Epidemiological features of Kawasaki disease in Korea, 2006–2008

Background: The aim of this study was to estimate the incidence and describe the epidemiological characteristics of Kawasaki disease among children in Korea. Methods: Questionnaires for surveying the epidemiology of Kawasaki disease were distributed to a total of 101 hospitals that conduct pediatric residency programs. Then, we retrospectively obtained the data, which covered a three‐year period (2006–2008) and analyzed them. Results: During the three‐year study period, a total of 9039 cases of Kawasaki disease were reported from 84 hospitals (response rate, 83.2%), comprising 5375 boys and 3664 girls (male : female ratio, 1.47:1). The outbreak rate per 100 000 children <5 years old was 108.7 in 2006, 118.3 in 2007 and 112.5 in 2008 (average rate, 113.1). The seasonal distribution showed a slightly higher incidence rate in winter and summer. The patients’ mean age of onset was 32.6 months, while the proportions of sibling cases and recurrent cases were 0.17% and 2.2%, respectively. Coronary arterial abnormalities were detected during follow up by echocardiogram in 17.5% of all cases including dilatations (16.4%) and aneurysms (2.1%). Conclusions: The average annual incidence rate of Kawasaki disease in Korea has been continuously increasing, and reached 113.1/100 000 children <5 years old, which is the second highest rate in the world.     

Incidence of Kawasaki disease in northern European countries

Background: The aim of the present study was to compare the epidemiologic features of Kawasaki disease (KD) in three northern European countries and Japan. Methods: Data were obtained from discharge databases for hospitals in Finland, Norway, and Sweden for 1999–2009 and from nationwide epidemiologic surveys in Japan for 1998–2008. Annual incidence for each country was calculated using regional census data. Results: During the 11 year period, 1390 KD patients were recorded in the registries of the three northern European countries. Average annual incidence rates per 100 000 children aged <5 years were: Finland, 11.4; Norway, 5.4; and Sweden, 7.4. Overall, 86.4% of Japanese KD patients were aged <5 years compared to only 67.8% in the four northern European countries (P < 0.001). Conclusions: The incidence of KD in northern Europe was constant over the study period and much lower than in Japan. There was a significant age difference between northern European and Japanese KD patients that remains unexplained.     

Nephrotic syndrome in Kawasaki disease

Abstract Renal manifestations in Kawasaki disease are rare, and nephrotic syndrome in this disorder has not been described previously. We report a 3 month old infant with Kawasaki disease, presenting with nephrotic syndrome which responded to steroid therapy. The patient, however, developed coronary aneurysms and died of acute myocardial infarction.     

Ischemic stroke in Kawasaki disease

Pediatric stroke is considered to be rare. Stroke resulting from cerebral vasculitis is also uncommon in young children. With the increasing prevalence of Kawasaki disease (KD) diagnosis, this acquired vasculitis has been reported with various clinical presentations including neurological symptoms. Herein we describe the case of a KD patient presenting with stroke. A 15‐month‐old boy was referred due to stroke that occurred on the fifth day of febrile illness. He was initially admitted to another hospital due to fever and diarrhea. He was discharged and re‐admitted 2 days afterward due to left hemiplegia. During the 10 days of the second hospitalization, he had a presumptive diagnosis of encephalomeningitis. Upon referral to the present hospital, he was found to have right middle cerebral artery branch stenosis and fusiform aneurysms of the coronary arteries. Retrospectively, the patient had the full clinical criteria for KD diagnosis. Therefore, stroke could be considered as one of the uncommon clinical manifestations of KD.     

Absence of hypercoagulability in acute Kawasaki disease

BACKGROUND: Kawasaki disease (KD) is a systemic vasculitis syndrome with the striking feature of cardiovascular involvement. Endothelial cell (EC) damage has been suggested to predispose individuals to the development of coronary vascular disorders. When EC are perturbed, prethrombotic complications ensue. The purpose of this study was to examine the clinical relevance of EC activation and hypercoagulability in the pathogenesis of KD and to determine if plasma levels of these markers are correlated with the development of coronary aneurysms. METHODS: EC function and coagulation status were assessed in 52 patients with acute KD, 20 febrile control subjects, and 20 healthy control subjects. Biological markers of EC and hypercoagulability were measured and included thrombomodulin, tissue factor, tissue factor pathway inhibitor, von Willebrand factor (vWF), coagulation factor VII (FVII), activated factor VII, prothrombin fragment 1 + 2 (F1 + 2), and D-dimer. RESULTS: Transient dilatation of coronary arteries was the most common complication (55.8%), and coronary aneurysm was noted in five patients (9.6%). Levels of vWF, FVII, F1 + 2 and D-dimer were higher in acute KD patients compared with healthy controls but not febrile controls. Markers of EC and hypercoagulability were not different between patients with cardiac complications and those without cardiac complications. Biological and immunological assays did not demonstrate the prethrombotic state in acute KD. CONCLUSIONS: Our results suggest that hypercoagulability does not occur during the acute stage of KD. Markers of EC damage and hypercoagulability are not predictive of coronary aneurysms in KD.     

