Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia
Abstract
Background/aims Congenital adrenal hyperplasia (CAH) is characterized by adrenal insufficiency with or without salt wasting. It is also accompanied by adrenomedullary hypofunction. The aim of the present study was to investigate adrenomedullary function in patients with CAH due to 21-hydroxylase and 11beta-hydroxylase deficiencies and in age-matched normal subjects. Methods We measured plasma catecholamines (epinephrine and norepinephrine) and urine metanephrine in 44 patients with CAH, 32 due to 21-hydroxylase deficiency (17 patients with the salt-wasting form and 15 patients with the simple virilizing form), and 12 due to 11beta-hydroxylase deficiency, and in 25 healthy controls. Results Plasma epinephrine and urine metanephrine levels were significantly higher in the controls than in patients with CAH (p = 0.02 and p Conclusion Despite the fact that CAH patients have insufficient epinephrine secretion, these patients have the ability to increase compensatory norepinephrine. However, this increase is much lower in patients with the salt-wasting form. These findings need to be confirmed by other studies.
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A large CAH study has shown increased plasma norepinephrine compared with controls.
“A large CAH study has shown increased plasma norepinephrine compared with controls (3), consistent with increased production of norepinephrine from the sympathetic nervous system (SNS), as occurs in patients with acquired adrenal insufficiency (20).”
From Decreased adrenomedullary function in infants with classical congenital adrenal hyperplasia. (2014) · cites this paper as [3]
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Decreased adrenomedullary function in infants with classical congenital adrenal hyperplasia.
Kim, M. S., Ryabets-Lienhard, A., Bali, B., Lane, C. J., Park, A. H., Hall, S., & Geffner, M. E. (2014) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2014-1274 · Cited by 38 · see citing works
on behalf of the German Society of Pediatric Endocrinology and Diabetology, Dörr, H. G., Wollmann, H. A., Hauffa, B. P., & Woelfle, J. (2018) · BMC Endocrine Disorders · doi:10.1186/s12902-018-0263-1 · Cited by 22 · see citing works
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- Authors
- Tutunculer, F., Saka, N., Arkaya, S. C., Abbasoglu, S., & Bas, F.
- Affiliations
- Istanbul University (TR)
- ORCID
- Arkaya, Selda Can
- Venue
- Hormone Research in Paediatrics
- Publisher
- S. Karger AG
- Published
- 2009
- Volume
- 72
- Issue
- 6
- Pages
- 331-336
- ISSN
- 1663-2818 (ISSN-L)
- 1663-2826
- OpenAlex
- W2040401691
- Cited by
- 15
- References
- 9
- Citations by year
- 2025: 1 · 2024: 1 · 2022: 1 · 2021: 2 · 2020: 2 · 2019: 1 · 2018: 4 · 2016: 1 · 2014: 1
- FWCI
- 0.24
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Hormone Research in Paediatrics (publishedVersion)
- PubMed (publishedVersion)
- Trakya University's Institutional Open Access System (Trakya University) (submittedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- congenital adrenal hyperplasia, adrenomedullary function, 21-hydroxylase deficiency, 11beta-hydroxylase deficiency, salt-wasting form, simple virilizing form, plasma catecholamines, epinephrine, norepinephrine, urinary metanephrines
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Adrenal Medulla, Adult, Child, Epinephrine, Female, Humans, Male, Metanephrine, Norepinephrine, Phenotype, Sodium, Steroid 11-beta-Hydroxylase, Steroid 21-Hydroxylase, Case-Control Studies
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