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Disorders of sex development — new terminology and new recommendations for therapeutic management
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Abstract
Disorders of sex development (DSD) as a medical term appeared relatively recently. Disorders of sex differentiation resulting in abnormalities in external and/or internal genitals were called hermaphroditism or intersexuality, and were regarded by patients and their families as stigmatizing and causing mental suffering. Thus was born the idea of a more neutral terminology. In 2006, a consensus with proposals to introduce a new classification and treatment for DSD was published. The consensus was established by experts from the American Lawson Wilkins Pediatric Endocrine Society (LWPES) and the European Society for Pediatric Endocrinology (ESPE). The new definition includes all inherited disorders in which there is abnormal development of the chromosomal, gonadal, genital, phenotypic and psychic sex. Attempts to develop more optimal diagnostic and therapeutic recommendations are being made simultaneously with the new definition. However, work is still underway to develop an unambiguous algorithm in children and adults with DSD.
Abstract
Disorders of sex development (DSD) as a medical term appeared relatively recently. Disorders of sex differentiation resulting in abnormalities in external and/or internal genitals were called hermaphroditism or intersexuality, and were regarded by patients and their families as stigmatizing and causing mental suffering. Thus was born the idea of a more neutral terminology. In 2006, a consensus with proposals to introduce a new classification and treatment for DSD was published. The consensus was established by experts from the American Lawson Wilkins Pediatric Endocrine Society (LWPES) and the European Society for Pediatric Endocrinology (ESPE). The new definition includes all inherited disorders in which there is abnormal development of the chromosomal, gonadal, genital, phenotypic and psychic sex. Attempts to develop more optimal diagnostic and therapeutic recommendations are being made simultaneously with the new definition. However, work is still underway to develop an unambiguous algorithm in children and adults with DSD.
Keywords
sex differentiation, chromosomal sex, gonadal sex, genital sex, phenotypic sex, psychic sex, disorders of sex development, 46,XY DSD, 46,XX DSD


Title
Disorders of sex development — new terminology and new recommendations for therapeutic management
Journal
Journal of Sexual and Mental Health
Issue
Article type
Review paper
Page views
2206
Article views/downloads
36550
Bibliographic record
Seksuologia Polska 2014;12(2).
Keywords
sex differentiation
chromosomal sex
gonadal sex
genital sex
phenotypic sex
psychic sex
disorders of sex development
46
XY DSD
46
XX DSD
Authors
Katarzyna Bajszczak
Maria Szarras-Czapnik
Jolanta Słowikowska-Hilczer