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- W2749093853 abstract "Complete androgen insensitivity (CAIS) occurs in approximately 1 in 13,000 to 1 in 62,000 girls and results in a female phenotype but the presence of XY gonads. Fortunately there is very little controversy in regards to gender rearing these patients as females. Rather, the controversy centers primarily around the timing of the gonadectomy. All clinicians agree that XY gonads should be removed in patients with CAIS to avoid the potential risk of germ cell malignancies but when this should occur continues to be a source of debate. In this clinical opinion, I have asked Dr. Lisa Allen, a pediatric gynecologist, and Dr. Darcie Kiddoo, a pediatric urologist, to provide their opinion in favor of either an early or a delayed gonadectomy. Opinion One: A Case for Delayed GonadectomyJournal of Pediatric and Adolescent GynecologyVol. 22Issue 6PreviewComplete androgen insensitivity (CAIS) is an X-linked condition, involving mutations in the androgen receptor, specifically in a gene localized to the long arm of the X chromosome, Xq11-12. The estimated incidence is 1:13,000–1:20,000.1,2 As the karyotype is 46XY, under the direction of testis determining factor on the Y chromosome the gonads develop into testis. The Sertoli cells of the testis produce mullerian inhibiting substance (MIS) bilaterally, which results in inhibition of the mullerian ducts during embryo development. Full-Text PDF Opinion Two: A Case For Early GonadectomyJournal of Pediatric and Adolescent GynecologyVol. 22Issue 6PreviewAndrogen insensitivity is the most common cause of the undervirilized male and results from testosterone receptor abnormalities. Phenotypic presentation ranges from complete external feminization (complete androgen insensitivity) to ambiguous genitalia (partial androgen insensitivity) and finally infertile males. The diagnosis of complete androgen insensitivity is generally made as a result of primary amenorrhea, or after finding a testicle at inguinal herniorrhaphy. Earlier diagnosis is becoming increasingly more common with amniocentesis. Full-Text PDF" @default.
- W2749093853 created "2017-08-31" @default.
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- W2749093853 date "2009-12-01" @default.
- W2749093853 modified "2023-09-26" @default.
- W2749093853 title "Introduction" @default.
- W2749093853 doi "https://doi.org/10.1016/j.jpag.2009.08.003" @default.
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