Cogulu O, Ozkinay F, Ozkinay C, Sapmaz G, Yalman O, Deveci H B
Department of Paediatrics, Ege University, Faculty of Medicine, Izmir, Turkey.
Indian J Pediatr. 1999 May-Jun;66(3):455-60. doi: 10.1007/BF02845540.
Progressive pseudorheumatoid arthropathy of childhood (PPAC) described by Spranger et al is a rare autosomal recessive disorder. An 11 year-old girl was diagnosed as having PPAC at Ege University, Faculty of Medicine, Department of Paediatrics. Her complaints of painful joints, difficulty in walking and joint contractures began at the age of 3 years and she was treated for juvenile rheumatoid arthritis for 8 years. Her symptoms did not respond to nonsteroid anti-inflammatory treatment. During her last hospitalisation period, she was reinvestigated. Radiological examination showed spondyloepiphyseal dysplasia, severe acetabular irregularity and osteoporosis. All the laboratory test results for rheumatoid arthritis were negative. The clinical and radiological findings of the patient are illustrated.
施普朗格等人描述的儿童进行性假类风湿性关节病（PPAC）是一种罕见的常染色体隐性疾病。一名11岁女孩在伊兹密尔艾杰大学医学院儿科学系被诊断为患有PPAC。她关节疼痛、行走困难和关节挛缩的症状始于3岁，曾接受8年的幼年型类风湿性关节炎治疗。她的症状对非甾体抗炎治疗无反应。在她最后一次住院期间，进行了重新检查。放射学检查显示脊椎骨骺发育不良、严重髋臼不规则和骨质疏松。所有类风湿性关节炎的实验室检查结果均为阴性。展示了该患者的临床和放射学检查结果。
