Fujita K, Kobayashi K, Okino F
Department of Paediatrics, Yamaguchi University School of Medicine, Japan.
Eur J Pediatr. 1988 Nov;148(2):118-9. doi: 10.1007/BF00445916.
A sister and brother with congenital leucocyte adhesion deficiency developed systemic-onset juvenile rheumatoid arthritis (JRA). They showed polyarthritis, spiking fever, reddish eruptions, anaemia, hepatosplenomegaly, increased erythrocyte sedimentation rate, and positive rheumatoid factor. Occurrence of JRA in our patients was thought to be mainly due to a combination of recurrent bacterial infections and abnormal lymphocyte function as a consequence of membrane adhesion-protein deficiency. In view of the familial occurrence, hereditary factors may have played a role in the development of JRA in our patients.
一对患有先天性白细胞黏附缺陷症的姐弟患上了全身型幼年类风湿关节炎（JRA）。他们表现出多关节炎、弛张热、皮疹、贫血、肝脾肿大、红细胞沉降率升高以及类风湿因子阳性。我们的患者发生JRA被认为主要是由于反复细菌感染以及膜黏附蛋白缺乏导致的淋巴细胞功能异常共同作用的结果。鉴于家族性发病情况，遗传因素可能在我们患者JRA的发病过程中发挥了作用。
