[Prenatal treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. 9 treated pregnancies]

Ann Pediatr (Paris). 1993 Sep;40(7):421-5.
[Article in French]

Abstract

Prenatal treatment based on administration of dexamethasone to the mother during pregnancy was initiated early during nine pregnancies with a high risk of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. The purpose of this treatment was to prevent fetal virilization by reducing production of androgens by the adrenal glands. Prenatal diagnosis was achieved by comparing amniotic fluid cell HLA genotypes and more recently by subjecting trophoblasts to molecular genetic studies. Together with prenatal determination of fetal sex, this allowed to determine that only two female fetuses were affected. Efficacy of continued prenatal treatment in these two cases was good in one case and mediocre in the other. The treatment was well tolerated by the mothers and fetuses.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • Adrenal Hyperplasia, Congenital* / complications
  • Adrenal Hyperplasia, Congenital* / diagnosis
  • Adrenal Hyperplasia, Congenital* / drug therapy*
  • Adrenal Hyperplasia, Congenital* / epidemiology
  • Amniocentesis
  • Dexamethasone / pharmacology
  • Dexamethasone / therapeutic use*
  • Female
  • Fetal Diseases / etiology*
  • Fetal Diseases / prevention & control
  • Follow-Up Studies
  • Genotype
  • HLA Antigens / analysis
  • Histocompatibility Testing
  • Humans
  • Infant, Newborn
  • Male
  • Polymerase Chain Reaction
  • Polymorphism, Restriction Fragment Length
  • Pregnancy
  • Pregnancy Outcome
  • Prenatal Care / methods*
  • Risk Factors
  • Sex Determination Analysis
  • Treatment Outcome
  • Trophoblasts
  • Virilism / etiology*
  • Virilism / prevention & control

Substances

  • HLA Antigens
  • Dexamethasone