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Nowotny, Hanna ; Neumann, Uta ; Tardy-Guidollet, Véronique ; Ahmed, S. Faisal ; Baronio, Federico ; Battelino, Tadej ; Bertherat, Jérôme ; Blankenstein, Oliver ; Bonomi, Marco ; Bouvattier, Claire ; Brac de la Perrière, Aude ; Brucker, Sara ; Cappa, Marco ; Chanson, Philippe ; Claahsen-van der Grinten, Hedi L. ; Colao, Annamaria ; Cools, Martine ; Davies, Justin H. ; Dörr, Helmuth-Günther ; Fenske, Wiebke Kristin ; Ghigo, Ezio ; Giordano, Roberta ; Gravholt, Claus H. ; Huebner, Angela ; Husebye, Eystein Sverre ; Igbokwe, Rebecca ; Juul, Anders ; Kiefer, Florian ; Léger, Juliane ; Menassa, Rita ; Meyer, Gesine ; Neocleous, Vassos ; Phylactou, Leonidas A. ; Rohayem, Julia ; Russo, Gianni ; Scaroni, Carla ; Touraine, Philippe ; Unger, Nicole ; Vojtková, Jarmila ; Yeste, Diego ; Lajic, Svetlana ; Reisch, Nicole
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.