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Effect of intravenous pamidronate therapy on everyday activities in children with osteogenesis imperfecta

Kristina Löwing1, Eva Aström, Katarina Allbrink Oscarsson

  • 1Department of Woman and Child Health, Astrid Lindgren Children's Hospital, Karolinska Institutet, Sweden. kristina.lowing@ki.se

Acta Paediatrica (Oslo, Norway : 1992)
|June 21, 2007
PubMed

Insights

Pamidronate therapy improved daily activities, reduced pain, and increased bone density in children with osteogenesis imperfecta (OI). This bisphosphonate treatment positively impacted overall well-being and functional status in young patients.

Area of Science:

  • Pediatric Endocrinology
  • Bone Metabolism
  • Genetic Disorders

Background:

  • Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by fragile bones and skeletal deformities.
  • Current treatments aim to improve bone strength and reduce fracture risk, but effects on daily function and well-being require further evaluation.

Purpose of the Study:

  • To assess the impact of intravenous pamidronate therapy on functional abilities, pain levels, well-being, and bone mineral density in children diagnosed with osteogenesis imperfecta.
  • To determine if pamidronate treatment influences the health status of pediatric OI patients.

Main Methods:

  • A prospective observational study involving 43 children with OI (age range: 4 months to 16 years).
  • Monthly intravenous pamidronate infusions administered over one year.
  • Outcome measures included the Pediatric Evaluation of Disability Inventory (PEDI), a well-being score, pain frequency, and bone density via DXA scans, with data collected pre- and post-treatment.

Main Results:

  • Significant improvements were observed in self-care and mobility scores on the PEDI (Functional Skill and Caregiver Assistance scales).
  • Children experienced a reduction in skeletal pain and reported increased well-being.
  • Bone mineral density (BMD) showed an increase following one year of pamidronate treatment, with younger children demonstrating greater PEDI improvements.

Conclusions:

  • Intravenous pamidronate therapy demonstrated significant benefits for children with osteogenesis imperfecta.
  • The treatment positively influenced functional status, skeletal pain, well-being, and bone density, aligning with the International Classification of Functioning, Disability and Health (ICF).
  • Bisphosphonate therapy represents a valuable therapeutic option for managing OI in pediatric populations.
Abstract

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