CORONARY ARTERY ABNORMALITIES

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。列出的机构是 中心，不一定是研究者的机构。 川崎（KD）是一种以发热、粘膜皮肤病变和淋巴结病为特征的全身性血管炎疾病，在夏威夷的患病率是美国大陆的三倍：夏威夷每年有45例/100，000名儿童。 尽管KD的发病率很高，但其病因仍不清楚。 KD诊断后两周，冠状动脉异常（CAA）可在高达25%的病例中得到证实。 IVIG治疗的引入使CAA的发病率降至8%。 CAA可能导致心肌缺血，增加KD的发病率和死亡率。 因此，诊断和充分治疗KD对于预防CAA及其长期并发症至关重要。 KD的诊断基于临床特征，但在临床不完全表现中，根据美国心脏协会和美国儿科学会的指南，CAA本身可能是诊断标准的一部分。 检测CAA的金标准方法是二维超声心动图测量三支冠状动脉的直径：左主干冠状动脉（LMCA）、左前降支（LAD）和右冠状动脉（RCA）。 儿童冠状动脉的正常值和异常检测以前是根据日本卫生部制定的标准。 de Zorzi等人根据儿童体表面积引入了正常值和Z评分（正常值的标准差），而不是这些年龄相关值。 他们的研究报告了与KD相关的CAA的高发病率，特别是在疾病的早期发热期。 发热是对感染和炎症过程的一般反应，导致血液循环的重新分布和特定血管的血管舒张。 发热时代谢率的增加需要更高的心输出量，这需要增加冠状动脉循环。 因此，可以假设发热本身可能导致冠状动脉扩张。 什么原因导致冠状动脉的特殊血管炎在发热性疾病，如川崎病尚未确定。 在这项研究中，我们的目的是比较三个冠状动脉的直径在发热和非发热儿童。将发热期住院患者（6个月至6岁儿童）的冠状动脉测量值与既往接受超声心动图检查的健康非发热儿童的冠状动脉测量值进行比较。CAA将根据de Zorzi的标准确定。我们还将确定夏威夷川崎病患者的冠状动脉直径，并将其与de Zorzi的标准化Z评分测量结果进行比较。 如果发热本身可能导致短暂冠状动脉扩张的假设被证明是有效的，则不完全KD的诊断标准可能需要修订。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Kawasaki disease (KD), a general vasculitis illness characterized by fever, mucocutaneous lesions and lymphadenopathy, is three times more prevalent in Hawaii than in the continental United States: 45 cases/100,000 children/year in Hawaii. Despite its high incidence, the etiology of KD is still unknown. Two weeks after diagnosis of KD, coronary artery abnormalities (CAA) can be demonstrated in up to 25% of the cases. The introduction of IVIG therapy had reduced the incidence of CAA to 8%. CAA may lead to myocardial ischemia later in life increasing the morbidity and mortality of KD. Therefore, diagnosing and adequately treating KD is crucial to preventing CAA and its long-term complications. The diagnosis of KD is based on clinical features, but in clinically incomplete presentations, CAA itself may be part of the diagnostic criteria according to the guidelines of the American Heart Association and the American Academy of Pediatrics. The gold standard method of detecting CAA is 2-dimensional echocardiography measuring the diameter of three coronary arteries: left main coronary artery (LMCA), left anterior descending artery (LAD) and right coronary artery (RCA). Normal values of coronary arteries in children and the detection of abnormalities were previously based on criteria constituted by the Japanese Ministry of Health. Instead of these age dependent values, de Zorzi et al introduced normal values and Z-scores (standard deviation from normal) based on the body surface area of children. Their study reported a much higher incidence of CAA associated with KD, especially in the early, febrile period of the illness. Fever, a general response to infectious and inflammatory processes, results in redistribution of blood circulation and vasodilation of specific vessels. Increased metabolic rate in fever demands higher cardiac output that requires increased coronary circulation. Thus, it can be hypothesized that fever itself may result in dilatation of the coronary arteries. What causes the specific vasculitis of the coronary arteries in a febrile illness like KD is yet to be determined. In this study we aim to compare the diameter of three coronary arteries in febrile and non-febrile children. The coronary artery measurements of hospitalized patients (6 months to 6 years old children) in a febrile period of their illness will be compared to coronary artery measurements of healthy non-febrile children, who previously underwent echocardiography. CAA will be determined according to de Zorzi's criteria. We will also determine the diameter of the coronary arteries of patients with Kawasaki disease in Hawaii and compare it to de Zorzi's standardized Z-score measurements. If the hypothesis, that fever itself may result in transient coronary artery dilatation, proves to be valid, the diagnostic criteria of incomplete KD may need to be revised.