title: Male gender identity in children with 46,XX DSD with congenital adrenal hyperplasia after delayed presentation in mid-childhood.
abstract: OBJECTIVE
  Girls with congenital adrenal hyperplasia (CAH) diagnosed at birth have some masculine behaviors but rarely convert to male gender. In developing countries, however, diagnosis and treatment (with secondary androgen suppression) are delayed. We aimed to assess effect of delayed treatment of CAH on gender identity.
  
  
  METHODS
  As part of a cross-sectional, case-control study of children with disorders of sex development (DSD), there were 11 patients with CAH. Patients and caregivers answered a questionnaire about gender identity, and behavior was assessed by observing toy play. Patients were examined for Prader score and gender identity.
  
  
  RESULTS
  Of 11 CAH patients initially raised as girls, 3 (27%) had converted to male gender at presentation (5, 9, 9years) (Prader 3, 4, 4). Of the remaining 8 patients, one 4-year-old (Prader 2) had a male gender identity score. The remaining girls (2-13years, mean 8.1) (Prader 1-3) had gender identity scores in the female range.
  
  
  CONCLUSIONS
  One third (4/11) of CAH patients presenting in mid-childhood had male gender identity scores, and ¾ had assumed male gender role. Although social and cultural factors are important in developing countries, this result suggests that delayed treatment may trigger male gender identity, and delayed female genital surgery may be unwise.
authors.affiliation: Department of Pediatric Surgery, Chittagong Medical College Hospital, Chittagong, Bangladesh.
authors.forename: Tanvir Kabir
authors.initials: TK
authors.lastname: Chowdhury
authors.affiliation: Department of Pediatric Surgery, Chittagong Medical College Hospital, Chittagong, Bangladesh.
authors.forename: Kamrun
authors.initials: K
authors.lastname: Laila
authors.affiliation: Department of Urology, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; F Douglas Stephens Surgical Research Laboratory, Murdoch Children's Research Institute, Melbourne, Australia.
authors.forename: John M
authors.initials: JM
authors.lastname: Hutson
authors.affiliation: Department of Pediatric Surgery, Chittagong Medical College Hospital, Chittagong, Bangladesh. Electronic address: proftahmina@gmail.com.
authors.forename: Tahmina
authors.initials: T
authors.lastname: Banu
mesh_terms: D058489:46, XX Disorders of Sex Development / Q000150:complications / Q000175:diagnosis / Q000523:psychology*; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000150:complications / Q000175:diagnosis / Q000235:genetics / Q000523:psychology*; D001459:Bangladesh; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D057210:Delayed Diagnosis; D003906:Developing Countries; D005260:Female; D005783:Gender Identity; D006801:Humans; D008297:Male; D011795:Surveys and Questionnaires
keywords: Congenital adrenal hyperplasia; Disorders of sex development; Gender identity