Growth in infants with congenital adrenal hyperplasia due to 21-hydroxylase deficiency an analysis of the factors affecting height

الملخص EN

Background: It has been suggested that overtreatment of infants with congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency might affect their growth and final height.

Objective: To assess the axiological parameters of infants with CAH due to 21-hydroxylase deficiency from their first presentation in neonatal period and during infancy, in an attempt to decide the optimum doses of glucocorticoid, mineralocorticoid and salt therapy that can achieve androgen suppression, prevent life-threatening salt-wasting crises, and allow normal growth velocity for age.

Materials and methods: Retrospective study conducted in the Diabetes Endocrine and Metabolism Pediatric Unit at Children’s Hospital, Cairo University included baseline and follow-up data of 28 infants with 21-hydroxylase deficiency.

Results: Median age at presentation was 34 days.

Lower doses of hydrocortisone (<20 mg/m2/ day) were associated with better growth, not reaching a statistical significance.

Delta change in length standard deviation score (SDS) was significantly higher in males (p= 0.01).

Delta change in length and weight SDS of cases with mean basal length SDS below 2 were significantly higher than those with mean basal length SDS above 2 (p= 0.02 and 0.04 respectively).

No correlations were found between delta height-SDS and age at diagnosis, duration of follow-up, glucocrticoid or mineralocorticoid dosing.

Conclusion: Poor growth in infants with salt wasting CAH was not related to age at diagnosis and start of therapy, doses of glucocorticoid, fludrocortisones, or saline therapy.

Male sex and mean basal length SDS below 2 were associated with a more favorable growth catch up.

Further prospective studies with inclusion of intercurrent illnesses and nutritional status are needed.