Case Report

Denosumab Treatment of Severe Disuse Osteoporosis in a Boy With Spinal Muscular Atrophy

Abstract

Denosumab is a fully human recombinant monoclonal antibody to the receptor activator of nuclear factor-κB ligand. Denosumab is used in the treatment of postmenopausal osteoporosis and cancer-related bone disorders. There are only very scarce data on denosumab treatment in children. 14-year-old boy with spinal muscular atrophy (SMA) and severe disuse osteoporosis (spinal bone mineral density L1-L4 BMD-6.2SD Z-score) and two prevalent fragility fractures was treated with denosumab. He received 60 mg  subcutaneous injection at the baseline and seven months later. Six months after the initial injection there was a 19% increase in L1-L4 BMD. The injections were well tolerated without any adverse reactions. Calcemia remained stable (2.3-2.4 mmol/L). He was scheduled for the third denosumab injection six months later. Prior to this date, he acquired pneumonia and died due to respiratory failure, which is a frequent cause of death in patients with SMA. There was no relation to the denosumab treatment. In conclusion, one dose of denosumab significantly increased BMD in a child with severe osteoporosis.

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IssueVol 55, No 10 (2017) QRcode
SectionCase Report(s)
Keywords
Denosumab Spinal muscular atrophy Fractures Bone Osteoporosis Bone mineral density

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Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Kutilek S. Denosumab Treatment of Severe Disuse Osteoporosis in a Boy With Spinal Muscular Atrophy. Acta Med Iran. 2017;55(10):658-660.