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目的分析全身型幼年特发性关节炎(JIA)患儿的临床资料,总结该病的临床特点。方法回顾分析1995年2月至2004年9月中山大学附属第一医院儿科收治的93例全身型JIA患儿的临床症状、体征及辅助检查资料。结果93例患儿均有发热,其中不规则发热59例,弛张热34例;皮疹59例(63.4%);常见临床症状为疲乏、纳差、体重下降、面色苍白;关节痛以大关节为多见,累及关节由多到少依次为:膝、肘、踝、腕、掌指、髋关节、全身大关节、髂关节;淋巴结肿大46例(49.5%);肝和(或)脾肿大38例(40.9%)。实验室检查结果:白细胞总数≥10×109/L80例,轻中度贫血34例(37.7%),多为小细胞低色素性贫血;血沉增快85例(占94.4%);C反应蛋白(CRP)检测87例,升高79例(占90.8%);行血清免疫球蛋白IgG检查83例,升高55例,IgA升高34例IgM升高42例;行CD+3、CD+4、CD+8、CD+4/CD+8比值测定41例,CD+3(0.65±0.12),CD+4(0.33±0.09),CD8(0.27±0.09)%,CD+4/CD+8比值为(1.38±0.49),与正常对照组相比,t检验示CD3:P>0.05,CD+4、CD+8及CD+4/CD+8比值P值均<0.01。34例行血清铁蛋白检查,升高18例(占47.8%);心电图检查70例,异常44例(占62.6%);行病变关节X线检查26例,异常5例;类风湿因子检查90例,88例阴性,2例阳性。结论全身型JIA临床表现多样化,发热、大关节痛、皮疹、疲乏、纳差等常见。肝、脾和淋巴结肿大,血沉和CRP大部分升高,并有细胞免疫、体液免疫异常。
Objective To analyze the clinical data of children with systemic juvenile idiopathic arthritis (JIA) and summarize the clinical features of the disease. Methods The clinical symptoms, signs and auxiliary examinations of 93 cases of JIA children admitted to the First Affiliated Hospital of Sun Yat-sen University from February 1995 to September 2004 were retrospectively analyzed. Results All the 93 children had fever, of which 59 were irregular fever, 34 were remission fever, 59 were rash (63.4%). The common clinical symptoms were fatigue, anorexia, weight loss and paleness. More common, involving more or less from the joint as follows: knee, elbow, ankle, wrist, metacarpophalangeal, hip, general joint, iliac joint; swollen lymph nodes in 46 cases (49.5%); liver and Edema 38 cases (40.9%). The results of laboratory tests showed that the total number of white blood cells was ≥10 × 109 / L in 80 cases, mild to moderate anemia in 34 cases (37.7%), most of which were small cell hypochromic anemia, 85 cases (94.4%) of erythrocyte sedimentation rate increased rapidly, C-reactive protein Serum immunoglobulin IgG was detected in 83 cases, 55 cases increased, IgA increased in 34 cases IgM increased in 42 cases; CD +3, CD +4 , CD + 3 (0.65 ± 0.12), CD + 4 (0.33 ± 0.09), CD8 (0.27 ± 0.09)%, CD + 4 / CD + 8 (P <0.05). The P value of CD3: P> 0.05, CD + 4, CD + 8 and CD + 4 / CD + 8 were all less than 0.01.34, compared with the normal control group Ferritin examination, elevated in 18 cases (47.8%); electrocardiogram in 70 cases, abnormal 44 cases (62.6%); line lesions joint X-ray examination in 26 cases, abnormal 5 cases; rheumatoid factor 90 cases, 88 cases Negative, 2 cases were positive. Conclusion The clinical manifestations of systemic JIA are diverse, including fever, joint pain, rash, fatigue, and anorexia. Liver, spleen and lymph nodes, erythrocyte sedimentation rate and CRP most of the rise, and cellular immunity, humoral immunity.