Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Clinics
; 68(2): 147-152, 2013. ilus, tab
Article
en En
| LILACS
| ID: lil-668799
Biblioteca responsable:
BR1.1
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-∝-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-∝-fludrocortisone doses decreased over time during the first and second years of life; the median fludrocortisone doses were 200 µg at 0-6 months, 150 µg at 7-18 months and 125 µg at 19-24 months. The cortisone acetate dose per square meter was stable during follow-up (median = 16.8 mg/m²/day). The serum sodium, potassium and plasma rennin activity levels during treatment were normal, except in the first month of life, when periodic 9-∝-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.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
LILACS
Asunto principal:
Fludrocortisona
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Hiperplasia Suprarrenal Congénita
/
Antiinflamatorios
Tipo de estudio:
Guideline
/
Observational_studies
Límite:
Female
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Humans
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Infant
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Male
/
Newborn
Idioma:
En
Revista:
Clinics
Asunto de la revista:
MEDICINA
Año:
2013
Tipo del documento:
Article
/
Project document
País de afiliación:
Brasil
Pais de publicación:
Brasil