Evolving phenotype of Marfan's syndrome

Arch Dis Child. 1997 Jan;76(1):41-6. doi: 10.1136/adc.76.1.41.

Abstract

Aim: To examine evolution of the physical characteristics of Marfan's syndrome throughout childhood.

Methods: 40 children were ascertained during the development of a regional register for Marfan's syndrome. Evolution of the clinical characteristics was determined by repeat evaluation of 10 patients with sporadic Marfan's syndrome and 30 with a family history of the condition. DNA marker studies were used to facilitate diagnosis in those with the familial condition.

Results: Musculoskeletal features predominated and evolved throughout childhood. Gene tracking enabled early diagnosis in children with familial Marfan's syndrome.

Conclusions: These observations may aid the clinical diagnosis of Marfan's syndrome in childhood, especially in those with the sporadic condition. Gene tracking has a role in the early diagnosis of familial Marfan's syndrome, allowing appropriate follow up and preventive care.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Age of Onset
  • Body Height
  • Body Weight
  • Cardiovascular Diseases
  • Child
  • Child, Preschool
  • Eye Diseases / etiology
  • Face / abnormalities
  • Female
  • Fingers / abnormalities
  • Flatfoot
  • Genetic Markers
  • Humans
  • Infant
  • Joint Instability
  • Male
  • Marfan Syndrome / genetics
  • Marfan Syndrome / pathology*
  • Marfan Syndrome / physiopathology
  • Palate / abnormalities
  • Pedigree
  • Scoliosis
  • Thorax / abnormalities

Substances

  • Genetic Markers