Management of adolescents with congenital adrenal hyperplasia
Open accessAbstract
The management of congenital adrenal hyperplasia involves suppression of adrenal androgen production, in addition to treatment of adrenal insufficiency. Management of adolescents with congenital adrenal hyperplasia is especially challenging because changes in the hormonal milieu during puberty can lead to inadequate suppression of adrenal androgens, psychosocial issues often affect adherence to medical therapy, and sexual function plays a major part in adolescence and young adulthood. For these reasons, treatment regimen reassessment is indicated during adolescence. Patients with non-classic congenital adrenal hyperplasia require reassessment regarding the need for glucocorticoid drug treatment. No clinical trials have compared various regimens for classic congenital adrenal hyperplasia in adults, thus therapy is individualised and based on the prevention of adverse outcomes. Extensive patient education is key during transition from paediatric care to adult care and should include education of females with classic congenital adrenal hyperplasia regarding their genital anatomy and surgical history. Common issues for these patients include urinary incontinence, vaginal stenosis, clitoral pain, and cosmetic concerns; for males with classic congenital adrenal hyperplasia, common issues include testicular adrenal rest tumours. Transition from paediatric to adult care is most successful when phased over many years. Education of health-care providers on how to successfully transition patients is greatly needed.
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Patients with classic 21OH deficiency also have epinephrine deficiency, due to abnormal adrenomedullary formation; this places patients at risk for hypoglycemia, especially when fasting, or during acute illness.
“Patients with classic 21OH deficiency also have epinephrine deficiency, due to abnormal adrenomedullary formation; 73 this places patients at risk for hypoglycemia, especially when fasting, or during acute illness.”
From Congenital adrenal hyperplasia (2017) · cites this paper as [73]
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Hodentumoren bei präpubertären Jungen – Organerhalt häufiger möglich als gedacht
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- Authors
- Merke, D. P., & Poppas, D. P.
- Affiliations
- National Institutes of Health (US)
- NewYork–Presbyterian Hospital (US)
- Corresponding author
- Merke, Deborah P
- ORCID
- Merke, Deborah P
- Venue
- The Lancet Diabetes & Endocrinology
- Publisher
- Elsevier BV
- Published
- 2013-12
- Volume
- 1
- Issue
- 4
- Pages
- 341-352
- ISSN
- 2213-8587 (ISSN-L)
- PMC
- PMC4163910
- OpenAlex
- W2149437278
- Cited by
- 134
- References
- 108
- Citations by year
- 2026: 6 · 2025: 6 · 2024: 12 · 2023: 11 · 2022: 30 · 2021: 13 · 2020: 6 · 2019: 11 · 2018: 12 · 2017: 11 · 2016: 8 · 2015: 6 · 2012: 1
- FWCI
- 0.44
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- The Lancet Diabetes & Endocrinology (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health (Z99CL999999)
- 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, adolescents, puberty, androgen deprivation therapy, glucocorticoid therapy, non-classical congenital adrenal hyperplasia, adrenal insufficiency, medication adherence, sexual function, transition to adult care, patient education, urinary incontinence, vaginal stenosis, clitoral pain, personalized treatment
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Glucocorticoids, Humans, Disease Management
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