S S Beck-Nielsen1, K Brixen, J Gram, C Mølgaard. 1. Institute of Clinical Research, University of Southern Denmark, Winsløwparken 19.3, 5000, Odense C, Denmark. sbeck-nielsen@health.sdu.dk
Abstract
UNLABELLED: Bone mineral apparent density (BMAD) in children with X-linked hypophosphatemia (XLH) was evaluated, as they are unlikely to have extra-skeletal ossifications contributing to the elevated bone mineral density of the spine in adult patients. Children with XLH also had significantly higher BMAD of the spine compared to femoral neck. INTRODUCTION: BMAD obtained by dual-energy X-ray absorptiometry scans in children with XLH was evaluated, as they are unlikely to have the extra-skeletal ossifications contributing to the elevated bone mineral density of the spine in adult patients. METHODS: A total of 15 children with biochemically and genetically verified XLH were recruited. Anthropometric measurements were performed, and to correct for the short stature (small bones), the BMAD of the spine and the femoral neck was evaluated. RESULTS: Z-scores of BMAD of the spine (mean (95 % CI); 2.0 (1.3-2.7); p < 0.001) were significantly elevated compared to reference children. Z-scores of the femoral neck (1.0 (-0.0 to 2.1); p = 0.059) tended to be elevated. Spine Z-scores were significantly higher than the Z-scores of the femoral neck, (paired t test, p = 0.02). BMAD of the spine was evaluated according to the Molgaard's approach; XLH children had normal bone size of the spine for age due to a normal sitting height Z-score of -0.4 (-1.0 to 0.1); p = 0.1. Z-scores of bone mineral content (BMC) of the spine for bone area were elevated (1.4 (0.8-2.1); p < 0.001). No reference data were available to allow evaluation of the BMAD of the femoral neck by the Molgaard's approach. CONCLUSIONS: Children with XLH have an increased BMAD and a high BMC for bone area at the lumbar spine, and this was due to causes other than extra-skeletal ossifications and corrected for bone size. The BMAD of the spine was significantly higher compared to the femoral neck.
UNLABELLED: Bone mineral apparent density (BMAD) in children with X-linked hypophosphatemia (XLH) was evaluated, as they are unlikely to have extra-skeletal ossifications contributing to the elevated bone mineral density of the spine in adult patients. Children with XLH also had significantly higher BMAD of the spine compared to femoral neck. INTRODUCTION: BMAD obtained by dual-energy X-ray absorptiometry scans in children with XLH was evaluated, as they are unlikely to have the extra-skeletal ossifications contributing to the elevated bone mineral density of the spine in adult patients. METHODS: A total of 15 children with biochemically and genetically verified XLH were recruited. Anthropometric measurements were performed, and to correct for the short stature (small bones), the BMAD of the spine and the femoral neck was evaluated. RESULTS: Z-scores of BMAD of the spine (mean (95 % CI); 2.0 (1.3-2.7); p < 0.001) were significantly elevated compared to reference children. Z-scores of the femoral neck (1.0 (-0.0 to 2.1); p = 0.059) tended to be elevated. Spine Z-scores were significantly higher than the Z-scores of the femoral neck, (paired t test, p = 0.02). BMAD of the spine was evaluated according to the Molgaard's approach; XLH children had normal bone size of the spine for age due to a normal sitting height Z-score of -0.4 (-1.0 to 0.1); p = 0.1. Z-scores of bone mineral content (BMC) of the spine for bone area were elevated (1.4 (0.8-2.1); p < 0.001). No reference data were available to allow evaluation of the BMAD of the femoral neck by the Molgaard's approach. CONCLUSIONS:Children with XLH have an increased BMAD and a high BMC for bone area at the lumbar spine, and this was due to causes other than extra-skeletal ossifications and corrected for bone size. The BMAD of the spine was significantly higher compared to the femoral neck.
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