| 袁园,宋晓翔,封其华.全身型幼年特发性关节炎 66例分析[J].安徽医药,2020,24(10):1999-2003. |
| 全身型幼年特发性关节炎 66例分析 |
| Clinical study of 66 cases with systemiconset juvenile idiopathic arthritis |
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| DOI：10.3969/j.issn.1009?6469.2020.10.021 |
| 中文关键词: 关节炎，幼年型类风湿 铁蛋白质类 免疫球蛋白类 嘧啶二聚物 巨噬细胞活化综合征 儿童 |
| 英文关键词: Arthritis,juvenile rheumatoid Ferritins Immunoglobulins Pyrimidine dimers Macrophage activation syn?drome Child |
| 基金项目:苏州市科技发展计划(民生科技)项目(SYS2018065) |
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| 中文摘要: |
| 目的研究全身型幼年特发性关节炎(SoJIA)的临床表现、实验室检查特征和诊断及治疗措施。方法选取苏州大学附属儿童医院从 2011年 4月至 2016年 12月住院确诊的 66例 SoJIA病儿作为所研究的对象，对其发病特点、临床特征与辅助检查、治疗方法等方面展开研究与分析。结果 JIA病儿无特异性的临床表现，所有病儿均有发热， 2/3以上的病儿可出现关节炎及皮疹症状。 JIA无特殊的辅助检查手段，炎性因子的升高如 C反应蛋白、血沉，免疫球蛋白、铁蛋白、 D?二聚体等可有不同程度异常。大多数病儿经过初始治疗后，症状能够得到有效的控制，部分病儿病情可出现反复，需采用免疫抑制剂或生物制剂治疗； 8例确诊巨噬细胞活化综合征(MAS)的病儿均采取个体化的治疗方案， 1例病儿死亡，其余病儿症状均缓解。结论 SoJIA若是呈现持续发热不退或热退后复起、肝功能异常、多个系统的损伤以及血清铁蛋白明显升高时要考虑 MAS的可能，早期诊断、积极的联合治疗，是降低 MAS死亡率的重要措施。 |
| 英文摘要: |
| Objective To investigate characteristics of clinical manifestation，diagnosis and therapy in children with systemic onset juvenile idiopathic arthritis(SoJIA).Methods A total of 66 children with SoJIA who diagnosed in Children’s Hospital of Soochow University from April 2011 to December 2016were selected as objects of study，and a series of studies and analyses were conducted on the characteristics of their diseases，clinical symptoms and detection，and treatment methods.Results Children with JIA had no typical clinical syndrome.All the children had fever，more than 2/3 of the children could have arthritis and rash symptoms.The diag? nosis of JIA didn’t have specific auxiliary examination，and the elevation of inflammatory factors，such as c?reactive protein，blood sedimentation，immunoglobulin，ferritin，d?dimer，etc.might have different degrees of abnormalities.After the initial treatment，the symptoms of most sick children could be effectively controlled，and some children might have recurring conditions and need to betreated with immunosuppressants or biological agents.8 cases of children with macrophage activation syndrome(MAS)were treated with individualized treatment，1 case died，and the symptoms of the remaining sick children were relieved.Conclusion Consider? ing the possibility of MAS in patients with SoJIA，such as fever，liver damage，multiple system damage and ferritin，the early diagno? sis of MAS and active combination therapy are important measures to reduce the mortality of MAS. |
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