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本文分析了先天性肾上腺皮质增生所致女性外生殖器男性化畸形32例。31例为21-羟化酶缺乏,1例为11-羟化酶缺乏。外生殖器男性化畸形按 Prader 标准分为5型。本文详细描述了5型的外观表现。32例中以Ⅱ与Ⅲ型最为多见。4例按男性长大,其中2例幼时检查后纠正为女性,另2例已成年,难于改变性别。准确诊断性别对此症十分重要。一旦确诊,需终身补充皮质激素,以抑制 ACTH过度分泌而导致雄激素过剩及糖和(或)盐皮质激素的不足。外生殖器男性化畸形需行阴蒂与阴道整形术。文章并介绍了手术时间与方法。治疗后随诊月经,4例自然初潮,15例用药后初潮。9例已婚,5例已妊娠分娩,婴儿均健康。
This article analyzes 32 cases of male genital malformations caused by congenital adrenal hyperplasia. 31 cases of 21-hydroxylase deficiency, 1 case of 11-hydroxylase deficiency. External genital masculine deformity according to Prader standard is divided into 5 types. This article describes the appearance of Type 5 in detail. Among 32 cases, type Ⅱ and Ⅲ were the most common. 4 cases grew up by men, two of which were corrected to be female after childbearing examination and the other 2 cases were adulthood, making it difficult to change the sex. Accurate diagnosis of gender is very important for this disease. Once diagnosed, lifelong corticosteroids need to be added to inhibit over-secretion of ACTH resulting in excess androgen and sugar and (or) mineralocorticoid deficiency. External genital masculine deformity required clitoris and vaginal plastic surgery. The article also introduced the operation time and method. After treatment, menstruation followed up, 4 cases of natural menarche, and 15 cases of menarche after treatment. Nine were married and five had gestational childbirth, and the infants were healthy.