Profound hypocalcemia following effective response to zoledronic acid treatment in a patient with juvenile Paget's disease.

Abstract

Juvenile Paget's disease (JPD) is a rare, autosomal recessive osteopathy. Although it has phenotypic overlap with Paget's disease of bone (PDB), it is probably a distinct entity. Because of its rarity, optimal disease management has not yet been established by randomized controlled trials. However, clinical, biochemical, and radiographic improvement has been reported after treatment with antiresorptive agents, including calcitonin and bisphosphonates (BPs). Compared with other BPs, zoledronic acid (ZOL) has a higher affinity to bone mineral and is a stronger inhibitor of the enzyme farnesyl pyrophosphate synthase (the main target of nitrogen-containing BPs), properties that explain the prolonged effect of ZOL on bone turnover and render it a therapeutic option for JPD, similar to PDB. We describe hereby, for the first time in the literature, the case of a patient with JPD who developed severe hypocalcemia and secondary hyperparathyroidism following effective treatment with ZOL.

• Authors: Stergios A Polyzos, Athanasios D Anastasilakis, Ioannis Litsas, Zoe Efstathiadou, Marina Kita, Georgios Arsos, Efstratios Moralidis, Athanasios Papatheodorou, Evangelos Terpos
• Journal: Journal of bone and mineral metabolism (J Bone Miner Metab) Vol. 28 Issue 6 Pg. 706-12 (Nov 2010) ISSN: 1435-5604 [Electronic] Japan
• PMID: 20533067 (Publication Type: Case Reports, Journal Article)

Chemical References

• Bone Density Conservation Agents
• Diphosphonates
• Imidazoles
• Zoledronic Acid

Topics

• Adult
• Bone Density Conservation Agents (adverse effects, therapeutic use)
• Bone and Bones (diagnostic imaging, drug effects, pathology)
• Diphosphonates (adverse effects, therapeutic use)
• Drug Monitoring
• Female
• Humans
• Hyperparathyroidism, Secondary (chemically induced, drug therapy)
• Hypocalcemia (chemically induced, drug therapy)
• Imidazoles (adverse effects, therapeutic use)
• Osteitis Deformans (blood, drug therapy)
• Radiography
• Zoledronic Acid