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静脉注射免疫球蛋白抵抗性川崎病的管理与结局

Management and outcome of intravenous gammaglobulin-resistant Kawasaki disease.

作者信息

Sittiwangkul R, Pongprot Y, Silvilairat S, Phornphutkul C

机构信息

Department of Paediatrics, Division of Cardiology, Faculty of Medicine, Chiang Mai University Hospital, 110 Intavaroros Road, Chiang Mai 50200, Thailand.

出版信息

Singapore Med J. 2006 Sep;47(9):780-4.

PMID:16924360
Abstract

INTRODUCTION

This study aimed to determine the prevalence and risk of intravenous gammaglobulin (IVIG)-resistant Kawasaki disease (KD) and report the outcome of treatment in patients with persistent or recurrent fever.

METHODS

70 KD patients, who received IVIG treatment (2 g/kg) at a tertiary care hospital from January 1995 to June 2004, were retrospectively reviewed.

RESULTS

Nine (13 percent) of the 70 patients failed to respond to initial treatment with IVIG. The patients who did not respond to IVIG had higher erythrocyte sedimentation rate (ESR) (104 versus 74 mm/h; p-value is 0.003), longer total days of fever (14.4 +/- 3.8 versus 9.2 +/- 2.3 days; p-value is 0.003) and higher initial coronary artery lesions (CAL) (7 of 9 [77.7 percent] versus 10 of 61 [16.3 percent]; p-value is 0.001) than those who responded to initial treatment. Seven of the nine patients who were retreated with IVIG (2 g/kg) responded to the second dose. The remaining two patients (two of nine, 22 percent) had persistent fever, which subsided after two to three doses of pulse intravenous methylprednisolone. At two months follow-up, IVIG-resistant patients had higher CAL by echocardiogram than IVIG-responsive patients (33 percent versus 3.2 percent, p-value is less than 0.05). Two IVIG-resistant KD patients had delayed diagnosis and developed giant aneurysms.

CONCLUSION

Patients with high ESR had increased risk of IVIG-resistant KD. IVIG-resistant Kawasaki patients had a higher prevalence of CAL at the acute phase and two months after onset.

摘要

引言

本研究旨在确定静脉注射丙种球蛋白(IVIG)抵抗性川崎病(KD)的患病率和风险,并报告持续或反复发热患者的治疗结果。

方法

回顾性分析了1995年1月至2004年6月在一家三级医疗中心接受IVIG治疗(2g/kg)的70例KD患者。

结果

70例患者中有9例(13%)对初始IVIG治疗无反应。未对IVIG产生反应的患者红细胞沉降率(ESR)更高(104对74mm/h;p值为0.003),发热总天数更长(14.4±3.8对9.2±2.3天;p值为0.003),初始冠状动脉病变(CAL)更多(9例中的7例[77.7%]对61例中的10例[16.3%];p值为0.001),高于对初始治疗有反应的患者。9例接受IVIG(2g/kg)再次治疗的患者中有7例对第二剂有反应。其余2例患者(9例中的2例,22%)持续发热,在两到三剂静脉注射甲基强的松龙脉冲治疗后体温消退。在两个月的随访中,IVIG抵抗性患者经超声心动图检查发现的CAL高于IVIG反应性患者(33%对3.2%,p值小于0.05)。2例IVIG抵抗性KD患者诊断延迟并出现巨大动脉瘤。

结论

ESR高的患者发生IVIG抵抗性KD的风险增加。IVIG抵抗性川崎病患者在急性期和发病后两个月时CAL的患病率更高。

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