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先天性肾上腺增生症是一组先天性内分泌疾病。其基本病变是某种合成肾上腺皮质激素所必需的酶系统存在缺陷。本病包括21—羟化酶缺乏症,11—β羟化酶缺乏症,17—α羟化酶缺乏症,3β—羟类固醇脱氢酶缺乏症,18—羟化酶缺乏症。其中伴有高血压者为11β—羟化酶缺乏症和17α—羟化酶缺乏症。11β—羟化酶缺乏症(11β—OHD):是一种比较常见的伴有高血压和男性化的先天性肾上腺增生症。当11β—羟化酶存在缺陷时便导致皮质醇
Congenital adrenal hyperplasia is a group of congenital endocrine diseases. The basic lesion is a deficiency of certain enzymatic systems necessary for the synthesis of adrenocorticotropic hormones. The disease includes 21-hydroxylase deficiency, 11-β-hydroxylase deficiency, 17-α-hydroxylase deficiency, 3β-hydroxysteroid dehydrogenase deficiency, 18-hydroxylase deficiency. Among those with hypertension are 11β-hydroxylase deficiency and 17α-hydroxylase deficiency. 11β-Hydroxylase Deficiency (11β-OHD): A common congenital adrenal hyperplasia associated with hypertension and masculinity. Cortisol is caused when 11 [beta] -hydroxylase is defective