川崎病发生巨噬细胞活化综合征的危险因素分析

目的 探讨川崎病（kawasaki disease,KD）患儿发生巨噬细胞活化综合征（macrophage activation syndrome,MAS）的危险因素.方法 回顾性分析2000-01～2008-05诊治的278例KD患儿的临床及实验室资料,采用多因素Logistic回归分析探讨KD发生MAS的危险因素.结果 （1）全组有6例KD患儿发生MAS,发生率为2.1%（6/278）.（2）KD发生MAS的年龄、住院天数、确诊时间、热程、静脉注射免疫球蛋白（IVIG）治疗次数、对IVIG反应率、肝脾肿大及关节痛发生率与KD无发生MAS者比较差异有统计学意义;而性别比例、手足硬肿、眼结合膜充血、草莓舌、淋巴结肿大、皮疹等并发症的发生率及冠脉并发症发生率两者差异无统计学意义.（3）KD发生MAS者血小板（PLT）计数、白细胞（WBC）计数、血清白蛋白（ALB）及纤维蛋白原（Fib）均低于未发生MAS的KD者,而谷丙转氨酶（ALT）、谷草转氨酶（AST）、乳酸脱氢酶（LDH）、甘油三酯（TG）、血清铁蛋白（SF）发生MAS者均高于单纯KD组,差异有统计学意义.结论 多因素Logisitic回归分析表明年龄＞7岁、热程＞10 d、对IVIG治疗无反应、关节疼痛、PLT计数＜100×10^9、ALT＞200 U/L、LDH＞1 000 U/L及血清铁蛋白＞500 μg/L等因素是KD患儿发生MAS的独立相关危险因素.

The risk factors of macrophage activation syndrome in children with kawasaki disease

Objective To investigate the risk factors of macrophage activation syndrome（MAS） in children with kawasaki disease. Methods A retrospective study was performed on the clinical and laboratory data of 278 KD children from January 2000-May 2008. Then logistic regression was used to identify the risk factors of macrophage ac- tivation syndrome in children with kawasaki disease. Results （ 1 ） Of the 278 KD patients, 6 patients （2. 1% ） were found to have a codiagnosis of MAS and KD; （2）The median age,duration of hospitalizition, time of MAS diagono- sis, the days of fever, reaction rate of the treatment of IVIG, hepatosplenomegaly, arthralgia and/or mylagia were higher in MAS patients, but there were not signifeant difference between two groups about redness of palms and soles, bilateral nonexudative conjunctivitis, lip or oral cavity changes, cervical lymphadenopathy, polymorphous exanthema. （3）The level of PLT, WBC, ALb, Fib in MAS patients were lower than that of KD patients. All MAS patients had elevated ALT, elevated AST. elevated LDH, elevated TG, elevated SF, there were signifcant difference between two groups. Conclusion Multivariate logisitic regression analysis showed that the risk factors of MAS in children with kawasaki disease were 〉7 age, lasting fever 〉 10 days, no reaction to the treatment of IVIG, arthralgia, PLT〈 ×10^9, ALT 〉200 U/L, LDH 〉 1 000 U/L, SF 〉500μg/L.