hrp0086p2-p422 | Gonads & DSD P2 | ESPE2016

Late Clinical Presentation, Biological Assessment and Management of PAIS in a Developing Country

Brindusa Gorduza Daniela , Tambo Mouafo Faustin , Gay Claire-Lise , Plotton Ingrid , Birraux Jacques , Dahoun Sophie , Morel Yves , Mouriquand Pierre , Le Coultre Claude , Mure Pierre-Yves

Background: Partial androgen insensitivity syndromes (PAIS) are rare 46,XY DSD (disorder of sex development).Objective and hypotheses: Three families with PAIS (six patients) are reported, focusing on their phenotype and treatment depending on sex of rearing. Biological investigations and surgical management are described.Method: Between 2009 and 2015 a consultation for uro-genital malformations in pediatric patients was set up in ...