Kawasaki disease (KD) is characterized by a systemic vasculitis of unknown etiology. More research indicates that KD is related to genetic. In 2003, Sekiya et al. studied the correlation of Th2-related genes and the KD in Japan. They found out that -431T allele would increase the concentration of Thymus and activation-regulated chemokine (TARC)/ CCL17 protein in serum by single nucleotide polymorphism (SNP) -431 C>T of chemokine TARC/ CCL17 operon 5¡¦-flanking region , which suggests that SNP has functionality. Therefore, this study explored the polymorphism and relationship between the regulation of chemokine of TARC/ CCL17 and KD. Firstly, we performed polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) to detect TARC/CCL17 -431 C>T genotype. Then enzyme immunoassay was used to detect TARC/CCL17 chemokine¡¦s expression. The results showed that the performance of TARC -431 C/T SNP, the alleles from KD patients with -431 T, were significantly less than the non-KD control group. It was observed that the -431 T alleles had a lower chance to occur in KD with aneurysms, but independent with coronary artery lesions (CAL). In addition, the acute stage of KD has a higher TARC protein expression, which gradually decreases during IVIG treatment period. However, the up-regulation of TARC protein may not be the direct consequence caused by the single nucleotide polymorphism of TARC -431 C>T.

Background: Kawasaki disease (KD) affects mainly children younger than five years of age, leading to coronary artery lesions, and even to life-threatening myocardial infarctions. Since 1976, Kawasaki disease has occurred among thousands of children in Taiwan. Evidence suggests that inflammation plays a key role in the pathogenesis of atherosclerosis. Significant determinants of high sensitivity C-reactive protein (hs-CRP), which is a sensitive indicator of inflammation, as well as white blood cell (WBC) count, and high-density lipoprotein cholesterol (HDLc) and coronary artery lesion were identified. The relationships between these factors¡¦ concentration and arterial lesion were likewise investigated and had reported. The aim of this study was to determine the serum levels of the hs-CRP, WBC count, and plasma HDLc levels in patients with later phase of KD. Methods and Materials: From July 2005 to June 2006, 97 children with Kawasaki disease at least 1 year after diagnosis were recruited in this study. These participated children had been diagnosed as KD and collected at the interval of 2001 to 2004. Diagnosis was based on the 1984 revised by the KD Research Committee in Japan. The participants were grouped into 45 patients with KD and coronary aneurysms (Group I), 52 patients with KD and normal coronary arteries (Group II), and 50 healthy age-matched children (Control Group III). Their WBC count, systemic and diastolic blood pressures, body mass index, age, sex, fasting total cholesterol concentrations, triglyceride, high-density lipoprotein cholesterol (HDLc), low-density lipoprotein cholesterol, serum hs-CRP levels, and coronary artery lesion by cardioechography were recorded and compared. The analytical differences between hs-CRP, WBC count, and plasma HDLc levels and the coronary artery events in KD were examined. Results: Serum hs-CRP levels of Group I patients (mean 0.264 mg/dl) was significantly greater than that of Group II (mean 0.155 mg/dl, p=0.006) and Group III patients (mean 0.116 mg/dl, p =0.017). Similarly, the WBC count of Group I patients (mean 6,543.11/mm3) was significantly greater than that of Group II (mean 5,720.19/mm3, p=0.029), and Group III patients (mean 5,611.27/mm3, p =0.012). However, plasma HDLc levels of Group I patients (mean 41.42 mg/dl) was significantly lesser than that of Group II (mean 44.79 mg/dl, p=0.035), and Control Group III patients (mean 46.58 mg/dl, p=0.027). There was a positive association between hs-CRP and WBC count levels (r = 0.641, p < 0.05), but none between hs-CRP and plasma HDLc levels. Conclusions: There is the possibility of ongoing low-grade inflammation late after the convalescent phase of Kawasaki disease in children with coronary aneurysms, which may have a role in increasing coronary artery dysfunction. These results also suggest that hs-CRP, WBC count, and plasma HDLc levels are useful parameters for predicting formation of coronary artery lesion even in children after onset of KD.

