Management of Congenital Adrenal Hyperplasia: Results of the ESPE Questionnaire
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.
Integrity signals
Registry
Cites
Statistics
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
Increased cardiovascular mortality has been reported in CAH in Sweden, second only to adrenal crisis as a cause of death.
“Increased cardiovascular mortality has been reported in CAH in Sweden, second only to adrenal crisis as a cause of death (368).”
From Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management (2022) · cites this paper as [368]
Cites
Krone, N., Braun, A., Roscher, A. A., Knorr, D., & Schwarz, H. P. (2000) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.85.3.6441 · Cited by 397 · see citing works
Self-citationClinical review 54: genetics, diagnosis, and management of 21-hydroxylase deficiency
Miller, W. L. (1994) · Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.78.2.241 · Cited by 167 · see citing works
Saedi, S. A., Dean, H., Dent, W., Stockl, E., & Cronin, C. (1996) · PEDIATRICS · doi:10.1542/peds.97.1.100 · Cited by 55 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Speiser, P. W., Azziz, R., Baskin, L. S., Ghizzoni, L., Hensle, T. W., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Montori, V. M., Oberfield, S. E., et al. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-2631 · Cited by 1,484 · see citing works
Speiser, P. W., Arlt, W., Auchus, R. J., Baskin, L. S., Conway, G. S., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Murad, M. H., Oberfield, S. E., et al. (2018) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2018-01865 · Cited by 1,234 · see citing works
Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management
Claahsen - van der Grinten, H. L., Speiser, P. W., Ahmed, S. F., Arlt, W., Auchus, R. J., Falhammar, H., Flück, C. E., Guasti, L., Huebner, A., Kortmann, B. B. M., et al. (2022) · Endocrine Reviews · doi:10.1210/endrev/bnab016 · Cited by 556 · see citing works
Self-citationCatecholaminergic systems in stress: structural and molecular genetic approaches
Kvetnansky, R., Sabban, E. L., & Palkovits, M. (2009) · Physiological Reviews · doi:10.1152/physrev.00042.2006 · Cited by 548 · see citing works
Forest, M. G. (2004) · Human Reproduction Update · doi:10.1093/humupd/dmh047 · Cited by 200 · see citing works
WISNIEWSKI, A. B., MIGEON, C. J., MALOUF, M. A., & GEARHART, J. P. (2004) · Journal of Urology · doi:10.1097/01.ju.0000125269.91938.f7 · Cited by 144 · see citing works
Mazziotti, G., Formenti, A. M., Frara, S., Roca, E., Mortini, P., Berruti, A., & Giustina, A. (2017) · European Journal of Endocrinology · doi:10.1530/eje-17-0154 · Cited by 96 · see citing works
Congenital adrenal hyperplasia – pharmacologic interventions from the prenatal phase to adulthood
Claahsen-van der Grinten, H. L., Stikkelbroeck, N. M. M. L., Otten, B. J., & Hermus, A. R. M. M. (2011) · Pharmacology & Therapeutics · doi:10.1016/j.pharmthera.2011.05.004 · Cited by 53 · see citing works
Claahsen-van der Grinten, H. L., Noordam, K., Borm, G. F., & Otten, B. J. (2006) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2005-1701 · Cited by 42 · see citing works
Ali, S. R., Bryce, J., Haghpanahan, H., Lewsey, J. D., Tan, L. E., Atapattu, N., Birkebaek, N. H., Blankenstein, O., Neumann, U., Balsamo, A., et al. (2021) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/clinem/dgaa694 · Cited by 39 · see citing works
Self-citation
- Authors
- Riepe, F. G., Krone, N., Viemann, M., Partsch, C.-J., & Sippell, W. G.
- Affiliations
- Christian-Albrechts-Universität zu Kiel (DE)
- ORCID
- Krone, Nils
- Venue
- Hormone Research in Paediatrics
- Publisher
- S. Karger AG
- Published
- 2002
- Volume
- 58
- Issue
- 4
- Pages
- 196-205
- ISSN
- 1663-2818 (ISSN-L)
- 1663-2826
- OpenAlex
- W2047610013
- Cited by
- 72
- References
- 3
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 1 · 2022: 2 · 2021: 3 · 2020: 3 · 2019: 2 · 2018: 2 · 2017: 4 · 2016: 2 · 2015: 1 · 2014: 4 · 2013: 2 · 2012: 3
- FWCI
- 1.73
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Hormone Research in Paediatrics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Reproductive Medicine › Ovarian function and disorders
- Keywords
- congenital adrenal hyperplasia, cortisol, glucocorticoid dosing, mineralocorticoid therapy, prenatal dexamethasone, newborn screening, feminizing surgery, clitoridectomy, vaginoplasty, psychodiagnosis, salt wasting, plasma renin activity, medication adherence
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Endocrinology, Europe, Female, Fetal Diseases, Genitalia, Glucocorticoids, Humans, Infant, Newborn, Male, Mineralocorticoids, Pediatrics, Prenatal Diagnosis, Surveys and Questionnaires, Societies, Medical, Sodium, Neonatal Screening
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
