儿童与成人血友病性骨关节病的探讨

Evaluation of Hemophilic Osteoarthropathy in Children and Adults

本文报告了经临床、X 线及实验室检查证实的9例血友病性骨关节病。年龄9～28岁,其中13岁以下5例。血友病甲7例,血友病乙2例。2例伴有假性骨肿瘤患者为血友病甲。9例均见膝关节受累,同时累及髋关节、踝关节各一例,2例假性骨肿瘤分别位于股骨上段及左髂骨、坐骨。X 线早期表现为关节内积血,软组织肿胀,关节间隙不规则狭窄,关节面凹凸不平,骨骺及干骺端增大,骨质稀疏及囊状透光区。文中阐述了本病的诊断和鉴别诊断,并结合文献就儿童与成人血友病性骨关节病区别进行了探讨。

Nine patients (age from 9 to 28 years) with hemophilic osteoarthropathy demon-strated by clinical manifestations, X-ray pictures and laboratory studies were studyreported. Of the 9 cases, 5 were younger than 13 years; 7 were hemophilia A, 2 hem-ophilia B; 2 cases associated with bone pseudotumor were hemophilia A. All pati-ents had lesions of the knee joint, one had also lesion of the hip joint, another hadlesion of the ankle. In two cases bone pseudotumors were located in the upper femurand left ilium and ischium. The early X-ray pictures were hematocele of the jointcavity, swelling of soft tissues, broadening of join slit. Chronic damages includedarticular cartilageous lesions irregular stenosing of the articular slit, irregular ofarticular facet, widening of the epiphysis and metaphysis, osteoporosis and saccatetransparent zone. The diagnosis and differential diagnosis of the disease, especiallydifferentiation of infantile and adult hemophylic osteoarthropathy, were discussed.