Study on Etiology and Pathogenesis of Kawasaki Disease

为探讨川崎病(KD)的遗传易感性，检测了川崎病(KD)恢复期患者外周血淋巴细胞(PBL)在PHA和PMA2刺激下产生的肿瘤坏死因子-α(TNF-α)水平。用聚合酶链式反应技术扩增出30%的KD患者血液标本中扩增出65 kDa热休克蛋白基因，从少数急性期KD患者中分离到溶血性链球菌，并在10%的康复期患者中检测到抗结核耶尔森菌抗体。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。…此外，我们没有排除任何证据表明KD是由细小病毒B19引起的。在KD的发病机制研究中，超抗原假说是一个重要的问题。经T细胞增殖试验，31例KD患者培养上清液均为阳性。在16例患者中可检测到已知的超抗原。在剩下的上清液中，我们将提纯具有增殖活性的蛋白质。含有增殖活性的部分刺激了具有特异性T细胞受体的T细胞，但我们不能将KD与新鲜分离的外周血淋巴细胞中表达特异性T细胞受体的T细胞的选择性扩增联系起来。我们观察了Acule KD患者淋巴组织中淋巴细胞的凋亡，但对外周血中T细胞的观察没有提示超抗原刺激T细胞的可能性。电子显微镜下，外周血中可见活化的巨噬细胞。对超抗原可能是病原体的可能性的调查必须是未来研究的主题。较少

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