INTRODUCTION: With the emergence of the COVID 19 pandemic, a new disease, Multisystem Inflammatory Syndrome in Children (MIS-C), had evolved. Increasing number of children are being reported to have MIS-C in the U.S. & worldwide. In the U.S. there are currently 2617 MISC cases reported. MIS-C & Kawasaki Disease (KD), have almost the same presentation, making clinical differentiation difficult. This study aims at differentiating KD & MIS-C which could assist clinicians to determine which one they could be dealing with in their practices. METHODS: Clinical features & laboratory values were collected from published studies found by queries on PubMed & other websites. Reported values were selected from published systemic reviews, meta-analyses, & large retrospective chart studies. RESULTS: In KD, the most prevalent clinical features are fever (100%) & the 5 KD-defining clinical features: oral mucosal changes (96.5%), rash (96%), non-purulent conjunctivitis (89%), extremity changes (75.6%), and cervical lymphadenopathy (62.7%). MIS-C also presents with fever (100%) but has lower prevalence of oral mucosal changes (23%), rash (38.2%), non-purulent conjunctivitis (44.0%), extremity changes (2.5%), & cervical lymphadenopathy (4%). MIS-C leads to higher rates of ventricular dysfunction (39.3%), myocarditis (23%), & shock. For cardiac biomarkers, MIS-C has elevated troponin I (x6 normal) & Beta Natriuretic Peptide (BNP) (x414 normal), while KD has elevations of troponin I (x1.9 normal) & BNP (x15 normal). MIS-C has higher elevations in ESR, CRP, and D-Dimer at x6, x30, and x40 from the normal values, respectively, while KD has elevations of x2.8, x2.1, x7.3 from the normal values, respectively. MIS-C is associated with neutrophilia, thrombocytopenia, & anemia in 22% of cases. KD is associated with mild neutrophilia & anemia. KD has thrombocytosis in the subacute phase (x1.46 normal). CONCLUSION: Our results demonstrated that there are overlaps & differences in clinical and laboratory features. Fever is present in both KD & MIS-C, however the 5 KD defining clinical features of KD are less frequent in MIS-C. MIS-C induces higher levels of troponin I & BNP, findings that could potentially explain for higher rates of ventricular dysfunction & myocarditis. MIS-C causes higher elevations in inflammatory markers & D-Dimers compared to KD. Uniquely, thrombocytopenia is commonly present in MISC rather than in KD. Differentiating KD & MIS-C can be challenging, but by focusing closely on the clinical & laboratory features, clinicians may be able to distinguish between the two &, therefore, deliver the most appropriate care to patients in their practices.

京都大学 / 0048 / 新制・課程博士 / 博士(社会健康医学) / 甲第21456号 / 社医博第90号 / 新制||社医||10(附属図書館) / 京都大学大学院医学研究科社会健康医学系専攻 / (主査)教授 滝田 順子, 教授 小杉 眞司, 教授 三森 経世 / 学位規則第4条第1項該当 / Doctor of Public Health / Kyoto University / DFAM

Mucocutaneous lymph node syndrome or Kawasaki syndrome is very severe disease.The most common symptom includes a high fever which is probably due to inflammation of blood vessels so-called vasculitis. . Several months old babies to preschool children are the most often affected group of patients. Specific and typical symptoms of Kawasaki disease include long term fever, conjunctivitis, erythema, lymphadenopathy, mucosal changes as red swollen lips and strawberry tongue and multiple rashes.Qualitative and quantitative research was applied in the diploma thesis "Kawasaki disease in the contemporary society through nurse´s eyes". Three basic objectives and three research questions were established in this diploma thesis.

Mucocutaneous lymph node syndrome or Kawasaki syndrome is very severe disease. The most common symptom includes a high fever which is probably due to inflammation of blood vessels so-called vasculitis. The characteristic statement is that the etiology of disease remains unknown and the origin is not clarified. Several months old babies to preschool children are the most often affected group of patients. It is relatively rare, modern and mystery disease in contemporary and industrial developed society. The first appearance of this disorder is in 20th century. The disorder was first described by well-known Tokyo origin pediatrician Tomisaku Kawasaki in Japan, who studied this disease very thoroughly. This rare disease is considered autoimmune in origin triggering by an infectious agent especially in those who are genetically predisposed. Specific and typical symptoms of Kawasaki disease include long term fever, conjunctivitis, erythema,lymphadenopathy, mucosal changes as red swollen lips and strawberry tongue and multiple rashes. Related cardiovascular complications should be pointed out especially coronary or other major arteries aneurysms and their ruptures, pericardial effusion, heart inflammations, coronary thrombosis, pericardial exudates, arrhythmias, or mitral valve disease. There is no specific test for identification of Kawasaki disease despite the contemporary technological and diagnostic options. So, the easiest way to establish diagnosis is to recognize typical symptoms, blood/urine/spinal fluid testing and then performing X-ray, electrocardiogram and echocardiogram. This disease has very low mortality, the short-term prognosis is excellent and relapse of symptoms is rare. In total, 337 children were diagnosed with Kawasaki disease and admitted to hospital during the evaluation period (2007-2015) in the Czech Republic. This is significantly lower incidence comparing to the other countries in the world. Diploma thesis "Kawasaki syndrome in contemporary society from a nurse´s point of view" was designed as theoretical with a supplement of short case study. The goal of presence of this case study is to better comprehend presented topic. The scope of problem is described from theoretical point of view in partial chapters of this diploma thesis. All the information mentioned in this thesis quotes verified sources, publications written by the Czech and foreign specialists in this particular field. The goal of the thesis was to describe, based on available literature, the issue of Kawasaki disease in children focused on specifics of nursing. All the information was searched in bibliographical issued writings, in databases, or on the Internet. Based on the goal determined in advance, scientific methods such as explanation, analysis, synthesis and demonstration of data were chosen for composing this diploma thesis. The output of theoretical work is to present complex view on the issue of Kawasaki syndrome particularly for non-medical professionals. It came out that the profession of nurse has its own irreplaceable place in the context of Kawasaki disease. As well as competences of nurse are essential. These competences are provided for improving quality of life of children with this unfamiliar and life-threatening disease.