小结
定义
病史和体格检查
关键诊断因素
- Paget 病家族史
- 无症状
其他诊断因素
- 累及股骨、骨盆和/或颅骨
- 长骨或背部疼痛
- 病理性骨折
- 骨骼畸形（例如额部隆起、下颌前突、骨弯曲）
- 局部体温增加
- 听力受损
- 面部疼痛
- 牙齿松动或咀嚼障碍
- 视觉灵敏度减退
危险因素
- Paget 病家族史
- 年龄＞50 岁
- 男性（45~74 岁）
- 感染
- 环境因素
诊断性检查
首要检查
- X 线平片
- 骨扫描
- 血清总碱性膦酸酶
- 骨特异性碱性膦酸酶
- 血清钙
- 血清原胶原 1 N-端肽 (P1NP)
- I 型胶原血清 C-端前肽 (CTX)
- 肝功能检查 (LFT)
- 血清 25 -羟基维生素 D
需考虑的检查
- CT 扫描或 MRI
- 骨活检
治疗流程
无症状患者，偶然诊断
有症状患者
撰稿人
作者
Julia F. Charles, MD, PhD
Associate Physician
Assistant Professor of Medicine
Brigham and Women's Hospital
Department of Orthopaedics and Medicine/Rheumatology
Boston
MA
利益声明
JFC has received royalties from Up To Date, Waltham, MA, for authorship on articles relating to Paget disease of bone (PDB). JFC has also received grant funding from the NIH, Rheumatology Research Foundation and Brigham and Women's Hospital for research into regulation of osteoclast function, but not specifically relating to PDB. JFC has had a manuscript published from a collaboration with Dr. Jae Shim describing a Pagetic phenotype in mice with osteoclast-specific loss of Chmp5.
鸣谢
Dr Julia F. Charles would like to gratefully acknowledge Dr Camilo Restrepo and Dr Javad Parvizi, previous contributors to this topic. CR and JP are authors of references cited in this topic.
同行评议者
Frederick Singer, MD
Director
Endocrine/Bone Disease Program
John Wayne Cancer Institute
Santa Monica
CA
利益声明
FS declares that he has no competing interests.
Joseph Lane, MD
Orthopaedic Surgeon
Hospital for Special Surgery
New York
NY
利益声明
JL declares that he has no competing interests.
鉴别诊断
- 骨软化症
- 骨纤维结构发育不良
更多 鉴别诊断指南
- 佩吉特骨病
更多 指南
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