Expertsʼ Opinion on the Prenatal Therapy of Congenital Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency – Guideline of DGKED in cooperation with DGGG (S1-Level, AWMF Registry No. 174/013, July 2015)

H. G. Dörr; G. Binder; N. Reisch; U. Gembruch; P. G. Oppelt; P. Wieacker; J. Kratzsch

doi:10.1055/s-0041-109717

Geburtshilfe und Frauenheilkunde, Table of Contents

Geburtshilfe Frauenheilkd 2015; 75(12): 1232-1238
DOI: 10.1055/s-0041-109717

Guideline

GebFra Science

Georg Thieme Verlag KG Stuttgart · New York

Expertsʼ Opinion on the Prenatal Therapy of Congenital Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency – Guideline of DGKED^[*] in cooperation with DGGG (S1-Level, AWMF Registry No. 174/013, July 2015)

Adrenogenitales Syndrom mit 21-Hydroxylase-Defekt (AGS) – Stellungnahme zur pränatalen Therapie. Leitlinie der DGKED mit Beteiligung der DGGG (S1-Level, AWMF-Registernummer 174/013, Juli 2015)

H. G. Dörr

¹Kinder- und Jugendklinik des Universitätsklinikums Erlangen, Erlangen

,

G. Binder

²Univ.-Kinderklinik Tübingen, Sektion Endokrinologie, Tübingen

,

N. Reisch

³Medizinische Klinik und Poliklinik IV. Klinikum der Universität München, München

,

U. Gembruch

⁴Abteilung für Geburtshilfe und Pränatalmedizin der Universität Bonn, Bonn

,

P. G. Oppelt

⁵Kinder- und Jugendgynäkologie, Frauenklinik des Universitätsklinikums Erlangen, Erlangen

,

P. Wieacker

⁶Institut für Humangenetik, Universitätsklinikum Münster, Münster

,

J. Kratzsch

⁷Institut für Laboratoriumsmedizin, Klinische Chemie und Molekulare Diagnostik der Universität Leipzig, Leipzig

› Author Affiliations

Abstract

Purpose: This guideline of the German Society of Pediatric Endocrinology and Diabetology (DGKED) is designed to be expertsʼ opinion on the current concept of prenatal therapy for congenital adrenal hyperplasia due to 21-hydroxylase deficiency (CAH). Several scientific medical societies have also participated in the guideline. It aims to offer guidance to physicians when they counsel affected families about prenatal therapy. Methods: The experts commissioned by the medical societies developed a consensus in an informal process. The consensus was subsequently confirmed by the steering committees of the respective medical societies. Recommendations: Prenatal CAH therapy is an experimental therapy. We recommend designing and using standardized protocols for the prenatal diagnosis, therapy and long-term follow-up of women and children treated prenatally with dexamethasone. If long-term follow-up is not possible, then prenatal therapy should not be performed.

Zusammenfassung

Ziel: Diese Leitlinie der Deutschen Gesellschaft für Kinderendokrinologie und -diabetologie (DGKED) e. V. unter Beteiligung mehrerer Fachgesellschaften wie der DGGG wurde als Stellungnahme von Experten zum derzeitigen Stand der pränatalen Therapie des Adrenogenitalen Syndroms mit 21-Hydroxylasedefekt konzipiert. Sie soll den beteiligten Ärzten als Orientierungshilfe bei der Entscheidung dienen, um betroffene Familien bei der pränatalen Therapie besser beraten zu können. Methoden: Die von den beteiligten Fachgesellschaften beauftragten Experten entwickelten in einem informellen Prozess einen Konsensus. Anschließend wurde der Konsensus von den Vorständen der beteiligten Fachgesellschaften bestätigt. Empfehlungen: Die pränatale AGS-Therapie ist eine experimentelle Therapie. Für die pränatale Diagnostik und Therapie sollen standardisierte Protokolle entworfen und verwendet werden. Dies gilt auch für die Langzeitnachsorge der Frauen und Kinder, die pränatal mit Dexamethason behandelt wurden. Wenn die Langzeitnachsorge der Kinder nicht gewährleistet ist, dann soll auch keine pränatale Therapie durchgeführt werden.

Key words

CAH - prenatal therapy - dexamethasone - off-label use

Schlüsselwörter

AGS - pränatale Therapie - Dexamethason - Off-Label-Use

Full Text

References

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Supplementary Material

German version of this article

Expertsʼ Opinion on the Prenatal Therapy of Congenital Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency – Guideline of DGKED[*] in cooperation with DGGG (S1-Level, AWMF Registry No. 174/013, July 2015)

Expertsʼ Opinion on the Prenatal Therapy of Congenital Adrenal Hyperplasia (CAH) Due to 21-Hydroxylase Deficiency – Guideline of DGKED^[*] in cooperation with DGGG (S1-Level, AWMF Registry No. 174/013, July 2015)