Document Detail

Intravenous bisphosphonate therapy in children with osteogenesis imperfecta.
MedLine Citation:
PMID:  12612238     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Several studies have reported beneficial effects of bisphosphonates in children with osteogenesis imperfecta (OI); however, these studies have differed in the protocols they used, and none has been independently replicated. We intended to confirm the efficacy of a specific intravenous bisphosphonate protocol in children with moderate to severe OI. METHODS: We used the protocol described by Glorieux et al and performed a prospective clinical trial in 6 children who were aged 22 months to 14 years. Each patient received intravenous pamidronate therapy for a minimum of 2 years in cycles of 1 mg/kg daily over 3 consecutive days at a mean cycle interval of 3.8 months. Outcome measures included lumbar spine areal bone mineral density (BMD) and z score, fracture rate, and occupational therapy functional assessment with serial Pediatric Evaluation of Disability Inventory. RESULTS: While on therapy, the average annual increase in areal BMD was 48% and the average annual increase in BMD z score was 1.0. This increase in z score is statistically significant. There was no clear correlation between changes in BMD and fracture rate. All patients experienced functional improvement in mobility. CONCLUSIONS: Our results support the findings of Glorieux et al that cyclic administration of intravenous pamidronate in children with OI has beneficial effects with respect to BMD z scores and physical disability. Long-term follow-up will be required to determine whether bisphosphonate therapy will decrease fracture rates and increase mobility in children with moderate to severe OI.
Marni J Falk; Shauna Heeger; Katherine A Lynch; Kathleen R DeCaro; Deborah Bohach; Karen S Gibson; Matthew L Warman
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Publication Detail:
Type:  Clinical Trial; Comparative Study; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Pediatrics     Volume:  111     ISSN:  1098-4275     ISO Abbreviation:  Pediatrics     Publication Date:  2003 Mar 
Date Detail:
Created Date:  2003-03-03     Completed Date:  2003-06-02     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0376422     Medline TA:  Pediatrics     Country:  United States    
Other Details:
Languages:  eng     Pagination:  573-8     Citation Subset:  AIM; IM    
Department of Genetics and Center for Human Genetics, University Hospitals of Cleveland and Case Western Reserve University School of Medicine, Cleveland, Ohio, USA.
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MeSH Terms
Age Factors
Bone Density / drug effects,  physiology
Child, Preschool
Diphosphonates / administration & dosage,  pharmacology,  therapeutic use*
Disability Evaluation
Fractures, Bone / diagnosis
Infusions, Intravenous
Knee Joint / radiography
Lumbar Vertebrae / drug effects,  physiology
Occupational Therapy
Osteogenesis Imperfecta / diagnosis,  drug therapy*,  radiography
Treatment Outcome
Grant Support
Reg. No./Substance:
0/Diphosphonates; 40391-99-9/pamidronate

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