Document Detail

Osteoporosis in children with severe congenital neutropenia: bone mineral density and treatment with bisphosphonates.
MedLine Citation:
PMID:  16679916     Owner:  NLM     Status:  MEDLINE    
A high incidence of decreased bone mineral density (BMD) has been described in patients with severe congenital neutropenia (SCN). The objectives of the study are to describe changes in BMD in children with SCN treated with granulocyte colony-stimulating factor and evaluate the response to treatment with bisphosphonates in those who had osteoporosis. A prospective open-label study was performed evaluating BMD and metabolism in 9 Chilean patients with SCN, administrating bisphosphonates in those with osteoporosis. Follow-up ranged between 7 months and 3.5 years. Six out of 9 patients had reduced BMD on initial assessment: 3 had osteoporosis (z score <-2) and 3 had osteopenia (z score <-1). Four children presented vertebral fractures. Two presented osteopenia on follow-up without clinical symptoms. Five patients were treated with bisphosphonates, increasing their BMD z score (mean increase 1.2, range 0.27 to 2.62). z Score of hydroxyproline/creatinine ratios, which was elevated in 4 patients with osteoporosis, decreased during treatment (mean decrease 2.18, range 1.56 to 2.53). Four patients remodeled and reexpanded fractured vertebrae during treatment. No side effects of bisphosphonates were seen on follow-up. Osteoporosis is an important comorbidity in SCN patients probably due to increased bone resorption. Bisphosphonates seem to be an effective treatment for osteoporosis in these patients.
Arturo Borzutzky; María Loreto Reyes; Valeria Figueroa; Cristián García; Mirta Cavieres
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Publication Detail:
Type:  Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  28     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2006 Apr 
Date Detail:
Created Date:  2006-05-08     Completed Date:  2006-06-13     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  205-9     Citation Subset:  IM    
Department of Pediatrics, Medical School, Pontificia Universidad Católica de Chile, Lira 85, Santiago, Chile.
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MeSH Terms
Bone Density*
Bone Density Conservation Agents / therapeutic use*
Child, Preschool
Diphosphonates / therapeutic use*
Motor Activity
Neutropenia / complications*,  congenital*
Osteoporosis / epidemiology*
Prospective Studies
Spine / radiography
Treatment Outcome
Reg. No./Substance:
0/Bone Density Conservation Agents; 0/Diphosphonates

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