[Vitamin D1alpha-hydroxylase deficiency as the cause of severe rickets in a 1-year-old-old boy]

Ugeskr Laeger. 2006 Feb 13;168(7):700-2.
[Article in Danish]

Abstract

We describe a 15-month-old boy referred to the hospital because of delayed development of motor skills and growth retardation. Blood samples and X-rays of the wrists and knees revealed rickets. He was treated with oral calcium and vitamin D with modest clinical and biochemical effect. 1,25-dihydroxyvitamin D was undetectable in laboratory tests. Vitamin D1alpha-hydroxylase deficiency was suspected and confirmed by DNA analysis, which revealed a 7 bp duplication in exon 8 of the CYP27B1 gene. The treatment was changed to an activated formula of vitamin D, alphacalcidol, whereupon the clinical and biochemical symptoms rapidly improved.

Publication types

  • Case Reports

MeSH terms

  • 25-Hydroxyvitamin D3 1-alpha-Hydroxylase / deficiency*
  • 25-Hydroxyvitamin D3 1-alpha-Hydroxylase / genetics
  • Calcium / administration & dosage
  • Growth Disorders / diagnostic imaging
  • Growth Disorders / drug therapy
  • Growth Disorders / enzymology
  • Growth Disorders / etiology
  • Hand / diagnostic imaging
  • Humans
  • Hydroxycholecalciferols / administration & dosage
  • Infant
  • Male
  • Motor Skills
  • Radiography
  • Rickets / diagnostic imaging
  • Rickets / drug therapy
  • Rickets / enzymology*
  • Rickets / etiology
  • Steroid Hydroxylases / deficiency*

Substances

  • Hydroxycholecalciferols
  • Steroid Hydroxylases
  • vitamin D 1-alpha hydroxylase
  • 25-Hydroxyvitamin D3 1-alpha-Hydroxylase
  • Calcium
  • alfacalcidol