Treatment of congenital adrenal hyperplasia in children aged 0-3 years: a retrospective multicenter analysis of salt supplementation, glucocorticoid and mineralocorticoid medication, growth and blood pressure

Eur J Endocrinol. 2022 Apr 11;186(5):587-596. doi: 10.1530/EJE-21-1085.

Abstract

Objectives: International guidelines recommend additional salt supplementation during infancy in classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. The influence of corticoid medication and growth has not been assessed.

Aim: To investigate the current use of salt supplementation, fludrocortisone (FC) and hydrocortisone (HC) dosage as well as weight, height, BMI and blood pressure (BP) in CAH children aged 0-3 years.

Methods: Retrospective multicentre analysis using data from the I-CAH registry. Salt-treated (ST) and non-salt-treated (NST) children were compared regarding FC and HC dosage, weight, height and BP at 0, 3, 6, 9, 12, 18, 24, 30, and 36 months.

Results: We analysed 2483 visits of 331 patients born after year 2000 in 13 countries (male, n = 145) with 203 ST patients (61%). NST children had significantly higher FC dosages at 1.5-4.5 months and higher HC dosages until 1.5 months of age. No differences in weight, length and BP between subgroups were observed. Children of the whole cohort showed increased BMI-SDS during the study period and about half of the reported BP readings were >P95.

Conclusion: In children treated with additional salt supplementation, FC and HC dosages are lower during the first months of life but without differences in weight, length and BP until 3 years of age compared to NST children. All children showed an increase in BMI-SDS and a high rate of BP readings >P95 until 3 years, indicating the start of weight gain and negative effects on blood pressure already in very early life.

Publication types

  • Multicenter Study

MeSH terms

  • Adrenal Hyperplasia, Congenital* / drug therapy
  • Blood Pressure
  • Child
  • Child, Preschool
  • Dietary Supplements
  • Fludrocortisone / therapeutic use
  • Glucocorticoids* / therapeutic use
  • Humans
  • Hydrocortisone / therapeutic use
  • Male
  • Mineralocorticoids / therapeutic use
  • Retrospective Studies
  • Sodium Chloride, Dietary / therapeutic use

Substances

  • Glucocorticoids
  • Mineralocorticoids
  • Sodium Chloride, Dietary
  • Fludrocortisone
  • Hydrocortisone