Neonatal screening for congenital adrenal hyperplasia: transitory elevation of 17-hydroxyprogesterone

J Pediatr Endocrinol Metab. 2011;24(3-4):155-62.

Abstract

Aim: The aim of the study was to identify patients with transitory elevation (TE) of 17-hydroxyprogesterone (17-OHP) using neonatal screening for congenital adrenal hyperplasia due to 21-hydroxylase deficiency (21-OHD) and to compare them with patients with 21-OHD.

Methods: This was a retrospective study of patients with high 17-OHP levels detected during newborn screening in Madrid, Spain.

Results: 17-OHP levels were significantly higher in the 33 21-OHD patients, who tended to present hyponatraemia and hyperkalemia. The TE-17-OHP group was characterized by normal initial physical examination (88.8% vs. 39.4%), lower gestational age and a higher number of stressful perinatal factors. 17-OHP levels decreased spontaneously in this group. Molecular diagnosis allowed us to discard the most frequent mutations associated with 21-OHD.

Conclusions: Newborns with slightly increased 17-OHP levels and normal results for physical examination, acid-base equilibrium, glycemia, electrolytes and perinatal stress factors should be carefully evaluated. Decisions on treatment should be postponed until these results are available.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 17-alpha-Hydroxyprogesterone / blood*
  • Adrenal Hyperplasia, Congenital / blood
  • Adrenal Hyperplasia, Congenital / diagnosis*
  • Clinical Chemistry Tests
  • DNA Mutational Analysis
  • Female
  • Humans
  • Infant, Newborn
  • Male
  • Mutation
  • Neonatal Screening / methods*
  • Retrospective Studies
  • Steroid 21-Hydroxylase / blood
  • Steroid 21-Hydroxylase / genetics

Substances

  • 17-alpha-Hydroxyprogesterone
  • CYP21A2 protein, human
  • Steroid 21-Hydroxylase