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迟发性先天性全垂体功能减退症

Congenital panhypopituitarism of late onset.

作者信息

Navarro P, Halperin I, Rodríguez C, González J M, Vidal J, Vilardell E

机构信息

Endocrinology and Nutrition Unit, Hospital Clinic, Barcelona, Spain.

出版信息

J Endocrinol Invest. 1994 May;17(5):347-50. doi: 10.1007/BF03348997.

Abstract

A 29-year-old male was diagnosed congenital panhypopituitarism due to pituitary hypoplasia, absence of pituitary stalk and ectopic neurohypophysis. This report, together with bibliographic review, may induce to reconsidering isolated GH failures, partial hypopituitarisms and panhypopituitarisms which have up to now been interpreted as idiopathic. The availability of new image techniques has allowed a better definition of the anatomic substrate of these alterations, and more subtle hormonal studies have pointed out, in some cases, their possible hypothalamic origin. On this basis, some of these cases may be interpreted as presenting the same alteration as those described in necropsy of newborns dead due to severe panhypopituitarism. Therefore, a unique clinical picture with various degrees of severity may be postulated.

摘要

一名29岁男性因垂体发育不全、垂体柄缺如和异位神经垂体被诊断为先天性全垂体功能减退症。本报告结合文献回顾,可能促使重新考虑孤立性生长激素缺乏症、部分垂体功能减退症和全垂体功能减退症,这些病症至今一直被解释为特发性。新影像技术的应用使得对这些改变的解剖学基础有了更好的定义,更精细的激素研究在某些情况下指出了它们可能的下丘脑起源。在此基础上,其中一些病例可能被解释为与因严重全垂体功能减退症死亡的新生儿尸检中描述的改变相同。因此,可以推测出一种具有不同严重程度的独特临床症状。

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