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Steroid 21-hydroxylase (EC (21-OHase) (Cytochrome P-450c21) (Cytochrome P450 21) (Cytochrome P450 XXI) (Cytochrome P450-C21) (Cytochrome P450-C21B) [CYP21] [CYP21B]


Transient hyper-17-OHPnemia unrelated to cross-reactions with residual fetal adrenal cortex products.

To clarify the pathogenesis of transient hyper-17alpha-hydroxyprogesteronemia, we initiated a laboratory investigation in a pre-term infant with persistently high serum 17alpha-hydroxyprogesterone (17-OHP) until 2 months of age. Serum 17-OHP level was measured by high-performance liquid chromatography and radioimmunoassay, and gene analysis of CYP21A2 (21-hydroxylase) was performed. Serum 17-OHP level on the 29th day of life was 25.4 ng/ml, and the urinary steroid profile showed low pregnanetriolone. Gene analysis of 21-hydroxylase disclosed no mutation, and 17-OHP normalized by 3 months of age without specific treatment. Transient elevations in 17-OHP, which do not appear related to cross-reactions with products of a residual fetal adrenal cortex, may occur in the first few months of life.

MeSH Terms

  • 17-alpha-Hydroxyprogesterone
  • Adrenal Cortex
  • Adrenocorticotropic Hormone
  • Aging
  • Chromatography, High Pressure Liquid
  • Cross Reactions
  • Fetus
  • Humans
  • Infant, Newborn
  • Male
  • Polymerase Chain Reaction
  • Pregnanetriol
  • Radioimmunoassay
  • Steroid 21-Hydroxylase
  • Time Factors