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

• Published in: Journal of bone and mineral metabolism 2010-11, Vol.28 (6), p.706-712
• Main Authors: Polyzos, Stergios A, Anastasilakis, Athanasios D, Litsas, Ioannis, Efstathiadou, Zoe, Kita, Marina, Arsos, Georgios, Moralidis, Efstratios, Papatheodorou, Athanasios, Terpos, Evangelos
• Format: Article
• Language: English
• Subjects: Adult; Biological and medical sciences; Bone and Bones - diagnostic imaging; Bone and Bones - drug effects; Bone and Bones - pathology; Bone Density Conservation Agents - adverse effects; Bone Density Conservation Agents - therapeutic use; Bones; Case Report; Diphosphonates - adverse effects; Diphosphonates - therapeutic use; Disease; Diseases of the osteoarticular system; Drug Monitoring; Endocrinopathies; Female; Humans; hyperparathyroidism; Hyperparathyroidism, Secondary - chemically induced; Hyperparathyroidism, Secondary - drug therapy; hypocalcemia; Hypocalcemia - chemically induced; Hypocalcemia - drug therapy; Imidazoles - adverse effects; Imidazoles - therapeutic use; Juvenile Paget's disease; Medical sciences; Medicine; Medicine & Public Health; Metabolic Diseases; Non tumoral diseases. Target tissue resistance. Benign neoplasms; Orthopedics; Osteitis Deformans - blood; Osteitis Deformans - drug therapy; Osteoporosis. Osteomalacia. Paget disease; Paget's disease of bone; Parathyroids. Parafollicular cells. Cholecalciferol. Phosphocalcic homeostasis (diseases); Radiography; Zoledronic acid
• ISSN: 0914-8779; 1435-5604
• DOI: 10.1007/s00774-010-0198-8

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.