2026-04-17T15:06:26Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/78892025-10-24T10:31:39Zcom_2072_378070com_2072_378040col_2072_378092

Prenatal dexamethasone treatment for classic 21-hydroxylase deficiency in Europe Nowotny, Hanna Neumann, Uta Tardy-Guidollet, Véronique Ahmed, S. Faisal Baronio, Federico Battelino, Tadej Yeste Fernandez, Diego Institut Català de la Salut [Nowotny H] Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, LMU München, Munich, Germany. [Neumann U] Centre for Chronic Sick Children, Department of Paediatric Endocrinology and Diabetology, Charité Universitätsmedizin Berlin, Berlin, Germany. [Tardy-Guidollet V] Laboratoire de Biochimie et Biologie Moléculaire, Hospices Civils de Lyon, Centre National de Référence ‘Développement Génital: du fœtus à l’adulte DEV-GEN’ Université Lyon I, Lyon, France. [Ahmed SF] Developmental Endocrinology Research Group, University of Glasgow, Glasgow, UK. [Baronio F] Paediatric Endocrinology Unit, Department of Medical and Surgical Sciences, S.Orsola-Malpighi University Hospital, Bologna, Italy. [Battelino T] Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre Ljubljana, University Children’s Hospital, Ljubljana, Slovenia. [Yeste D] Servei d’Endocrinologia Pediàtrica, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Universitat Autònoma de Barcelona, Bellaterra, Spain. CIBERER, ISCIII, Madrid, Spain Vall d'Hebron Barcelona Hospital Campus Hiperplàsia - Tractament Malalties congènites - Tractament DISEASES::Male Urogenital Diseases::Urogenital Abnormalities::Disorders of Sex Development::Adrenogenital Syndrome::Adrenal Hyperplasia, Congenital Other subheadings::Other subheadings::Other subheadings::/drug therapy ENFERMEDADES::enfermedades urogenitales masculinas::anomalías urogenitales::trastornos del desarrollo sexual::síndrome adrenogenital::hiperplasia suprarrenal congénita Otros calificadores::Otros calificadores::Otros calificadores::/farmacoterapia Dexamethasone; Prenatal Dexametasona; Prenatal Dexametasona; Prenatal Objective To assess the current medical practice in Europe regarding prenatal dexamethasone (Pdex) treatment of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. Design and methods A questionnaire was designed and distributed, including 17 questions collecting quantitative and qualitative data. Thirty-six medical centres from 14 European countries responded and 30 out of 36 centres were reference centres of the European Reference Network on Rare Endocrine Conditions, EndoERN. Results Pdex treatment is currently provided by 36% of the surveyed centres. The treatment is initiated by different specialties, that is paediatricians, endocrinologists, gynaecologists or geneticists. Regarding the starting point of Pdex, 23% stated to initiate therapy at 4–5 weeks postconception (wpc), 31% at 6 wpc and 46 % as early as pregnancy is confirmed and before 7 wpc at the latest. A dose of 20 µg/kg/day is used. Dose distribution among the centres varies from once to thrice daily. Prenatal diagnostics for treated cases are conducted in 72% of the responding centres. Cases treated per country and year vary between 0.5 and 8.25. Registries for long-term follow-up are only available at 46% of the centres that are using Pdex treatment. National registries are only available in Sweden and France. Conclusions This study reveals a high international variability and discrepancy in the use of Pdex treatment across Europe. It highlights the importance of a European cooperation initiative for a joint international prospective trial to establish evidence-based guidelines on prenatal diagnostics, treatment and follow-up of pregnancies at risk for CAH. This work was supported by the Deutsche Forschungsgemeinschaft (Heisenberg Professorship, 325768017 to N R and 314061271-TRR205 to N R and A H), the European Commission for funding EndoERN CHAFEA FPA grant no. 739527, the Eva Luise und Horst Köhler Stiftung & Else Kröner-Fresenius-Stiftung (2019_KollegSE.03 to H N) and the Stockholm County Council (Senior clinical research fellowship dnr RS 2019-1140 to S L), Stiftelsen Frimurare Barnhuset i Stockholm and Lisa and Johan Grönbergs Stiftelse. 2022-07-21T07:31:37Z 2022-07-21T07:31:37Z 2022-03-23 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Nowotny H, Neumann U, Tardy-Guidollet V, Ahmed SF, Baronio F, Battelino T, et al. Prenatal dexamethasone treatment for classic 21-hydroxylase deficiency in Europe. Eur J Endocrinol. 2022 Mar 23;186(5):K17–K24. 1479-683X https://hdl.handle.net/11351/7889 10.1530/EJE-21-0554 35235536 000802046600004 http://hdl.handle.net/11351/7889 eng European Journal of Endocrinology;186(5) https://doi.org/10.1530/EJE-21-0554 Attribution 4.0 International http://creativecommons.org/licenses/by/4.0/ info:eu-repo/semantics/openAccess application/pdf BioScientifica Scientia