Abstract

* Abbreviation: DSD — : disorder of sexual development The birth of a child with a disorder of sexual development (DSD) is challenging from multiple perspectives, particularly that of sex assignment. Patients, parents, and health care professionals who care for patients with DSDs understand that the physical, biochemical, and mental characteristics of these patients better fit a model of fluidity. Yet, sex continues to be predominantly binary, and the decision that families make early on often has medical, social, and legal implications. Until recently, all US birth certificates listed either male or female sex. However, in December 2016, the state of New York issued the nation’s first intersex birth certificate to a 55-year-old individual who was born with a DSD consisting of male chromosomes, female external genitalia, and mixed internal genitalia.1 This legal landmark challenges the traditional legal framework of the binary division of sex. Therefore, we are taking this opportunity to discuss the fluidity of sex assignment and how this unprecedented event may provide an opportunity to improve the care of patients with a DSD. Multiple terms and classification systems have been used to describe patients with DSDs. The classification systems were initially based on anatomy and were modified over time as the chromosomal basis of sex, gonadal characteristics, and sexual differentiation were better understood. However, the subclassifications had the qualifiers of male or female, implying the sex to be assigned.2 It was not until 2006 that the Pediatric Endocrine Society and the European Society of Paediatric Endocrinology revised the nomenclature into a classification system … Address correspondence to Lefkothea Karaviti, MD, PhD, Baylor College of Medicine, Section of Endocrinology and Diabetes, Department of Pediatrics, 6701 Fannin St, Ste. D.1060.01, Houston, TX 77030. E-mail: lpkaravi{at}texaschildrens.org

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