Weidner W, Zöller G, Ringert R H
Klinik und Poliklinik für Urologie der Georg-August-Universität Göttingen.
Urologe A. 1992 Nov;31(6):322-7.
Abnormal sex differentiation with ambiguous genitalia now leads to prompt and comprehensive diagnostic evaluation in the neonate. If necessary, phenotypic gender assignment is done very early and appropriate surgical correction is performed. Management at puberty and later is more difficult. Andrological guidance at this time covers problems of genital identification, sexuality and fertility. The different types of abnormal differentiation, i.e. genetic, gonadal and phenotypic alterations, may result in different andrological problems, e.g. hypogonadism including micropenis and undescended testes, lack of secondary sex characteristics and impotence. With respect to fertility the major problems are testicular azoospermia and/or congenital abnormalities of the male reproductive tract. Any proposals for treatment have to take account of all these different aspects of pathogenesis and the natural course of the disease. In each case, andrological guidance must be formulated with due consideration for the results of genital determination in childhood, including surgical correction.
新生儿出现伴有生殖器模糊的异常性分化,现在会促使进行迅速而全面的诊断评估。如有必要,会在很早的时候就进行表型性别指定,并实施适当的手术矫正。青春期及之后的管理则更加困难。此时的男科指导涵盖生殖器识别、性取向和生育能力等问题。不同类型的异常分化,即基因、性腺和表型改变,可能导致不同的男科问题,例如性腺功能减退,包括小阴茎和隐睾、缺乏第二性征以及阳痿。关于生育能力,主要问题是睾丸无精子症和/或男性生殖道先天性异常。任何治疗方案都必须考虑到发病机制的所有这些不同方面以及疾病的自然病程。在每种情况下,制定男科指导时都必须充分考虑儿童期生殖器确定的结果,包括手术矫正。