Effects of hemophilia on articulations of children and adults.
Effects of hemophilia on articulations of children and adults.
复制标题
血友病对儿童和成人关节的影响。
DOI：
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发表时间：
1996
期刊：
影响因子：
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通讯作者：
Rodríguez
中科院分区：
文献类型：
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作者：
Rodríguez
血友病患者的大多数出血事件发生在关节内。在这些关节积血中，膝关节、肘关节和踝关节几乎占80%。如果出血持续，滑膜开始肥大，并形成慢性滑膜炎的恶性循环，导致关节破坏。在未成熟的关节，滑膜炎导致肥大的骨骺生长板和重大的结构缺陷可能会迅速发展。这种对生长板的刺激导致骨骼肥大、腿长不等和角度畸形。在成熟的关节中，血友病对关节软骨有重大的不利影响。随着病情逐渐加重，关节功能也会恶化。关节间隙丢失是与关节活动度相关的最重要影像学发现。随着滑膜瘢痕化程度的增加，脆弱的充血组织逐渐转化为纤维化瘢痕组织。这个过程是血友病性关节病的自然过程。成人血友病患者的一个现象是，他们的关节，在放射学上看起来严重破坏，似乎功能良好多年。
: The majority of bleeding episodes in hemophilic patients occur within the joints. Of these hemarthroses, the knees, elbows, and ankles account for almost 80%. Should the bleeding persist, the synovium starts to hypertrophy and a vicious cycle of chronic synovitis develops, leading to joint destruction. In immature articulation, synovitis causes hypertrophy of the epiphyseal growth plates and significant structural deficiencies may rapidly develop. This stimulus to the growth plates results in bone hypertrophy, leg length discrepancy, and angular deformities. In mature articulation, hemophilia has a major detrimental effect on the joint cartilage. As it progressively exacerbates, joint function deteriorates. Loss of joint space is the most important radiographic finding related to range of motion. As the synovium becomes increasingly scarred, there is gradual conversion from friable hyperemic tissue to fibrotic scar tissue. This process is the natural course of hemophilic arthropathy. A phenomenon in adult hemophilic patients is that their joints, which radiographically appear severely destroyed, seem to function reasonably well for many years.