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Bone mineral density in developing children with osteogenesis imperfecta: a longitudinal study with 9 years of

Dieke H J Kok1, Ralph J B Sakkers, Hans E H Pruijs

  • 1Department of Orthopaedic Surgery, Wilhelmina Children's Hospital, University Medical Center Utrecht, the Netherlands. diekekok@tele2.nl

Acta Orthopaedica
|September 3, 2013
PubMed

Insights

Bone mineral density (BMD) increased in children with osteogenesis imperfecta (OI) over 9 years, particularly in girls. This suggests potential differences in growth spurts and peak bone mass timing between sexes in OI patients.

Area of Science:

  • Pediatric Endocrinology
  • Orthopedics
  • Genetics

Background:

  • Osteogenesis imperfecta (OI) is a genetic disorder affecting collagen type I synthesis, leading to bone fragility, low bone mass, and skeletal deformities in children.
  • These bone abnormalities can result in varying degrees of short stature and impact overall skeletal development.

Purpose of the Study:

  • To investigate the longitudinal development of bone mineral density (BMD) in a cohort of children diagnosed with osteogenesis imperfecta.
  • To identify factors influencing BMD changes over time in pediatric patients with OI.

Main Methods:

  • Retrospective analysis of lumbar bone mineral density (BMD) in 74 children with OI, with a mean age of 16.3 years.
  • Longitudinal data analysis was performed on 52 children with repeated BMD measurements over a mean follow-up of 9 years.
  • Cross-sectional analysis of the latest BMD measurements was conducted for the entire cohort.

Main Results:

  • A mean annual increase in BMD of 0.038 g/cm²/year was observed in the longitudinal cohort.
  • BMD increase was significantly higher in girls compared to boys, both in unadjusted and adjusted analyses.
  • In cross-sectional analysis, higher latest-measured lumbar BMD was associated with being female, having OI type I, being ambulatory (walker), and older age.

Conclusions:

  • Children with OI experienced an increase in bone mineral density over a 9-year follow-up period.
  • The increase in BMD was more pronounced in girls, potentially due to later growth spurts and peak bone mass attainment in boys.
  • Findings highlight sex-based differences in bone density development in pediatric osteogenesis imperfecta.
Abstract

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