text: 第六章先天性肾上腺皮质增生症【概述】先天性肾上腺皮质增生症（congenitaladrenalhyperplasia，CAH）是一组常染色体隐性遗传性疾病，因类固醇激素合成过程中某种酶的先天性缺陷，导致肾上腺皮质合成的皮质醇完全或部分受阻，皮质醇缺乏，对下丘脑-垂体的负反馈作用消除，促使下丘脑-垂体分泌的促肾上腺皮质激素释放激素（corticotrophic-relieasinghormone，CRH）和促肾上腺皮质激素（adrenocorticotrophichormone，ACTH）分泌增加，导致肾上腺皮质增生，有些酶的缺乏同时可导致盐皮质激素和性激素合成障碍。
entities.entity: 先天性肾上腺皮质增生症
entities.label: dis
entities.entity: 先天性肾上腺皮质增生症
entities.label: dis
entities.entity: congenitaladrenalhyperplasia
entities.label: dis
entities.entity: CAH
entities.label: dis
entities.entity: 常染色体隐性遗传性疾病
entities.label: dis
entities.entity: 类固醇激素
entities.label: bod
entities.entity: 酶
entities.label: bod
entities.entity: 肾上腺皮质
entities.label: bod
entities.entity: 皮质醇
entities.label: bod
entities.entity: 皮质醇缺乏
entities.label: sym
entities.entity: 下丘脑-垂体
entities.label: bod
entities.entity: 下丘脑-垂体
entities.label: bod
entities.entity: corticotrophic-relieasinghormone
entities.label: bod
entities.entity: CRH
entities.label: bod
entities.entity: 促肾上腺皮质激素
entities.label: bod
entities.entity: adrenocorticotrophichormone
entities.label: bod
entities.entity: ACTH
entities.label: bod
entities.entity: 肾上腺皮质增生
entities.label: sym
entities.entity: 酶
entities.label: bod
entities.entity: 盐皮质激素
entities.label: bod
entities.entity: 性激素
entities.label: bod