Chaussain J L, Roger M, Job J C
Arch Fr Pediatr. 1982 Feb;39(2):109-10.
Two adolescents with chronic adrenal insufficiency, presumably secondary to the cytomegalic type of congenital adrenal hypoplasia, developed a gonadotropin deficiency at puberty, which was both delayed and incomplete. Endocrine studies demonstrated in plasma low LH levels which failed to increase after LH-RH, higher levels of FSH, low basal testosterone concentration but increasing normally after HCG stimulation. Azoospermia was demonstrated in one case. In parallel with those previously reported, these two new cases show that the association between gonadotropin deficiency and congenital adrenal hypoplasia is not accidental, even if the relationship between the two components of the syndrome is still unclear.
两名患有慢性肾上腺功能不全的青少年，推测继发于巨细胞型先天性肾上腺发育不全，在青春期出现促性腺激素缺乏，表现为延迟且不完全。内分泌研究显示血浆中促黄体生成素（LH）水平低，注射LH释放激素（LH-RH）后无升高，促卵泡生成素（FSH）水平较高，基础睾酮浓度低，但人绒毛膜促性腺激素（HCG）刺激后正常升高。其中一例显示无精子症。与先前报道的病例一样，这两例新病例表明促性腺激素缺乏与先天性肾上腺发育不全之间的关联并非偶然，即使该综合征两个组成部分之间的关系仍不清楚。
