A patient with monosomy 1p36, atypical features and phenotypic similarities with Cantu syndrome

Am J Med Genet A. 2005 Dec 15;139(3):216-20. doi: 10.1002/ajmg.a.31013.

Abstract

We report on a 16-year-old boy with a distal 1p36 deletion with some clinical features consistent with Cantu syndrome (OMIM#239850). He also has hypercholesterolemia, type II diabetes, recurrent bony fractures, and non-alcoholic steatohepatitis, not previously described in either condition. The 1p36 deletion was detected in a screen of all chromosome subtelomeres using multiplex ligation-dependent probe amplification and was verified using FISH with a region-specific BAC clone. We suggest that patients suspected of having Cantu syndrome, especially those with unusual or more severe manifestations be analyzed for distal 1p36 deletions.

Publication types

  • Case Reports

MeSH terms

  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / pathology*
  • Adolescent
  • Chromosome Disorders / genetics
  • Chromosome Disorders / pathology*
  • Chromosomes, Human, Pair 1 / genetics*
  • Femur / diagnostic imaging
  • Humans
  • In Situ Hybridization, Fluorescence
  • Male
  • Monosomy / genetics
  • Monosomy / pathology*
  • Phenotype*
  • Radiography, Thoracic
  • Skull / diagnostic imaging
  • Spine / diagnostic imaging