Cryptic trisomy 5q35.2qter and deletion 1p36.3 characterised using FISH and array-based CGH

Eur J Med Genet. 2008 Jul-Aug;51(4):343-50. doi: 10.1016/j.ejmg.2008.03.002. Epub 2008 Mar 27.

Abstract

A 10(6/12)-year-old boy was referred to the genetics department because of mental retardation and dysmorphic findings including microcephaly, flat face, down-slanting palpebral fissures, strabismus, prominent ears, bulbous nasal tip, down-turned corners of the mouth, narrow palate, clinodactyly of the fifth fingers and generalised eczema. Cytogenetic analysis revealed a karyotype of 47,XY,+mar of paternal origin. Multicolour FISH showed the marker chromosome to be derived from chromosome 15. For further elucidation of the phenotype, array-based comparative genomic hybridisation (aCGH) was performed, which revealed dup(5)(q35.2qter) and del(1)(p36.3). Parental FISH analysis revealed that the translocation occurred de novo. Despite the presence of a clinical phenotype along with a microscopically visible chromosomal aberration, a complex cryptic cytogenetic abnormality was causative for the phenotype of the patient. Elucidation of this complex aberration required combination of the whole cytogenetic toolbox.

Publication types

  • Case Reports
  • Comparative Study

MeSH terms

  • Adult
  • Child
  • Child, Preschool
  • Chromosome Deletion*
  • Chromosomes, Human, Pair 1 / genetics*
  • Chromosomes, Human, Pair 5 / genetics*
  • Developmental Disabilities / genetics
  • Developmental Disabilities / pathology
  • Genome, Human
  • Humans
  • In Situ Hybridization, Fluorescence*
  • Male
  • Oligonucleotide Array Sequence Analysis
  • Trisomy / genetics*