Molecular characterisation of chromosome 4p deletions resulting in Wolf-Hirschhorn syndrome

J Med Genet. 1994 Feb;31(2):103-7. doi: 10.1136/jmg.31.2.103.

Abstract

We present three patients with Wolf-Hirschhorn syndrome with small cytogenetic deletions of 4p16. One case is a de novo translocation and two cases represent de novo deletions. Using molecular techniques we determined the extent of these deletions and attempted to ascertain parental origin. Case 1 had a deletion of 4p16.3 with a breakpoint proximal to D4S10, case 2 had a larger deletion including D4S62 in 4p16.2, and case 3 had the largest deletion which included D4S240, but not the Raf2 locus in 4p16.1. The parental origin of the deletion in case 3 was paternal; the other two cases were indeterminable. Our results show that these three deletions include the currently proposed Wolf-Hirschhorn syndrome critical region within the most distal 2 Mb of 4p16.3 and offer supportive evidence for continuous terminal deletions.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / genetics*
  • Brain / abnormalities
  • Chromosome Deletion*
  • Chromosome Mapping
  • Chromosomes, Human, Pair 4*
  • Female
  • Growth Disorders / genetics
  • Head / abnormalities
  • Humans
  • Infant, Newborn
  • Intellectual Disability / genetics
  • Male
  • Parents
  • Syndrome
  • Translocation, Genetic*