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Severe perinatal Marfan syndrome.

D M Gross1, L K Robinson, L T Smith

  • 1University of Texas Medical School, Houston 77225.

Pediatrics
|July 1, 1989
PubMed
Summary

Severe perinatal Marfan syndrome presents significant cardiovascular challenges, often leading to limited life expectancy in infants. Early diagnosis and management are crucial for affected children.

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Area of Science:

  • Medical Genetics
  • Cardiology
  • Pediatrics

Background:

  • Marfan syndrome is a genetic disorder affecting connective tissue.
  • Cardiovascular complications are well-documented in older individuals with Marfan syndrome.
  • Severe perinatal Marfan syndrome requires specific clinical attention.

Observation:

  • Presents clinical, radiographic, and echocardiographic data from three perinatal Marfan syndrome patients.
  • Two patients diagnosed at birth died in infancy.
  • One patient diagnosed at six months survived to three years.

Findings:

  • Severe perinatal Marfan syndrome is associated with significant cardiorespiratory issues.
  • Early-onset severe symptoms correlate with a poorer prognosis and limited life expectancy.
  • Explores potential links between Marfan syndrome, Ehlers-Danlos syndrome, and osteogenesis imperfecta.

Implications:

  • Highlights the critical need for early diagnosis and intervention in severe perinatal Marfan syndrome.
  • Suggests that early cardiovascular and respiratory support may influence long-term outcomes.
  • Underscores the importance of distinguishing and understanding overlapping connective tissue disorders.

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