Hanna Nowotny, Uta Neumann, Véronique Tardy-Guidollet, S. Faisal Ahmed, Federico Baronio, Tadej Battelino, Jérôme Bertherat, Oliver Blankenstein, Marco Bonomi, Claire Bouvattier, Aude Brac de la Perrière, Sara Brucker, Marco Cappa, Philippe Chanson, Hedi L. Claahsen-van der Grinten, Annamaria Colao, Martine Cools, Justin H. Davies, Helmuth-Günther Dörr, Wiebke Kristin Fenske, Ezio Ghigo, Roberta Giordano, Claus H. Gravholt, Angela Huebner, Eystein Sverre Husebye, Rebecca Igbokwe, Anders Juul, Florian Kiefer, Juliane Léger, Rita Menassa, Gesine Meyer, Vassos Neocleous, Leonidas A. Phylactou, Julia Rohayem, Gianni Russo, Carla Scaroni, Philippe Touraine, Nicole Unger, Jarmila Vojtková, Diego Yeste, Svetlana Lajic, Nicole Reisch
- 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.
MetadatenAuthor: | Hanna NowotnyORCiDGND, Uta NeumannORCiD, Véronique Tardy-Guidollet, S. Faisal AhmedORCiD, Federico BaronioORCiD, Tadej BattelinoORCiDGND, Jérôme BertheratORCiD, Oliver BlankensteinGND, Marco BonomiORCiD, Claire BouvattierORCiD, Aude Brac de la PerrièreORCiD, Sara BruckerORCiDGND, Marco CappaORCiDGND, Philippe ChansonORCiDGND, Hedi L. Claahsen-van der GrintenORCiD, Annamaria ColaoORCiDGND, Martine CoolsORCiD, Justin H. DaviesORCiD, Helmuth-Günther DörrORCiDGND, Wiebke Kristin FenskeGND, Ezio GhigoORCiDGND, Roberta GiordanoORCiD, Claus H. GravholtORCiD, Angela HuebnerORCiD, Eystein Sverre HusebyeORCiD, Rebecca Igbokwe, Anders JuulORCiD, Florian KieferORCiDGND, Juliane LégerORCiD, Rita Menassa, Gesine MeyerORCiDGND, Vassos NeocleousORCiD, Leonidas A. PhylactouORCiD, Julia RohayemORCiDGND, Gianni Russo, Carla ScaroniORCiD, Philippe TouraineORCiDGND, Nicole UngerGND, Jarmila VojtkováORCiD, Diego YesteORCiD, Svetlana LajicORCiD, Nicole ReischORCiDGND |
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URN: | urn:nbn:de:hebis:30:3-633152 |
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DOI: | https://doi.org/10.1530/EJE-21-0554 |
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ISSN: | 1479-683X |
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Parent Title (English): | European journal of endocrinology |
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Publisher: | Oxford University Press |
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Place of publication: | Oxford |
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Document Type: | Article |
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Language: | English |
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Date of Publication (online): | 2022/03/23 |
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Date of first Publication: | 2022/03/23 |
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Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
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Release Date: | 2023/04/03 |
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Volume: | 186 |
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Issue: | 5 |
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Page Number: | 8 |
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First Page: | K17 |
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Last Page: | K24 |
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Note: | 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. |
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Note: | This work was supported by the European Reference Network on Rare Endocrine Conditions. |
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HeBIS-PPN: | 508736633 |
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Institutes: | Medizin |
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Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
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Sammlungen: | Universitätspublikationen |
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Licence (German): | Creative Commons - CC BY - Namensnennung 4.0 International |
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