Kawasaki disease in india

Kawasaki disease was first described 30 years ago. The authors report 5 children with Kawasaki disease who presented within 18-month-period and describe their clinical presentation and management.     

Current issues in Kawasaki disease

The most important clinical aspect of Kawasaki disease is the cardiovascular problems. This article reports on the cardiovascular spectrum in acute Kawasaki disease, analysis of myocardial infarction and fatal cases, a follow-up study on the natural history of coronary artery lesions, long-term cardiovascular problems including premature atherosclerosis, problems in the adult cardiology and the treatment of acute Kawasaki disease. The etiology and pathogenesis of this disease are still unknown. Current hypotheses and leading studies on the etiology and the pathogenesis of Kawasaki disease are also reviewed.     

Kawasaki disease with severe cardiac sequelae: lessons from recent New Zealand experience

OBJECTIVES: To review recent cases of Kawasaki disease (KD) with significant cardiac sequelae in New Zealand. It is known that intravenous immunoglobulin (IVIG) reduces the risk of coronary artery aneurysm formation if given within 8-10 days of onset of KD. METHODS: Retrospective review of medical course, criteria for KD, laboratory and cardiac findings for six children identified with KD and significant coronary artery sequelae. RESULTS: There was delay in diagnosis of KD in three of the six children. Three cases were atypical by extremes of age (2 months, 10 years, 14 years). By definition all six children had significant coronary artery involvement. One patient had a thrombus detected in a coronary aneurysm 3 weeks after KD. One patient underwent coronary artery bypass grafting for unstable angina 2 years after KD. One patient developed coronary artery aneurysms after an initial 'toxic shock' type illness evolving to KD. Three patients died, one due to rupture of a coronary artery aneurysm, two from rapid early coronary artery obstruction occurring at three and 4 months after initial KD. CONCLUSIONS: Kawasaki disease remains an important cause of mortality and morbidity for children. Diagnostic delay beyond 8 days reduces the chances of successful IVIG therapy in KD. Current studies supported by the Paediatric Surveillance Unit should establish the epidemiology of KD in New Zealand.     

A practical approach to refractory Kawasaki disease

Kawasaki disease (KD) is a medium vessel vasculitis and is the most common cause of acquired heart disease in childhood. If left untreated, KD leads to coronary artery aneurysms in 15–25% of patients and the mortality rate in the UK is currently 0.4%. As such, KD is an important preventable cause of heart disease in the young. The aetiology of KD remains unknown, but most likely it represents an aberrant inflammatory host response to one or more as yet unidentified immunological trigger(s) in genetically predisposed individuals. The purpose of this article is not to provide an exhaustive review of KD. Rather we provide practical guidance to the clinical approach to refractory KD. Only brief background on the pathogenesis and epidemiology of KD, and emerging newer clinical trials is provided, to place our clinical approach in context.     

Hemodynamic factors of thrombus formation in coronary aneurysms associated with Kawasaki disease

BACKGROUND: We studied the mechanism of thrombus formation in coronary aneurysms based on rheological findings. METHODS: We studied 43 coronary aneurysms in 33 patients with Kawasaki disease (mean (+/- SD) age 6.1 +/- 4.3 years). These lesions were divided into three groups on the basis of maximum diameter: (i) small (group S); (ii) medium sized (group M); and (iii) large (group L) aneurysms. Using a Doppler flow guidewire and a pressure-monitoring guidewire, we measured coronary flow velocity and perfusion pressure inside aneurysms and in adjacent normal-looking vessels. We calculated the average peak velocity (APV) index, the mean coronary perfusion pressure (P) index and shear index. RESULTS: The APV index and shear index decreased significantly (p < 0.005) as the aneurysm size increased (APV index in groups S, M and L was 0.893 +/- 0.149, 0.573 +/- 0.242 and 0.128 +/- 0.131, respectively; shear index in groups S, M and L was 0.750 +/- 0.149, 0.328 +/- 0.153 and 0.020 +/- 0.028, respectively). However, coronary perfusion pressure showed no relationship to aneurysm size and was not significantly different from that in adjacent normal-looking vessels. CONCLUSIONS: The results of the present study suggest that the stagnation of flow and the reduction of shear stress in coronary aneurysms could initiate thrombus formation.     

