Original ArticlesOsteoporosis in Children With Severe Congenital Neutropenia: Bone Mineral Density and Treatment With BisphosphonatesBorzutzky, Arturo MD*; Reyes, María Loreto MD* †; Figueroa, Valeria MD*; García, Cristián MD* ‡; Cavieres, Mirta MD§Author Information Departments of *Pediatrics †Radiology ‡Endocrinology Unit, Medical School, Pontificia Universidad Católica de Chile §Hematology Unit, Hospital Luis Calvo Mackenna, Santiago, Chile Supported by Fundación Monseñor Fresno. Reprints: María Loreto Reyes, MD, Departamento de Pediatría, Pontificia Universidad Católica de Chile, Lira 85, 5to piso, Santiago, Chile (e-mail: email@example.com). Received for publication April 22, 2005; accepted January 26, 2006 Journal of Pediatric Hematology/Oncology: April 2006 - Volume 28 - Issue 4 - p 205-209 doi: 10.1097/01.mph.0000210409.48877.c3 Buy Metrics 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 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. © 2006 Lippincott Williams & Wilkins, Inc.