Study on Etiology and Pathogenesis of Kawasaki Disease

为了研究川崎病（KD）的遗传易感性，在PHA和PMA存在的情况下，检测外周血淋巴细胞（PBL）中TNF-a的产生。来自恢复期KD患者和同胞的PBL比没有KD的对照组产生更高的tnf - α。HLA DNA分型显示DPB1^<**>0202和DPB1^<**>0601可能与KD有关。这些结果提示KD可能受遗传因素控制。用聚合酶链反应（PCR）技术在30%的患者血样中扩增出65 kDa热休克蛋白基因，在少数急性KD患者中分离出溶血性链球菌，在10%的恢复期患者中检出抗假结核耶尔森菌抗体。进一步的研究需要澄清KD与这些微生物的关系。急性期未检测到细小病毒B19基因，恢复期未检测到抗细小病毒B19抗体。我们没有找到任何证据表明KD是由细小病毒B19引起的。在KD发病机制的研究中，超抗原假说是一个重要的问题。采用T细胞增殖试验，31例KD患者均分离到阳性上清液。已知的超级抗原可在16例患者中检测到。在剩下的上清液中，我们将纯化具有增殖活性的蛋白质。含有增殖活性的部分刺激携带特定T细胞受体的T细胞，但我们不能将KD与新分离的外周血淋巴细胞中表达T细胞的特定T细胞受体的选择性扩增联系起来。我们观察到急性肾病患者淋巴结淋巴细胞凋亡，但外周血T细胞观察未发现超抗原刺激T细胞的可能性。电镜下观察外周血巨噬细胞活化。对超抗原作为致病因子的可能性的调查必须是今后研究的主题。少

To investigate genetic susceptibility to Kawasaki disease (KD), peripheral blood lymphocytes (PBL) were tested for TNF-a production in the presence of PHA and PMA.PBL from patients with convalescent KD and the compatriots produced higher amount of TNF-alpha than those from controls without KD.And HLA DNA typing revealed that DPB1^<**>0202 and DPB1^<**>0601 may relate to KD.These results suggested that KD may be controlled under the genetic factors.Mycobacterium 65 kDa heat shock protein gene were amplified using polymerase chain reaction (PCR) technique in 30% of blood samples from patients and Streptococcus haemolyticus were isolated from a few percents of patients with acute KD and anti-Yersinia pseudotuberculosis-antibody raised in 10% of convalescent patients. Furthermore studies are needed to clarify the relation of KD to those microorganisms.Parvovirus B19 gene was not detected using PCR technique in acute phase and anti-parvovirus B19 antibody did not rise in convalescent phase. … More We failed to fined any evidence that KD is caused by parvovirus B19.In study on pathogenesis of KD,superantigen hypothesis is important issue. Using T cell proliferation assay, supernatants positive were isolated from all 31 KD patients. Known superantigens could be detected in 16 patients. In the remaining supernatants, we are going to purify the protein which have proliferative activity. A fraction containing proliferative activity stimulated T cells bearing the specific T cell receptor but we were not able to relate KD to selective expansion of specific T cell receptor expressing T cells in freshly isolated peripheral blood lymphocytes. We observed apoptosis of lymphocytes in lymph node from acule KD patients but observation of T cells in peripheral blood did not indicate the possibility that T cells may be stimulated with superantigen. And with electron microscope activated macrophages were found in peripheral blood. The investigation of the possibility that superantigen may be the causative agents must be the subject of future research. Less