Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Clinics
;
68(2): 147-152, 2013. ilus, tab
Article
Dans Anglais
| LILACS
| ID: lil-668799
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.
Texte intégral:
Disponible
Indice:
LILAS (Amériques)
Sujet Principal:
Fludrocortisone
/
Hyperplasie congénitale des surrénales
/
Anti-inflammatoires
Type d'étude:
Guide de pratique
/
Étude observationnelle
Limites du sujet:
Femelle
/
Humains
/
Bébé
/
Mâle
/
Nouveau-né
langue:
Anglais
Texte intégral:
Clinics
Thème du journal:
Médicament
Année:
2013
Type:
Article
/
descriptif de projet
Pays d'affiliation:
Brésil
Institution/Pays d'affiliation:
Universidade de São Paulo/BR
Documents relatifs à ce sujet
MEDLINE
...
LILACS
LIS