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Recent advances in clinical management of androgen insensitive syndrome

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Author:: No author available

Journal Title:: Chinese Journal of Pediatric Surgery

Issue:: 9

DOI:: 10.3760/cma.j.cn421158-20200325-00199

Key Word:: 雄激素类;雄激素不敏感综合征;性别;Androgens;Androgen insensitivity syndrome;Sexuality

Abstract： As one of the most common sexual dysplasia diseases, androgen insensitivity syndrome (AIS) is divided into complete, partial and mild types according to the degree of androgen receptor function impairment. Complete androgen insensitivity syndrome (CAIS) shows a normally feminized external genital phenotype while partial androgen insensitivity syndrome (PAIS) usually presents with vague external genitalia at birth. Mild androgen insensitivity syndrome (MAIS) is considered in adolescent males with feminized breasts and infertility. The endocrinology of AIS is characterized by persistent androgen resistance and the core of its clinical management lies in gender allocation. After assessments of a multidisciplinary team, CAIS is assigned as the female and most cases of PAIS belong to the males. And vigilance is needed for gender redistribution due to gender anxiety in adulthood. At the same time, plastic surgery of external genital after sex allocation, long-term hormone replacement, optimal timing of gonadectomy and fertility potential and genetic recommendations all require personalized management. This review summarized the latest researches in clinical management of androgen insensitivity syndrome.

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