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Marissa J. Perman, MD; Anne W. Lucky, MD; James E. Heubi, MD; Richard G. Azizkhan, MD, PhD

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Report of a Case

A 15-year-old boy with recessive dystrophic epidermolysis bullosa (RDEB) had severe osteopenia (lumbar spine bone mineral density, 0.348 g/cm²) (z score, –4.6; bone age–corrected z score, –3.6 [normal z score, greater than –2]) and compression fractures causing him to be bedridden. Prior to infusion of bisphosphonate, levels of serum ionized calcium (4.36 mg/dL; normal, 3.40-5.80 mg/dL), parathyroid hormone, phosphorous, and 25-hydroxyvitamin D were normal, as were results from renal function tests. He received intravenous elemental calcium (25 mg/kg) for hypocalcemia prophylaxis prior to his single intravenous bisphosphonate zoledronic acid infusion, 0.1 mg/kg (total dose, 4 mg, the recommended adult dose). This treatment was followed by administration of a repeated dose of intravenous elemental calcium (25 mg/kg), and then oral elemental calcium was prescribed (1500 mg, twice daily) along with cholecalciferol (6000 . . . [Full Text of this Article]

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