真假两性畸形的诊断及处理体会

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正确的性别应该在染色体(核性别)、外生殖器形态、生殖道和性腺都是一致的。如果出现矛盾现象或同时具有男性及女性特征者,就应称为两性畸形。但是有少数病例外生殖器近乎正常,也可能是两性畸形。此外,抚养性别及心理情况也应符合个体性别。两性畸形又根据存在的性腺分为真两性畸形和假两性畸形。真两性畸形具有两种性腺,外生殖器一般多是混合型,假两性畸形分为男假两性畸形和女假两性畸形。前者性腺为睾丸,后者性腺为卵巢。现仅就两性畸形的诊断和处理谈谈我们的粗浅体会。1.关于真两性畸形的细胞遗传学检查,核型为46XX 者占60%,余者为46XY,46XX/46XY,46XX/47XXY,45XO/46XY 及45XO/47XXY 等嵌合体。因此真两性畸形染色体不能做为主要诊断依据。必须证实卵巢、睾丸同时存在或卵睾性腺。染色体检查对真两性畸形的手术选择有指导意义。 The correct sex should be in the chromosome (nuclear gender), external genital morphology, reproductive tract and gonads are the same. If there is a contradiction or both male and female characteristics, it should be referred to as amphipation. However, there are a few cases of genitalia near normal, may also be genital deformity. In addition, the gender and psychosocial support should also be gender-specific. Amphoteric deformity is divided according to the existence of the gonads true bisexual deformities and false bisexual deformities. True hermaphroditism with two gonads, external genitalia are mostly mixed, false bisexual deformity is divided into male false bisexual deformity and female false bisexual deformity. The former gonad testis, the latter gonads ovary. Now only on the diagnosis and treatment of amphiprotic talk about our experience. 1. About cytogenetic examination of true hermaphroditism, karyotype 46XX accounted for 60%, the remaining 46XY, 46XX / 46XY, 46XX / 47XXY, 45XO / 46XY and 45XO / 47XXY and other chimeras. So true amphiprotic chromosomes can not be used as the main basis for diagnosis. Must prove that the ovaries, testicles exist or egg gonads. Chromosome examination for the selection of true hermaphroditism has guiding significance.
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