Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency

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dc.contributor Sistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.author GOMES, Larissa G. FMUSP-HC
MADUREIRA, Guiomar FMUSP-HC
MENDONCA, Berenice B. FMUSP-HC
BACHEGA, Tania A. S. S. FMUSP-HC
dc.date.issued 2013
dc.identifier.citation CLINICS, v.68, n.2, p.147-151, 2013
dc.identifier.issn 1807-5932
dc.identifier.uri http://observatorio.fm.usp.br/handle/OPI/1788
dc.description.abstract OBJECTIVE: The protocols for glucocorticoid replacement in children with salt wasting 21-hydroxylase deficiency are well established; however, the current recommendation for mineralocorticoid replacement is general and suggests individualized dose adjustments. This study aims to retrospectively review the 9-/fludrocortisone dose regimen in salt wasting 21-hydroxylase deficient children who have been adequately treated during infancy. METHODS: Twenty-three salt wasting 21-hydroxylase deficient patients with good anthropometric and hormonal control were followed in our center since diagnosis. The assessments of cortisone acetate and 9-alpha-fludrocortisone doses, anthropometric parameters, and biochemical and hormonal levels were rigorously evaluated in pre-determined intervals from diagnosis to two years of age. RESULTS: The 9-alpha-fludrocortisone doses decreased over time during the first and second years of life; the median fludrocortisone doses were 200 mg at 0-6 months, 150 mu g at 7-18 months and 125 mu g at 19-24 months. The cortisone acetate dose per square meter was stable during follow-up (median = 16.8 mg/m(2)/day). The serum sodium, potassium and plasma rennin activity levels during treatment were normal, except in the first month of life, when periodic 9-alpha-fludrocortisone dose adjustments were made. CONCLUSIONS: The mineralocorticoid needs of salt wasting 21-hydroxylase deficient patients are greater during early infancy and progressively decrease during the first two years of life, which confirms that a partial aldosterone resistance exists during this time. Our study proposes a safety regiment for mineralocorticoid replacement during this critical developmental period.
dc.description.sponsorship · FAPESP [2008/57616-5]
· CNPq [305117/2009-2, 305743/2011-2]
dc.language.iso eng
dc.publisher HOSPITAL CLINICAS, UNIV SAO PAULO
dc.relation.ispartof Clinics
dc.rights openAccess
dc.subject Salt Wasting Form; 21-hydroxylase Deficiency; Mineralocorticoid Replacement
dc.subject.other partial aldosterone resistance; sodium homeostasis; brazilian patients; management; receptor; height; term
dc.title Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency
dc.type article
dc.rights.holder Copyright HOSPITAL CLINICAS, UNIV SAO PAULO
dc.description.group LIM/42
dc.identifier.doi 10.6061/clinics/2013(02)OA05
dc.identifier.pmid 23525308
dc.type.category original article
dc.type.version publishedVersion
hcfmusp.author GOMES, Larissa G.:HC:ICHC
hcfmusp.author MADUREIRA, Guiomar:HC:ICHC
hcfmusp.author MENDONCA, Berenice B.:FM:MCM
hcfmusp.author BACHEGA, Tania A. S. S.:FM:MCM
hcfmusp.origem.id 2-s2.0-84875667066
hcfmusp.origem.id WOS:000316764000005
hcfmusp.origem.id SCIELO:S1807-59322013000200005
hcfmusp.publisher.city SAO PAULO
hcfmusp.publisher.country BRAZIL
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dc.description.index MEDLINE
hcfmusp.citation.scopus 3
hcfmusp.citation.wos 2


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