Prenatal dexamethasone treatment for classic 21-hydroxylase deficiency in Europe
Nowotny, Hanna (Universität München)
Neumann, Uta (Charité - Universitätsmedizin Berlin)
Tardy-Guidollet, Véronique (Université Lyon I)
Ahmed, S. Faisal (University of Glasgow)
Baronio, Federico (Paediatric Endocrinology Unit)
Battelino, Tadej (Department of Endocrinology)
Bertherat, Jérôme (Hôpitaux Universitaires Paris-Centre)
Blankenstein, Oliver (Charité - Universitätsmedizin Berlin)
Bonomi, Marco (University of Milan)
Bouvattier, Claire (Hôpital de Bicêtre)
Brac de la Perrière, Aude (Fédération d'Endocrinologie)
Brucker, Sara (Department of Women's Health)
Cappa, Marco (Endocrinology Unit)
Chanson, Philippe (Assistance Publique-Hôpitaux de Paris)
Claahsen-van der Grinten, Hedi L. (Amalia Children's Hospital)
Colao, Annamaria (Universita' Federico II di Napoli)
Cools, Martine (Universitair Ziekenhuis Gent)
Davies, Justin H. (Universitair Ziekenhuis Gent)
Dörr, Helmut-Günther (Universitätsklinikum Erlangen)
Fenske, Wiebke K. (University Hospital Bonn (Bonn, Alemanya))
Ghigo, Ezio (University of Turin)
Giordano, Roberta (University of Turin)
Gravholt, Claus H. (Aarhus University Hospital (Aarhus, Dinamarca))
Huebner, Angela (Universitätsklinikum Dresden)
Husebye, Eystein Sverre (Haukeland University Hospital (Bergen, Noruega))
Igbokwe, Rebecca (Birmingham Women's Hospital)
Juul, Anders (Copenhagen University Hospital)
Kiefer, Florian W. (University of Vienna)
Léger, Juliane (AP-HP Paris Nord Université de Paris)
Menassa, Rita (Université Lyon I)
Meyer, Gesine (Goethe University Frankfurt)
Neocleous, Vassos (Cyprus School of Molecular Medicine)
Phylactou, Leonidas A. (Cyprus School of Molecular Medicine)
Rohayem, Julia (Centre of Reproductive Medicine and Andrology)
Russo, Gianni (Scientific Institute San Raffaele)
Scaroni, Carla (Università di Padova)
Touraine, Philippe (Sorbonne Université)
Unger, Nicole (University Hospital Essen (Alemanya))
Vojtková, Jarmila (Comenius University in Bratislava)
Yeste Fernández, Diego (Hospital Universitari Vall d'Hebron)
Lajic, Svetlana (Karolinska University Hospital and Karolinska Institutet (Suècia))
Reisch, Nicole (Medizinische Klinik und Poliklinik IV)
Universitat Autònoma de Barcelona

Fecha:	2022
Resumen:	To assess the current medical practice in Europe regarding prenatal dexamethasone (Pdex) treatment of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. 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. 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. 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.
Derechos:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Lengua:	Anglès
Documento:	Article ; recerca ; Versió publicada
Publicado en:	European journal of endocrinology, Vol. 186 (march 2022) , p. K17-K24, ISSN 1479-683X

DOI: 10.1530/EJE-21-0554
PMID: 35235536

8 p, 867.3 KB

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