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Skeletal evolution in Marfan syndrome: growth curves from a French national cohort

Grégoire Benoist1, Florence Tubach1, Carine Roy1

  • 1Department of Pediatrics, Ambroise Paré Hospital, APHP, Boulogne-Billancourt, France.

Pediatric Research
|August 29, 2017
PubMed

Insights

Children with Marfan syndrome (MFS) are significantly taller than their peers, with growth patterns differing from unaffected children. These findings aid in counseling and disease detection for MFS patients.

Area of Science:

  • Genetics
  • Pediatrics
  • Endocrinology

Background:

  • Marfan syndrome (MFS) is a genetic disorder affecting connective tissue.
  • Growth patterns in children with MFS require detailed characterization for clinical management.
  • Understanding these patterns can aid in early detection of associated complications.

Purpose of the Study:

  • To describe and compare the growth patterns of children with Marfan syndrome (MFS) against unaffected controls.
  • To develop growth charts specific to children with MFS.
  • To identify potential differences in growth trajectories influenced by FBN1 gene mutations.

Main Methods:

  • An observational study involving children with confirmed FBN1 mutations (MFS group) and mutation-negative siblings (non-MFS group).
  • Comparison of anthropometric data (height, BMI) with French-accepted reference nomograms.
  • Statistical analysis to determine significant differences in growth parameters between groups.

Main Results:

  • MFS children exhibited significantly greater height than both non-MFS and reference groups across all ages.
  • Overgrowth in MFS patients decreased with age, though they remained taller at 17 years.
  • Mean BMI in MFS children was comparable to non-MFS peers and lower than the general French population.

Conclusions:

  • Growth patterns in children with FBN1 mutations significantly differ from unaffected individuals.
  • Established growth parameters and charts can improve patient counseling and early detection of MFS-related conditions.
  • These growth curves may assist in predicting final adult height for MFS patients.

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