Endocrine/Bone Disease Program, John Wayne Cancer Institute at Providence Saint Johns Health Center, Santa Monica, 2200 Santa Monica Blvd. Santa Monica, CA, 90404, USA; Clinical Professor of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Best Pract Res Clin Rheumatol. 2020 Jun;34(3):101506. doi: 10.1016/j.berh.2020.101506. Epub 2020 Apr 11.
Paget's disease of bone is a localized skeletal disorder, which is more common in England and in countries to which the English migrated. In recent decades, the prevalence in most countries has decreased. A family history of the disorder is present in approximately 15% of patients. Patients may be asymptomatic and may be diagnosed accidently as a consequence of an elevated serum alkaline phosphatase level or a finding on an x-ray or nuclear bone scan. The diagnosis is made by x-ray but nuclear bone scans define the extent of the disease. Salmon calcitonin and bisphosphonate drugs have proven effective, but by far, the most effective therapy is a single 5 mg intravenous infusion of zoledronic acid. This can normalize alkaline phosphatase levels for up to 6.5 years. A variety of gene mutations may predispose individuals to develop the disease but environmental factors such as measles virus likely play an important role.
佩吉特病是一种局部骨骼疾病,在英国和英国移民的国家更为常见。近几十年来,大多数国家的患病率都有所下降。大约 15%的患者有该疾病的家族史。患者可能无症状,可能由于血清碱性磷酸酶水平升高或 X 射线或核骨扫描发现而意外诊断出该疾病。诊断是通过 X 射线进行的,但核骨扫描可确定疾病的范围。降钙素和双膦酸盐药物已被证明有效,但到目前为止,最有效的治疗方法是单次 5mg 静脉注射唑来膦酸。这可以使碱性磷酸酶水平在长达 6.5 年内恢复正常。多种基因突变可能使个体易患该疾病,但麻疹病毒等环境因素可能发挥重要作用。