Extensive Literature Review of 46, XX Newborns with Congenital Adrenal Hyperplasia (CAH) and Severe Genital Masculinization: Should They be Assigned and Reared Male?
Tom Mazur1, Jennifer O’donnell2, Peter A Lee31Clinical Associate Professor of Psychiatry and Pediatrics, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo and John R. Oishei Hospital, Buffalo, NY2M.D. Candidate, Class of 2024 Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY
3Professor Emeritus, Department of Pediatrics, Penn State College of Medicine, Penn State Health, Hershey, PA
INTRODUCTION: 46, XX individuals born with severely masculinized genitals due to congenital adrenal hyperplasia (CAH) who have been assigned males at birth and reared male can successfully establish a male gender identity/role, find employment, marry, function sexually with a female partner, and develop positive mental health status. While there were a few individuals who reportedly did not fare well or who changed gender to female, the majority of those identifying as males appear to have an overall good quality of life. Parental/family support, along with the support of others, appears essential to a positive outcome as a male or as a female. Reasons are given to support why serious consideration of a male gender assignment and rearing, in certain situations, is justified and should be seriously considered. Disorders of Sex Differentiation (DSD) teams should inform parents of the option of male assignment and rearing in 46, XX CAH infants with severe genital masculinization, a rare condition. To provide this option is concordant with the principles of ethics, transparency and with the Endocrine Society Guidelines and the American Academy of Pediatrics’ policy of fully informed consent.
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Corresponding Author: Tom Mazur, United States of America
Manuscript Language: English
