Prenatal dexamethasone treatment for classic 21-hydroxylase deficiency in Europe

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • Hanna Nowotny - , Ludwig Maximilian University of Munich (Author)
  • Uta Neumann - , Charité – Universitätsmedizin Berlin (Author)
  • Veronique Tardy-Guidollet - , Universite Claude Bernard Lyon 1 (Author)
  • S. Faisal Ahmed - , University of Glasgow (Author)
  • Federico Baronio - , University of Bologna (Author)
  • Tadej Battelino - , University of Ljubljana (Author)
  • Jerome Bertherat - , Assistance publique – Hôpitaux de Paris (Author)
  • Oliver Blankenstein - , Charité – Universitätsmedizin Berlin (Author)
  • Marco Bonomi - , University of Milan, IRCCS Istituto Auxologico Italiano - Milano (Author)
  • Claire Bouvattier - , Assistance publique – Hôpitaux de Paris, Reference center for genital development: from fetus to adult (DEV-GEN) (Author)
  • Aude Brac De la Perrière - , Hospices civils de Lyon (Author)
  • Sara Brucker - , University of Tübingen (Author)
  • Marco Cappa - , IRCCS Ospedale pediatrico Bambino Gesù - Roma (Author)
  • Philippe Chanson - , Université Paris-Saclay (Author)
  • Hedi L.Claahsen Van der Grinten - , Radboud University Nijmegen (Author)
  • Annamaria Colao - , Universita' di Napoli Federico II (Author)
  • Martine Cools - , Ghent University (Author)
  • Justin H. Davies - , University Hospital Southampton NHS Foundation Trust (Author)
  • Helmut Gunther Dorr - , Friedrich-Alexander University Erlangen-Nürnberg (Author)
  • Wiebke K. Fenske - , University of Bonn (Author)
  • Ezio Ghigo - , University of Turin (Author)
  • Roberta Giordano - , University of Turin (Author)
  • Claus H. Gravholt - , Aarhus University (Author)
  • Angela Huebner - , Department of Paediatrics, Technische Universität Dresden (Author)
  • Eystein Sverre Husebye - , University of Bergen (Author)
  • Rebecca Igbokwe - , Birmingham Women's and Children's NHS Foundation Trust (Author)
  • Anders Juul - , University of Copenhagen (Author)
  • Florian W. Kiefer - , Medical University of Vienna (Author)
  • Juliane Leger - , Université Paris 13 (Author)
  • Rita Menassa - , Universite Claude Bernard Lyon 1 (Author)
  • Gesine Meyer - , University Hospital Frankfurt (Author)
  • Vassos Neocleous - , Cyprus Institute of Neurology and Genetics (Author)
  • Leonidas A. Phylactou - , Cyprus Institute of Neurology and Genetics (Author)
  • Julia Rohayem - , University of Münster (Author)
  • Gianni Russo - , Vita-Salute San Raffaele University (Author)
  • Carla Scaroni - , University of Padua (Author)
  • Philippe Touraine - , Sorbonne Université (Author)
  • Nicole Unger - , University of Duisburg-Essen (Author)
  • Jarmila Vojtkova - , Comenius University (Author)
  • Diego Yeste - , Autonomous University of Barcelona, Instituto de Salud Carlos III (Author)
  • Svetlana Lajic - , Karolinska Institutet (Author)
  • Nicole Reisch - , Ludwig Maximilian University of Munich (Author)

Abstract

Objective: To assess the current medical practice in Europe regarding prenatal dexamethasone (Pdex) treatment of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase defic iency. 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 ce ntres. 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.

Details

Original languageEnglish
Pages (from-to)K17-K24
JournalEuropean journal of endocrinology
Volume168
Issue number5
Publication statusPublished - May 2022
Peer-reviewedYes

External IDs

PubMed 35235536

Keywords