Children's Healthcare of Atlanta/Emory University, Atlanta, GA, USA.
Haemophilia. 2011 Jul;17(4):571-8. doi: 10.1111/j.1365-2516.2010.02472.x. Epub 2011 Feb 22.
Recurrent haemarthroses in patients with severe and moderate haemophilia can result in the development of one or more target joints and subsequent degenerative joint disease. This debilitating process is characterized by physical and physiological changes in articular cartilage, synovium and bone. Models of degenerative joint disease have been examined after the addition of whole blood or blood components to cell cultures or animal joints, or by monitoring biomarkers in individuals with and without haemophilia. Inhibition of cartilage-based proteoglycan synthesis and induction of proliferative synovitis are commonly observed in these models of degenerative joint disease. Clinical evaluation of joint disease includes use of specially designed physical examination and radiographic tools. Efforts to prevent or limit arthropathy include the use of prophylactic factor infusion regimens, surgical joint intervention or both.
反复关节积血可导致重型和中型血友病患者出现一个或多个靶关节，并随后发生退行性关节病。这种使人虚弱的过程的特征是关节软骨、滑膜和骨骼的物理和生理变化。向细胞培养物或动物关节中添加全血或血液成分，或监测有无血友病个体的生物标志物，可检查退行性关节病模型。这些退行性关节病模型中通常可观察到基于软骨的蛋白聚糖合成抑制和增生性滑膜炎诱导。关节疾病的临床评估包括使用专门设计的体格检查和影像学工具。预防或限制关节病的方法包括使用预防性因子输注方案、关节手术干预或两者兼用。
