[Genetic screening to determine an etiologic diagnosis in children with mental retardation]

Rev Med Chil. 2008 Dec;136(12):1542-51. Epub 2009 Mar 23.
[Article in Spanish]

Abstract

Background: Mental retardation or intellectual disability affects 2% of the general population, but in 60% to 70% of cases the real cause of this retardation is not known. An early etiologic diagnosis of intellectual disability can lead to opportunities for improved educational interventions, reinforcing weak areas and providing a genetic counseling to the family.

Aim: To search genetic diseases underlying intellectual disabilities of children attending a special education school.

Material and methods: A clinical geneticist performed the history and physical examination in one hundred and three students aged between 5 and 24 years (51 males). A blood sample was obtained in 92 of them for a genetic screening that included a standard karyotype, fragile X molecular genetic testing and search for inborn errors of metabolism by tandem mass spectrometry.

Results: This approach yielded an etiological diagnosis in as much as 29 patients. Three percent of them had a fragile X syndrome. Inborn errors of metabolism were not detected.

Conclusions: This type of screening should be done always in children with intellectual disability to establish an etiological diagnosis.

Publication types

  • English Abstract

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cytogenetic Analysis / methods*
  • Education, Special
  • Female
  • Fragile X Syndrome / diagnosis
  • Fragile X Syndrome / genetics
  • Genetic Testing / methods*
  • Humans
  • Intellectual Disability / genetics*
  • Karyotyping
  • Male
  • Mutation / genetics*
  • Severity of Illness Index
  • Young Adult