of neurohypophyseal ectopy presenting ACTH deficiency and partial GH deficiency.

作者信息

Takamura T, Ohsawa K, Nishimura Y, Yamagishi S, Komatsu Y, Iwata A, Osada S, Nagai Y, Miyakoshi H, Kobayashi K

机构信息

Department of Internal Medicine, Kanazawa Red Cross Hospital, Japan.

出版信息

Endocr J. 1995 Feb;42(1):83-8. doi: 10.1507/endocrj.42.83.

DOI:10.1507/endocrj.42.83

PMID:7599703

Abstract

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.

摘要

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。