Osteoporosis in children with severe congenital neutropenia: Bone mineral density and treatment with bisphosphonates
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Date
2006
Journal Title
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Volume Title
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LIPPINCOTT WILLIAMS & WILKINS
Abstract
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 two 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 biphosphonates, 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 osteoperosis, decreases 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 bisphosphonate were seen on follow-up. Osteoporosis is an important comorbidity in SCN patients probably due to an increased bone resorption. Bisphosphonates seem to be an effective treatment for osteoporosis in these patients.
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Keywords
severe congenital neutropenia, osteoporosis, bisphosphonates, bone metabolism, Kostmann syndrome, COLONY-STIMULATING FACTOR, KOSTMANN-SYNDROME, NORMAL VALUES, CHILDHOOD, TURNOVER, MICE