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目的:探讨静脉注射丙种球蛋白(IVIG)无反应性川崎病(KD)的临床特征、治疗方法、冠状动脉病变(CAL)发生的危险因素及预后。方法:回顾性分析总结该院心血管内科病房2008年6月~2011年11月收治的195例川崎病患儿的病历资料。按照对首次大剂量IVIG治疗的反应性,分为敏感组及无反应组。结果:195例KD患儿中,IVIG敏感组149例,IVIG无反应组46例。合并CAL 46例,IVIG敏感组15例,发生率10.07%;IVIG无反应组31例,发生率67.39%,明显高于IVIG敏感组,差异有统计学意义(P<0.05)。IVIG无反应组中血红蛋白明显低于敏感组,差异有统计学意义(P<0.05),血小板,血沉及谷丙转氨酶明显高于敏感组,差异有统计学意义(均P<0.05)。IVIG无反应性KD患儿经追加IVIG 1 g/kg或2 g/kg或糖皮质激素治疗后,均能退热。对46例合并CAL患儿出院后随访2年,45例恢复正常,1例巨大冠状动脉瘤有缩小,IVIG敏感组与IVIG无反应组预后(修)差异无统计学意义(P>0.05)。结论:IVIG无反应性KD较IVIG敏感性KD更易发生CAL。血小板、血沉及谷丙转氨酶升高、血红蛋白降低是IVIG无反应性的危险因素。IVIG无反应组经追加IVIG(1 g/kg或2 g/kg)或糖皮质激素治疗后,CAL发生率差异无统计学意义,预后良好。
Objective: To investigate the clinical features, treatment and risk factors of coronary artery disease (CAL) in patients with intraventricular gamma globulin (IVIG) -reactive Kawasaki disease (KD). Methods: A retrospective analysis of medical records of 195 children with Kawasaki disease admitted to our hospital from June 2008 to November 2011 was performed. According to the response to the first large-dose IVIG treatment, divided into sensitive group and non-responsive group. Results: Among 195 children with KD, 149 were in IVIG-sensitive group and 46 in IVIG non-responsive group. There were 46 cases with combined CAL and 15 cases with IVIG sensitive group, the incidence rate was 10.07%. There were 31 cases with IVIG non-responsive group, the incidence rate was 67.39%, which was significantly higher than IVIG sensitive group (P <0.05). The levels of hemoglobin in IVIG nonsusceptible group were significantly lower than those in susceptible group (P <0.05). The levels of platelet, erythrocyte sedimentation rate and alanine aminotransferase were significantly higher than those in sensitive group (all P <0.05). IVIG non-responsive KD children with anti-fever after additional IVIG 1 g / kg or 2 g / kg or glucocorticoid treatment. Forty-six patients with CAL were followed up for 2 years after discharge, 45 cases returned to normal, 1 case had giant coronary artery aneurysm shrinking, and there was no significant difference in prognosis between IVIG sensitized group and IVIG non-responsive group (P> 0.05). CONCLUSIONS: The IVIG non-responsive KD is more likely to develop CAL than IVIG-sensitive KD. Platelets, erythrocyte sedimentation rate and elevated alanine aminotransferase, hemoglobin is a risk factor for IVIG non-responsiveness. There was no significant difference in the incidence of CAL between IVIG non-responsive group and IVIG (1 g / kg or 2 g / kg) or glucocorticoid treatment. The prognosis was good.