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Vol. 58, No. 4, 2002
Issue release date: 2002
Section title: ESPE Bulletin Board
Horm Res 2002;58:196–205
(DOI:10.1159/000065492)

Management of Congenital Adrenal Hyperplasia: Results of the ESPE Questionnaire

Riepe F.G. · Krone N. · Viemann M. · Partsch C.-J. · Sippell W.G.
Division of Paediatric Endocrinology, Department of Paediatrics, Christian Albrechts University, Kiel, Germany

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Article / Publication Details

First-Page Preview
Abstract of ESPE Bulletin Board

Received: 6/10/2002
Accepted: 7/4/2002
Published online: 9/19/2002

Number of Print Pages: 10
Number of Figures: 2
Number of Tables: 7

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP

Abstract

The management of children and adolescents with congenital adrenal hyperplasia (CAH) remains difficult. To assess the current European practice in diagnosis and management of CAH, an ESPE (European Society for Paediatric Endocrinology) survey was circulated in 2000/2001. The questionnaire was answered by 34% of ESPE members, representing 125 institutions which cared for 6,553 CAH patients. Paediatric endocrinologists, surgeons, gynaecologists, geneticists, and psychologists are involved in the immediate care of the CAH neonate and his family. 44% of centres take part in neonatal screening programmes. In families at risk, prenatal dexamethasone therapy is started at a median gestational age of 6 weeks in a median dose of 20 µg/kg/day. 53% reported maternal adverse events, 8% observed adverse fetal events. Regarding feminizing surgery, 33% reported simultaneous clitoric reduction and vaginoplasty during infancy. However, clitoridectomy is still reported by 13% of centres, and vaginal dilatations have been performed by 27%. Although 71% of female CAH patients with psychosexual problems, only 17% undertake routine psychodiagnostics and counselling. Hydrocortisone is the substance used for the treatment of CAH during growth in 84%; the median dose (mg/m²/day) is 17.5 in infants, 15 in children and adolescents, and 13.75 in adults. The glucocorticoid dose is increased two- to sixfold during intercurrent stress. Mineralocorticoid is administered in cases of clinically manifest salt wasting and of elevated plasma renin activity, to decrease high glucocorticoid doses, or according to genotype. All participating ESPE members feel the need for further improvement in prenatal diagnosis and treatment, compliance during puberty, screening programmes, psychological aspects, and corrective surgery.


Article / Publication Details

First-Page Preview
Abstract of ESPE Bulletin Board

Received: 6/10/2002
Accepted: 7/4/2002
Published online: 9/19/2002

Number of Print Pages: 10
Number of Figures: 2
Number of Tables: 7

ISSN: 1663-2818 (Print)
eISSN: 1663-2826 (Online)

For additional information: http://www.karger.com/HRP


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