Eberle P, Mohr W, Claes L
Z Rheumatol. 1984 Sep-Oct;43(5):249-52.
The enzymatic defect leading to alcaptonuria is well known. However, the pathogenesis of the associated arthropathy is poorly understood. From clinical and morphological observations it is assumed that the breakdown of ochronotic cartilage is due to an increased fragility. To test the hypothesis that polymerisation products of homogentisic acid change the biomechanical behaviour of hyalin cartilage, investigations on natural ochronotic cartilage and tissues following in vitro incubation with homogentisic acid were performed. It could be demonstrated that the "ochronotic situation" is associated with an increased hardness and a decreased elasticity of the hyalin cartilage. For the pathogenesis of ochronotic arthropathy it is assumed that these alterations, in association with mechanical stress to the joints, lead to cartilage destruction.
导致尿黑酸尿症的酶缺陷已为人熟知。然而，相关关节病的发病机制却知之甚少。从临床和形态学观察来看，人们认为褐黄病性软骨的破坏是由于其脆性增加所致。为了验证尿黑酸的聚合产物会改变透明软骨生物力学行为这一假说，对天然褐黄病性软骨以及与尿黑酸进行体外孵育后的组织进行了研究。结果表明，“褐黄病状态”与透明软骨硬度增加和弹性降低有关。对于褐黄病性关节病的发病机制，人们认为这些改变与关节所受的机械应力共同作用，导致了软骨破坏。
