[The Cri du Chat syndrome: a study on the quality of care]

Minerva Pediatr. 2012 Aug;64(4):395-400.
[Article in Italian]

Abstract

Aim: The Cri du Chat syndrome (SCdC / [OMIM #123450]) is a rare disease characterized by the deletion of the short arm of chromosome 5. The typical clinical features are the cat-like cry, microcephaly, a distinct facial phenotype and a severe psychomotor and mental retardation. The aim of this study was to provide an analysis on the data concerning the life quality and families assistance to whom have a child affected by Cri du Chat syndrome such as: the life's change of parents after the child's birth, the frequency of treatments, the collaboration between family and center of reference.

Methods: We have also analyzed the improvement obtained, the type of school attended, the aide and the time spent at home in postprimary education. Through a questionnaire sent to 100 families, we have been picked up information on 76 patients.

Results: These families have to adapt to the reality of a child with a rare genetic disorder for which there are no pharmacological or surgical therapies.

Conclusion: Therefore, it seems important to give information and tips for dealing with the disease and the early start of rehabilitation and educational therapy.

Publication types

  • English Abstract

MeSH terms

  • Adolescent
  • Cri-du-Chat Syndrome / diagnosis
  • Cri-du-Chat Syndrome / epidemiology
  • Cri-du-Chat Syndrome / genetics*
  • Cri-du-Chat Syndrome / rehabilitation*
  • Humans
  • Infant
  • Intellectual Disability / genetics
  • Italy / epidemiology
  • Microcephaly / genetics
  • Quality of Health Care*
  • Quality of Life*
  • Surveys and Questionnaires
  • Young Adult

Associated data

  • OMIM/123450