PMID: 7015786Mar 1, 1981Paper

Reinstitution of mineralocorticoid therapy in congenital adrenal hyperplasia. Effects on control and growth

Acta paediatrica Scandinavica
M JansenJ L van den Brande

Abstract

The results of reintroduction of mineralocorticoid substitution therapy (9-alpha-fluorohydrocortisone) in five children with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency are described. All children had shown a salt-losing syndrome in infancy, but were off mineralocorticoids during several years; at the start of this study they all had elevated plasma renin activity (PRA). Four of them had increased and fluctuating pregnanetriol excretion during the year preceding reintroduction of mineralocorticoids, indicating poor control despite substantial substitution with hydrocortisone: 26 +/- 1.9 mg/m2/day (mean +/- S.E.M.). Reintroduction of mineralocorticoid therapy at ages 5.0-9.4 years resulted in a marked improvement of control, significant reduction in hydrocortisone requirements (to 17.6 +/- 1.4 mg/m2/day) and improvement in linear growth. The data suggest that, in all children with CAH and elevated PRA, continuation throughout childhood of mineralocorticoid therapy in addition to glucocorticoid therapy is necessary for optimal control and linear growth.

References

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Citations

Dec 27, 1990·The New England Journal of Medicine·G B Cutler, L Laue
Jan 1, 1985·Annals of the New York Academy of Sciences·L S Levine
Jan 1, 2009·Expert Review of Endocrinology & Metabolism·Karen Lin-SuMaria I New
Nov 26, 1998·Journal of Pediatric Endocrinology & Metabolism : JPEM·L A LopesP C Sizonenko
Jan 1, 1985·Annals of the New York Academy of Sciences·J S Winter, R M Couch
Jun 22, 2005·Journal of Endocrinological Investigation·C M R GermanoL L K Elias
Sep 22, 2007·Reviews in Endocrine & Metabolic Disorders·Felix G Riepe, Wolfgang G Sippell
Aug 21, 2021·The Journal of Clinical Endocrinology and Metabolism·Busra Gurpinar TosunTulay Guran
Dec 1, 2009·Endocrinology and Metabolism Clinics of North America·Saroj NimkarnMaria I New

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