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Lookup NU author(s): Dr Matthew Thomas, Professor Timothy Cheetham
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© 2018 Walter de Gruyter GmbH, Berlin/Boston. The dose of hydrocortisone therapy required to maintain normal growth in infants with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is lower than in later childhood. This reflects the presence of excess non-aromatisable rather than aromatisable androgen but there has been relatively little focus on the role of mineralocorticoid therapy. Growth data of infants with CAH due to 21-hydroxylase deficiency (2008-2016) were reviewed and information regarding hydrocortisone and fludrocortisone regimen was collected. Change in height standard deviation (SD) and height velocity standard deviation score (SDS) were analysed during the first year of life. Growth data from 13 children (8 M) were analysed. Height (length) declined from a median of -0.69 SD at 3 months to -1.23 SD at 12 months with a reduction in height velocity SDS from 0.02 between 3 and 6 months to -2.22 between 9 and 12 months (p=0.017) despite a hydrocortisone dose at the lower end of the range as recommended in consensus guidelines. The glucocorticoid activity of hydrocortisone and fludrocortisone was negatively associated with growth velocity (r=-0.55; p=0.049) although renin activity was not suppressed. Infants with 21-hydroxylase deficiency can be managed with replacement hydrocortisone. The reasons for this paradigm are now understood although our data confirm that the glucocorticoid activity of fludrocortisone needs to be taken into consideration as well.
Author(s): Sellick J, Aldridge S, Thomas M, Cheetham T
Publication type: Article
Publication status: Published
Journal: Journal of Pediatric Endocrinology and Metabolism
Year: 2018
Volume: 31
Issue: 9
Pages: 1019-1022
Online publication date: 03/09/2018
Acceptance date: 02/08/2018
ISSN (print): 0334-018X
ISSN (electronic): 2191-0251
Publisher: De Gruyter
URL: https://doi.org/10.1515/jpem-2018-0260
DOI: 10.1515/jpem-2018-0260
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