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Cardiovascular health, growth and gonadal function in children and adolescents with congenital adrenal hyperplasia.

Authors :
Mooij CF
Webb EA
Claahsen van der Grinten HL
Krone N
Source :
Archives of disease in childhood [Arch Dis Child] 2017 Jun; Vol. 102 (6), pp. 578-584. Date of Electronic Publication: 2016 Dec 14.
Publication Year :
2017

Abstract

After the introduction of replacement therapy with glucocorticoids and mineralocorticoids in the 1950s, congenital adrenal hyperplasia (CAH) is no longer a life-limiting condition. However, due to the successful introduction of medical steroid hormone replacement, CAH has become a chronic condition, with associated comorbidities and long-term health implications. The aim of treatment is the replacement of mineralocorticoids and glucocorticoids and the normalisation of elevated androgen concentrations. Long-term consequences of the condition and current treatment regimens include unfavourable changes in the cardiovascular risk profile, impaired growth, testicular adrenal rest tumours (TART) in male and subfertility in both male and female patients with CAH. Optimising replacement therapy in patients with CAH remains challenging. On one hand, treatment with supraphysiological doses of glucocorticoids might be required to normalise androgen concentrations and decrease size or presence of TARTs. On the other hand, treatment with supraphysiological doses of glucocorticoids is associated with an increased prevalence of unfavourable cardiovascular and metabolic risk profiles as well as impaired longitudinal growth and gonadal function. Therefore, treatment of children and adults with CAH requires an individualised approach. Careful monitoring for early signs of complications is already warranted during paediatric healthcare provision to prevent and reduce the impact of comorbidities in later life.<br />Competing Interests: Competing interests: None declared.<br /> (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Details

Language :
English
ISSN :
1468-2044
Volume :
102
Issue :
6
Database :
MEDLINE
Journal :
Archives of disease in childhood
Publication Type :
Academic Journal
Accession number :
27974295
Full Text :
https://doi.org/10.1136/archdischild-2016-311910