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Acute paediatrics
Beneficial effect of long term intravenous bisphosphonate treatment of osteogenesis imperfecta
  1. E Åström1,
  2. S Söderhäll2
  1. 1Department of Woman and Child Health, Divison of Paediatric Neurology, Astrid Lindgren Children's Hospital and Södersjukhuset, Stockholm, Sweden
  2. 2Childhood Cancer Research Unit, Astrid Lindgren Children's Hospital
  1. Correspondence to:
    Dr E Åström, Division of Paediatric Neurology, Astrid Lindgren Children's Hospital, 171 76 Stockholm, Sweden;
    e4477601{at}home.se

Abstract

Aim: To find an effective symptomatic treatment for osteogenesis imperfecta (OI).

Methods: In a prospective observational study disodium pamidronate (APD) was given as monthly intravenous infusions to 28 children and adolescents (aged 0.6–18 years) with severe OI or a milder form of the disease, but with spinal compression fractures.

Results: During treatment for 2–9 years, dual energy x ray absorptiometry measurements of the total body and of the lumbar spine showed a gradual increase in bone density. All bone metabolism variables in serum (alkaline phosphatase, osteocalcin, procollagen 1 C-terminal peptide, collagen 1 teleopeptide) and urine (deoxypyridinoline) indicated that there was a decrease in bone turnover. All patients experienced beneficial effects and the younger patients reported a major improvement in wellbeing, pain, and mobility without significant side effects. Vertebral remodelling was also seen.

Conclusions: APD seems to be an efficient symptomatic treatment for children and adolescents with OI.

  • bisphosphonate
  • osteogenesis imperfecta
  • ALP, alkaline phosphatase
  • APD, disodium pamidronate
  • 1CTP, collagen 1 teleopeptide
  • DXA, dual energy x ray absorptiometry
  • OI, osteogenesis imperfecta
  • P1CP, procollagen 1 C-terminal peptide

https://doi.org/10.1136/adc.86.5.356

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