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目的探讨危重症幼年特发性关节炎(JIA)的临床表现和对策。方法回顾分析13例幼年特发性关节炎合并严重并发症患儿的病史,及心电图、心脏腹部超声、肝肾功能、骨髓像等相关检查,总结危重症幼年特发性关节炎临床特点、治疗并且随诊。结果13例患儿均诊断为全身型JIA。13例JIA中发生巨噬细胞活化综合征(MAS)9例,心功能衰竭6例,急性脑病5例,少尿、肾功能衰竭4例,肝脏衰竭2例,呼吸衰竭1例,休克9例,弥散性血管内凝血(DIC)4例,败血症合并深部巨大脓肿1例,胸腔积液4例,心包积液2例,肺炎7例。10例重要脏器功能衰竭在1~2周内恢复。严重的出血、贫血2周内恢复。1个月后复诊肝、肾功能均全部正常。抽搐、昏迷患儿智力正常。随诊中未发现继发性癫痫。随访中均未出现MAS。死亡3例。结论危重型JIA进展快,同时累及多个器官。充血性心力衰竭、急性肾功能衰竭、急性脑病最多见,常合并MAS。大剂量激素、综合对症治疗是关键。危重症JIA病死率高,但危重情况一旦纠正预后良好。
Objective To investigate the clinical manifestations and countermeasures of juvenile idiopathic arthritis (JIA) in critically ill patients. Methods A retrospective analysis of 13 cases of juvenile idiopathic arthritis with severe complications in children with history, and electrocardiogram, heart abdomen ultrasound, liver and kidney function, bone marrow and other related examination, summarize the clinical features of juvenile idiopathic arthritis, treatment And follow-up. Results All 13 cases were diagnosed as systemic JIA. Thirteen patients with JIA developed macrophage activation syndrome (MAS) in 9, heart failure in 6, acute encephalopathy in 5, oliguria, renal failure in 4, liver failure in 2, respiratory failure in 1 and shock in 9 , 4 cases of diffuse intravascular coagulation (DIC), 1 case of sepsis complicated with deep abscesses, 4 cases of pleural effusion, 2 cases of pericardial effusion and 7 cases of pneumonia. 10 cases of vital organs failure in 1 to 2 weeks recovery. Severe bleeding, anemia recovered within 2 weeks. 1 month after the referral liver and kidney function are all normal. Convulsions, coma children with normal intelligence. Follow-up did not find secondary epilepsy. MAS did not appear in the follow-up. 3 died. Conclusions The critical JIA progresses rapidly and involves multiple organs simultaneously. Congestive heart failure, acute renal failure, acute encephalopathy most common, often combined with MAS. High-dose hormones, integrated symptomatic treatment is the key. Critical illness JIA high mortality, but the critical situation once corrected the prognosis is good.