Analysis of Presentations and Outcomes of Care of Children with Disorders of Sexual Development in a Nigerian Hospital

Journal of Pediatric and Adolescent Gynecology
Abdulrasheed A NasirJames O Adeniran

Abstract

To describe the presentation, diagnosis, management, and short-term outcome of children with disorders of sexual development (DSD) in the context of multidisciplinary team care. Prospective descriptive study. University Teaching Hospital. All children who presented with genital ambiguity. Records of all patients diagnosed and managed for DSD between January 2011 and December 2016 were reviewed. The care pathway included clinical, laboratory, internal genitalia evaluation, and panel (including parents) meeting. Fifteen children presented with DSD at a median age of 20 months. Only 5/15 (33.3%) presented in the neonatal period. Ten of fifteen patients (66.7%) presented with genital ambiguity. Ovotesticular DSD was the most common diagnosis (9/15; 60%). Seven of the patients were genetically female (46, XX), 1 was genetically male (46, XY) and 1 without genetic diagnosis. Six patients were assigned male gender and they underwent male genitoplasty. Five of them had excision of Müllerian structures with gonadectomy. Three of fifteen patients (20%) were diagnosed as 46, XX DSD, at a median age of 7 years. All of them were due to congenital adrenal hyperplasia and underwent female genitoplasty. Two patients were diagnosed as XY, DSD. ...Continue Reading

Citations

Feb 19, 2020·Journal of Pediatric Endocrinology & Metabolism : JPEM·Suzanne Ngo Um SapPaul Koki Ndombo

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