Journal of the Japanese Society of Pediatric Surgeons
Online ISSN : 2187-4247
Print ISSN : 0288-609X
ISSN-L : 0288-609X
Two Cases of Complete Androgen Insensitivity Syndrome Diagnosed at Laparoscopic Inguinal Hernia Repair : The Efficacy of Screening by Laparoscopy
Hiroomi OkuyamaYoshiyuki ShimizuTakashi SasakiYuko TazukeAkio KubotaHisayoshi Kawahara
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2010 Volume 46 Issue 2 Pages 280-283

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Abstract
Complete androgen insensitivity syndrome (CAIS) is characterized by normal female appearance and external genitalia in the presence of a 46XY karyotype. Although testosterone is produced at the normal testis, androgen receptor defects permit the development of female external anatomy. Because of the normal female appearance, these patients are raised as girls, and the diagnosis of CAIS is usually not made until amenorrhea is investigated. Herein we report 2 cases of CAIS diagnosed at laparoscopic inguinal hernia repair. In both cases, the laparoscope revealed the presence of the bilateral intra abdominal testis and the lack of female internal genitalia. The postoperative karyotyping (46XY) confirmed the diagnosis of CAIS. In our institute, the incidence of CAIS in girls with inguinal hernia was 0.4% (2/504), suggesting that any girls with inguinal hernia have a high likelihood of CAIS. Checking of internal genitalia at laparoscopic inguinal hernia repair is thought to be an easy and useful CAIS screening procedure.
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© 2010 The Japanese Society of Pediatric Surgeons

この記事はクリエイティブ・コモンズ [表示 - 非営利 - 継承 4.0 国際]ライセンスの下に提供されています。
https://creativecommons.org/licenses/by-nc-sa/4.0/deed.ja
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