[两兄弟的低促性腺激素性性腺功能减退和外周肾上腺功能不全]
[Hypogonadotrophic hypogonadism and peripheral adrenal insufficiency in 2 brothers].
作者信息
Bessac L, Bost M, Bachelot Y, Andrini P
机构信息
Service de médecine infantile, CHRU de Grenoble, France.
出版信息
Pediatrie. 1988;43(2):129-30.
PMID:3393409
Abstract
Two siblings with chronic adrenal insufficiency, diagnosed at 12 years of age, failed to show a spontaneous onset of puberty in spite of adequate adrenocortical therapy. Testosterone plasma levels were low, without concomitant increase in gonadotropin levels. This association strongly suggest a sex-linked cytomegalic adrenocortical hypoplasia.
摘要
两名患有慢性肾上腺皮质功能不全的兄弟姐妹，12岁时被确诊，尽管接受了充分的肾上腺皮质治疗，但仍未出现青春期自发启动。血浆睾酮水平较低，促性腺激素水平未随之升高。这种关联强烈提示为X连锁巨细胞性肾上腺皮质发育不全。
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