Familial hypophosphatemic rickets: bone mass measurements in children following therapy with calcitriol and supplemental phosphate

Calcified Tissue International
J E BlockH K Genant

Abstract

Familial hypophosphatemic rickets is characterized by defective skeletal mineralization resulting in abnormal growth and development. The pathologic and radiologic correlates of this syndrome have been given some investigation, but the effect of this mineralization defect on bone mineral density has not been adequately assessed. We measured axial and appendicular bone mineral in 17 children (mean age 5.59 +/- 4.87) with familial hypophosphatemia at baseline and at 6-month intervals after initiation of therapy with vitamin D3 (calcitriol) and phosphate supplementation. Noninvasive quantitative techniques included single photon absorptiometry (SPA) of the radius, combined cortical thickness (CCT) of the second metacarpal, and quantitative computed tomography (QCT) of vertebral trabecular bone. Thoraco-lumbar and hand/wrist radiographs were qualitatively assessed for the prevalence and severity of osteosclerosis, rickets, and other parameters indicative of metabolic bone disease as well as skeletal age. Quantitative determinations of bone mineral by each technique were compared with normal values for age and sex, and individual standardized scores (z-scores) were calculated at each measurement interval. Standard scores were also c...Continue Reading

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Citations

Feb 8, 2013·Osteoporosis International : a Journal Established As Result of Cooperation Between the European Foundation for Osteoporosis and the National Osteoporosis Foundation of the USA·S S Beck-NielsenC Mølgaard
Jul 22, 2014·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·Vikram V ShanbhogueSigne Sparre Beck-Nielsen
Mar 28, 2013·The Journal of Clinical Endocrinology and Metabolism·Moira CheungFrank Rauch
Jan 1, 1990·Pediatric Radiology·F J BeekP P Kramer
Sep 7, 2012·Phytopathology·Tito CaffiVittorio Rossi
Aug 9, 2019·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Marie-Eve RobinsonFrank Rauch

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