Mixed gonadal dysgenesis in Yaoundé: A preliminary experience about three cases.

Autor: Mouafo Tambo FF; Pediatric Sugery Service, Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon., Dahoun S; University Teaching Hospitals of Geneva, Geneva, Switzerland., Kamadjou C; Pediatric Sugery Service, Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon., Nwaha Makon AS; Pediatric Sugery Service, Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon., Fossi G; Pediatric Sugery Service, Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon., Andze OG; Pediatric Sugery Service, Gyneco Obstetric and Paediatric Hospital, Yaoundé, Cameroon., Sosso MA; Department of Surgery and Subspecialties, Faculty of Medicine and Biomedical Sciences, University of Yaoundé, Yaoundé, Cameroon., Mure PY; Lyon Teaching Hospital, Lyon, France.
Jazyk: angličtina
Zdroj: African journal of paediatric surgery : AJPS [Afr J Paediatr Surg] 2016 Jul-Sep; Vol. 13 (3), pp. 145-9.
DOI: 10.4103/0189-6725.187822
Abstrakt: Unlabelled: Mixed gonadal dysgenesis is characterised by unilateral chromosomal abnormality, which is probably the result of anaphase lag during mitosis. The 45, XO/46, XY karyotype is the most common form of mosaicism involving the Y chromosome. It is a rare clinical entity with a worldwide incidence of 1.5/10,000 live births. Its epidemiology in Sub-Saharan Africa is not known. This study reports experience in the management of 3 cases at the Yaounde Gynecologic-Obstetric and Paediatric Hospital. From November 2009 to November 2014, 3 cases were successfully managed at our institution.
Results: All patients presented with asymmetrical gonadal differentiation. On one side of the body, a poorly-developed testicular gonad and on the other side a gonadal streak. A persistent Mόllerian remnant was equally found in the 3 cases. Management of mixed gonadal dysgenesis should be done in tertiary health care centres. A multidisciplinary team approach is recommended.
Databáze: MEDLINE
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