上海地区1998－2002年川崎病流行病学特征

目的 对上海地区川崎病发病情况进行调查，了解中国发达地区川崎病的流行病学特征。方法 参照日本流行病学调查方案，制作统一调查表和诊断指南，调查对象为1998年1月1日－2002年12月31日上海地区50家有儿科临床服务的二级及二级以上医院收治的所有川崎病病例。结果 调查表回收率100％，将18例不符合表格填写要求者剔除后，对768例进行分析。上海地区5岁以下儿童川崎病发病率呈逐年增高趋势， 1998年为16.79/10万，1999年为25.65/10万，2000年为28.16/10万，2001年为28.05/10万，2002年为36.76/10万。男女性别比为1.83:1；发病年龄为1个月～18.8岁（中位数1.8岁），发病年龄高峰为9.6个月；全年均可发病，但以春夏之交较多见。主要症状表现为发热持续5 d以上者最为常见（99.3％），其后依次为口唇、口腔黏膜损害（83.5％）、指（趾）端脱皮（82.9％）、皮疹（81.0％）、结膜充血（78.4％）、颈部淋巴结肿大（69.3％）、肢端充血硬肿（48.1％），45.2％的病例有肛周脱皮。心血管损害发生率24.3％，以冠状动脉扩张最为常见，占68％，其次为冠状动脉瘤，占10%。急性期病死率为0.26%，死因为冠状动脉瘤破裂和急性心力衰竭。再发率为1.82%。 结论 上海地区川崎病发病率明显低于日本，但高于西方国家。发病率呈增高趋势，性别分布和心血管损害与其他报道相似。发病季节分布与北京地区的报道相似，但与其他报道不同。     

Neutrophilic involvement in the damage to coronary arteries in acute stage of Kawasaki disease

BACKGROUND: There has been no morphological evidence that polymorphonuclear leukocytes (PMNL) infiltrate the coronary arterial lesions of acute Kawasaki disease (KD) patients, although clinical data indicate the activation of PMNL. METHODS: The experimental materials consisted of eight autopsy patients who died during the acute phase of KD. Duration of the illness ranged from 6 to 32 days. The tissues were fixed and embedded in paraffin. Hematoxylin and eosin, elastica van Gieson and azan-Mallory stainings were performed for routine histological examination. In addition, antibodies to CD3, CD20, CD68 and neutrophil elastase were used for immunohistochemistry to identify infiltrating cells in arterial lesions. RESULTS: The inflammatory cells that appeared in the coronary arterial lesions were mainly composed of macrophages in all patients. In addition, numerous neutrophils were also identified in the coronary arterial lesions of the patients who died 10 days after the onset of KD. Neutrophilic infiltration reached a peak earlier than the peaks of CD68+ macrophages, CD3+ T lymphocytes and CD20+ B lymphocytes. CONCLUSIONS: These results suggest that neutrophils are involved in the damage occurring to coronary arteries in the early stage of KD. Vasodilation might occur as a result of injury to vascular walls caused by neutrophils, as well as macrophages.     

Successful pregnancy after coronary artery bypass grafting for Kawasaki disease

Abstract A 13-year-old girl with a history of Kawasaki disease underwent coronary artery bypass grafting because of angina pectoris due to a giant coronary artery aneurysm on the left main trunk artery. Nine years after the operation, the patient had an uneventful pregnancy followed by a normal vaginal delivery. This is the first case of a successful pregnancy after coronary artery bypass grafting for Kawasaki coronary artery disease.     

An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient

We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three‐yr‐old girl two‐yr status post‐orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies.     

川崎病合并多发体循环动脉病变1例报告并文献复习

目的报告1例川崎病(Kawasaki disease,KD)合并多发体循环动脉病变并复习文献,以提高对该病的认识和临床诊疗水平。方法根据患儿的症状、体征、心脏超声及体动脉B超等检查结果进行诊断,并结合文献资料进行分析。结果女孩,5个月,诊断川崎病合并双侧冠状动脉瘤,符合静脉注射丙种球蛋白(intra-venous immunoglobulin,IVIG)无反应性川崎病诊断标准,体循环动脉B超提示存在多发体动脉病变。结论川崎病合并多发体循环动脉病变较罕见,应提高认识,及时诊断并长期随诊监测病情变化。