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Bone Health Status of Children with Spinal Muscular Atrophy

Joanna Yuet-Ling Tung1, Tsz-Kit Chow2, Monique Wai3

  • 1Department of Paediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong SAR, China.

Journal of Bone Metabolism
|December 11, 2023
PubMed

Insights

Children with spinal muscular atrophy (SMA) often have low bone mineral density (BMD) and fractures. However, the current definition of osteoporosis may not fully capture the bone health risks in this population.

Area of Science:

  • Pediatric Neuromuscular Disorders
  • Bone Metabolism and Health
  • Rare Genetic Diseases

Background:

  • Spinal muscular atrophy (SMA) is a group of rare, inherited neuromuscular disorders.
  • Bone health is often a neglected issue in children with SMA.
  • This study evaluated bone health in Hong Kong children with SMA.

Purpose of the Study:

  • To assess the bone health status of children with SMA.
  • To evaluate fracture history, bone mineral density (BMD), and vitamin D levels.
  • To determine the prevalence of osteoporosis based on current definitions.

Main Methods:

  • Retrospective study of 32 children with SMA from two Hong Kong centers.
  • Assessed bone health via fracture history, BMD (dual-energy X-ray absorptiometry), and serum 25-hydroxy-vitamin D (25[OH]D).
  • Analyzed associations between SMA type, age, sex, ambulation, 25(OH)D, and bone health outcomes.

Main Results:

  • Low BMD was observed in 16 of 17 patients assessed.
  • Four patients had a history of long bone fractures and received bisphosphonates.
  • No significant associations were found between clinical factors and fracture history or BMD Z-scores. Only one patient met the 2019 ISCD pediatric osteoporosis definition.

Conclusions:

  • Children with SMA on disease-modifying treatments frequently exhibit low BMD and fracture history.
  • Osteoporosis, by the 2019 ISCD pediatric definition, was uncommon.
  • A specialized definition for osteoporosis may be necessary for this high-risk pediatric population.
Abstract

Keywords:
Bone densityChildMuscular atrophy, spinalOsteoporosis

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