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女性假两性畸形主要由于先天性肾上腺皮质增生或后天性肾上腺皮质肿瘤等原因所引起。本文报道2例均因21-羟化酶缺陷所引起的先天性肾上腺皮质增生所致。 [例1] 陆××,女,20岁,住院号81-259340。患者初生时阴蒂大,无阴道。5岁阑尾手术时见子宫、输卵管、卵巢均正常。15岁月经初潮后,月经周期准,经量中,经血自尿道口流出。入院检查:血压110/70mmHg,身材中等。乳房发育差,多毛,阴毛呈男性分布,阴蒂增大,长约2.5cm,顶端有包皮样皱褶。阴蒂下见尿道下裂,尿及经血均由此排出。肛腹双合诊可触及2×2.5×2.4cm块物。实验室检查外周血细胞染色体核型为46,XX,尿17-羟皮质类固醇13mg/24h(正常值8～14),尿17-酮类固醇27.6mg/24h(正常值为6～12mg/
Female pseudohermaphroditism mainly due to congenital adrenal hyperplasia or acquired adrenal tumors caused by other reasons. This article reports two cases were due to 21-hydroxylase deficiency caused by congenital adrenal hyperplasia caused. [Example 1] Lu × ×, female, 20 years old, hospital number 81-259340. The patient has a large clitoris at birth, no vaginal. 5-year-old appendix surgical see the uterus, fallopian tubes, ovaries are normal. After 15 years of menarche, the menstrual cycle, by the amount of menstrual blood from the urethra outflow. Admission examination: blood pressure 110 / 70mmHg, medium size. Breast development is poor, hirsutism, pubic hair were male distribution, clitoris increases, about 2.5cm, the top has foreskin-like folds. See clitoral hypospadias, urine and menstrual blood are discharged. Anal and abdominal double consultation can reach 2 × 2.5 × 2.4cm block. Laboratory examination of peripheral blood cells karyotype 46, XX, urinary 17-hydroxy corticosteroids 13mg / 24h (normal 8 to 14), urinary 17-ketosteroid 27.6mg / 24h (normal 6-12mg /