Peroxisomal dysfunction in chondrodysplasia punctata, rhizomelic type

Ophthalmic Paediatr Genet. 1987 Nov;8(3):183-5. doi: 10.3109/13816818709031467.

Abstract

The rhizomelic type of chondrodysplasia punctata (RCDP) is recognizable at birth because of the typical phenotype and radiological features. Most patients die young, some survive until their teens but all are severely retarded. Recent studies showed RCDP to be a peroxisomal disorder. Peroxisomal investigations may be important in defining the prognosis for an individual patient, and are definitely of use in antenatal diagnosis.

MeSH terms

  • Adolescent
  • Brain Diseases / physiopathology
  • Child
  • Child, Preschool
  • Chondrodysplasia Punctata / classification
  • Chondrodysplasia Punctata / pathology
  • Chondrodysplasia Punctata / physiopathology*
  • Femur / pathology*
  • Humans
  • Humerus / pathology*
  • Infant
  • Kidney Diseases / physiopathology
  • Liver Diseases / physiopathology
  • Microbodies / physiology*
  • Reference Values
  • Syndrome