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pubmed-article:10558943pubmed:abstractTextKawasaki disease (KD), an acute febrile disease in children of unknown etiology, is characterized by a vasculitis that may result in coronary artery aneurysms (CAAs). In new patients with KD, a selective and prolonged T cell unresponsiveness to activation via the T cell antigen receptor CD3 was observed, whereas proliferation to other stimuli was intact. This "split T cell anergy" delineated KD from other pediatric infections and autoimmune diseases and correlated with CAA formation (P<.001). A transient immune dysfunction was also suggested by an incomplete responsiveness to measles-mumps-rubella (MMR) vaccination in patients with KD versus controls (P<.0001; odds ratio, 15.6; 95% confidence interval, 4.8-51.1), which was overcome by revaccination(s). The reduced responsiveness to MMR in patients with KD suggests a subtle and predetermining immune dysfunction. An inherent immaturity to clear certain antigens may be an important cause that precipitates KD and the immune dysregulation during acute disease.lld:pubmed
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pubmed-article:10558943pubmed:dateRevised2006-11-15lld:pubmed
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pubmed-article:10558943pubmed:articleTitleKawasaki disease: a maturational defect in immune responsiveness.lld:pubmed
pubmed-article:10558943pubmed:affiliationEmma Children's Hospital, Academic Medical Center, 1105 AZ Amsterdam, The Netherlands. T.W.Kuijpers@amc.uva.nllld:pubmed
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