Loomba Rohit S, Raskin Alexander, Gudausky Todd M, Kirkpatrick Edward
Department of Pediatric Cardiology, Children's Hospital of Wisconsin, Milwaukee, WI.
Am J Ther. 2016 Nov/Dec;23(6):e1293-e1299. doi: 10.1097/MJT.0000000000000045.
Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response.
对于川崎病患者，早期静脉注射免疫球蛋白（IVIG）治疗对于降低冠状动脉异常（如冠状动脉瘤）的风险以及缓解症状是必要的。然而，一些患者对初始剂量的IVIG没有反应，需要额外剂量。预测这些IVIG无反应者可能有助于改变初始治疗方案，使其更有效。江见评分已在日本人群中得到验证，可用于预测IVIG无反应者，但在美国人群中显示无效。本研究评估了美国中西部人群中的江见评分，按种族以及典型或非典型川崎病的诊断对患者进行细分。如果患者符合川崎病标准并在住院环境中接受了IVIG治疗，则纳入本研究。总共研究了182例患者，在所有研究组中，江见评分在预测IVIG无反应者方面敏感性较差。该评分的敏感性在不同种族之间以及典型和非典型川崎病之间存在差异。江见评分以及其他系统已被验证可预测IVIG无反应者。然而，这些在美国人群中缺乏敏感性。在美国开发的其他评分也缺乏敏感性，可能是由于没有将种族或川崎病分类作为变量。开发一种敏感的评分系统来预测美国人群中的IVIG无反应者将需要纳入种族和川崎病分类，这些因素似乎会改变IVIG反应。
