TY - JOUR T1 - Management guidelines for disorders/different sex development (DSD) JO - Anales de Pediatría (English Edition) T2 - AU - Guerrero-Fernández,Julio AU - Azcona San Julián,Cristina AU - Barreiro Conde,Jesús AU - Bermúdez de la Vega,José Antonio AU - Carcavilla Urquí,Atilano AU - Castaño González,Luis Antonio AU - Martos Tello,José María AU - Rodríguez Estévez,Amaya AU - Yeste Fernández,Diego AU - Martínez Martínez,Leopoldo AU - Martínez-Urrutia,María José AU - Mora Palma,Cristina AU - Audí Parera,Laura SN - 23412879 M3 - 10.1016/j.anpede.2018.06.006 DO - 10.1016/j.anpede.2018.06.006 UR - https://analesdepediatria.org/en-management-guidelines-for-disorders-different-sex-articulo-S2341287918301637 AB - Disorders of sex development (DSD) include a wide range of anomalies among the chromosomal, gonadal, and phenotypic (genital) characteristics that define sexual differentiation. At present, a definition as Different sexual development (DSD) is currently preferred. They originate in the pre-natal stage and are classified according to the sex chromosomes present in the karyotype. The known genetic causes are numerous and heterogeneous, although, in some cases, they may be secondary to maternal factors and/or exposure to endocrine-disrupting chemicals (EDCs). The diagnosis and treatment of DSD always requires multidisciplinary medical and psychosocial care. An aetiological diagnosis needs the interaction of clinical, biochemical (hormonal), genetic, imaging and, sometimes, surgical examinations. The treatment should deal with sex assignment, the possible need for hormone replacement therapy (adrenal if adrenal function is impaired, and with sex steroids from pubertal age if gonadal function is impaired), as well as the need for surgery on genital structures (currently deferred when possible) and/or on gonads (depending on the risk of malignancy), the need of psychosocial support and, finally, an adequate organisation of the transition to adult medical specialties. Patient Support Groups have a fundamental role in the support of families, as well as the interaction with professional and social media. The use of Registries and the collaboration between professionals in Working Groups of national and international medical societies are crucial for improving the diagnostic and therapeutic tools required for the care of patients with DSD. ER -