hrp0086p1-p697 | Endocrinology and Multisystemic Diseases P1 | ESPE2016

Contiguous Gene Syndrome Involving DAX1 Deletion with Congenital Adrenal Insufficiency

Dafsari Roschan Salimi , Haas Dorothea , Leube Barbara , Eichhorn Joachim G. , Mayatepek Ertan , Meissner Thomas , Kummer Sebastian

Background: In contrast to monogenic diseases, contiguous gene syndrome (CGS) describes a clinical phenotype caused by a deletion or duplication of several neighbouring genes. Angelman or Williams-Beuren syndrome are examples demonstrating that deletion of several adjacent genes causes a complex clinical syndrome. However, CGS are very rare events in pediatric endocrinology, and require knowledge of clinical associations pointing towards specific potentially affected genes in ...