title: Mineralocorticoid replacement during infancy for salt wasting congenital adrenal hyperplasia due to 21-hydroxylase deficiency.
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-α-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-α-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-α-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-α-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.
authors.affiliation: Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brazil.
authors.forename: Larissa G
authors.initials: LG
authors.lastname: Gomes
authors.forename: Guiomar
authors.initials: G
authors.lastname: Madureira
authors.forename: Berenice B
authors.initials: BB
authors.lastname: Mendonca
authors.forename: Tania A S S
authors.initials: TA
authors.lastname: Bachega
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000235:genetics; D000367:Age Factors; D000886:Anthropometry; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage*; D003348:Cortisone / Q000008:administration & dosage / Q000031:analogs & derivatives; D005260:Female; D005438:Fludrocortisone / Q000008:administration & dosage*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012189:Retrospective Studies; D018709:Statistics, Nonparametric; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D005438:Fludrocortisone; D003348:Cortisone