Department of Endocrinology and Metabolism, Ajou University School of Medicine, Suwon, Korea.
Department of Medical Genetics, Ajou University School of Medicine, Suwon, Korea.
Endocrinol Metab (Seoul). 2022 Oct;37(5):732-743. doi: 10.3803/EnM.2022.1575. Epub 2022 Oct 25.
Paget's disease of the bone is a prevalent bone disease characterized by disorganized bone remodeling; however, it is comparatively uncommon in East Asian countries, including China, Japan, and Korea. The exact cause still remains unknown. In genetically susceptible individuals, environmental triggers such as paramyxoviral infections are likely to cause the disease. Increased osteoclast activity results in increased bone resorption, which attracts osteoblasts and generates new bone matrix. Fast bone resorption and formation lead to the development of disorganized bone tissue. Increasing serum alkaline phosphatase or unique radiographic lesions may serve as the diagnostic indicators. Common symptoms include bone pain, bowing of the long bones, an enlarged skull, and hearing loss. The diagnosis is frequently confirmed by radiographic and nuclear scintigraphy of the bone. Further, bisphosphonates such as zoledronic acid and pamidronate are effective for its treatment. Moreover, biochemical monitoring is superior to the symptoms as a recurrence indicator. This article discusses the updates of Paget's disease of bone with a clinical case.
佩吉特病是一种常见的骨骼疾病，其特征为骨骼重塑紊乱；然而，包括中国、日本和韩国在内的东亚国家较为少见。确切病因仍不清楚。在遗传易感个体中，副粘病毒感染等环境诱因可能导致该病。破骨细胞活性增加导致骨吸收增加，继而吸引成骨细胞并产生新的骨基质。快速的骨吸收和形成导致了紊乱的骨骼组织的发展。血清碱性磷酸酶升高或独特的放射性影像学病变可作为诊断指标。常见症状包括骨痛、长骨弯曲、头颅增大和听力损失。该病通常通过骨骼的放射影像学和核闪烁成像来确诊。此外，唑来膦酸和帕米膦酸等双膦酸盐类药物对此病的治疗有效。而且，生化监测作为复发指标优于症状。本文结合临床病例讨论了佩吉特病的最新研究进展。
