[Genetic of the 21 hydroxylase deficiency]

Ann Endocrinol (Paris). 1982;43(1):3-14.
[Article in French]

Abstract

A dose genetic linkage exist between the HLA complex (especially HLA-B), and the 21 hydroxylase deficiency form of adrenal hyperplasia. By their polymorphisms HLA antigens can be used as "markers" to follow the segregation of 21-OH deficiency in families, to diagnose the heterozygous offspring and eventually to offer a prenatal diagnosis to couples at risk. In late onset forms of 21-OH deficiency the same genetic linkage has been demonstrated with a high frequency of HLA-B14 antigen.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Hyperplasia, Congenital* / diagnosis
  • Adrenal Hyperplasia, Congenital* / genetics*
  • Adrenal Hyperplasia, Congenital* / immunology
  • Female
  • Genetic Carrier Screening
  • Genetic Linkage*
  • HLA Antigens / genetics*
  • Humans
  • Male
  • Pedigree
  • Polymorphism, Genetic
  • Pregnancy
  • Prenatal Diagnosis
  • Steroid 21-Hydroxylase / genetics
  • Steroid Hydroxylases / deficiency*

Substances

  • HLA Antigens
  • Steroid Hydroxylases
  • Steroid 21-Hydroxylase