Kotowicz M A
University of Melbourne, Department of Clinical and Biomedical Sciences, Barwon Health, Geelong, Victoria.
Aust Fam Physician. 2004 Mar;33(3):127-31.
Paget disease was first described in 1877 by Sir James Paget. It is a focal disorder of bone remodelling, involving increased bone resorption and formation. The aetiology is uncertain but both environmental and genetic factors are thought to be involved in pathogenesis.
This article outlines the clinical presentation, diagnosis and treatment of Paget disease.
Paget disease is associated with musculoskeletal pain, significant disability and impaired quality of life. Complications include pathological fracture, arthritis in adjacent joints, hearing loss, other neurological complications, heart failure and, rarely, osteosarcoma. Recent clinical trial data has demonstrated histological and radiological improvements in bone of patients treated with bisphosphonates. There is little data evaluating the long term effect of therapy on the risk of complications, however, restoration of normal bony architecture offers the prospect that complications related to deformity and increased bone fragility might be reduced by effective therapy.
佩吉特病于1877年由詹姆斯·佩吉特爵士首次描述。它是一种局限性骨重塑疾病，涉及骨吸收和形成增加。病因尚不确定，但环境和遗传因素均被认为与发病机制有关。
本文概述了佩吉特病的临床表现、诊断和治疗。
佩吉特病与肌肉骨骼疼痛、严重残疾和生活质量受损有关。并发症包括病理性骨折、相邻关节的关节炎、听力丧失、其他神经并发症、心力衰竭，以及罕见的骨肉瘤。最近的临床试验数据表明，接受双膦酸盐治疗的患者骨骼在组织学和放射学上有改善。然而，评估治疗对并发症风险长期影响的数据很少，不过，恢复正常的骨结构有望通过有效治疗降低与畸形和骨脆性增加相关的并发症。
