Effect of intravenous pamidronate therapy on functional abilities and level of ambulation in children with osteogenesis imperfecta

J Pediatr. 2006 Apr;148(4):456-60. doi: 10.1016/j.jpeds.2005.10.041.

Abstract

Objective: To evaluate the functional abilities and the level of ambulation during pamidronate therapy in children with moderate to severe osteogenesis imperfecta.

Study design: Functional abilities, ambulation, and grip force were assessed in 59 patients (mean age, 6.1 years; range, 0.5-15.7 years; 30 girls) during 3 years of pamidronate treatment. Functional skills (mobility and self-care) were both assessed by using the Pediatric Evaluation of Disability Inventory. Ambulation level was assessed by using the modified Bleck score. For 48 patients, results after 3 years of pamidronate treatment could be matched to those of patients with similar age and disease severity who had not received pamidronate.

Results: Mobility and self-care scores increased during the study period (+43% and +30%, respectively). The average ambulation score changed from 0.8 to 1.9. Maximal isometric grip force increased by 63%. Mobility and ambulation scores and grip force measures were significantly higher than in patients who had not received pamidronate. The difference in self-care scores did not reach significance.

Conclusion: This study suggests that cyclical pamidronate treatment improves mobility, ambulation level, and muscle force in children with moderate to severe osteogenesis imperfecta.

Publication types

  • Controlled Clinical Trial
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Activities of Daily Living*
  • Adolescent
  • Analysis of Variance
  • Bone Density Conservation Agents / administration & dosage
  • Bone Density Conservation Agents / therapeutic use*
  • Child
  • Child, Preschool
  • Cross-Sectional Studies
  • Diphosphonates / administration & dosage
  • Diphosphonates / therapeutic use*
  • Female
  • Humans
  • Infant
  • Infusions, Intravenous
  • Logistic Models
  • Longitudinal Studies
  • Male
  • Osteogenesis Imperfecta / drug therapy*
  • Osteogenesis Imperfecta / rehabilitation
  • Pamidronate
  • Self Care
  • Walking*

Substances

  • Bone Density Conservation Agents
  • Diphosphonates
  • Pamidronate