Kawasaki disease: novel insights into etiology and genetic susceptibility.
Kawasaki disease (KD) is a vasculitis of young childhood that particularly affects the coronary arteries. Molecular analysis of the oligoclonal IgA response in acute KD led to production of synthetic KD antibodies. These antibodies identify intracytoplasmic inclusion bodies in acute KD tissues. Light and electron microscopic studies indicate that the inclusion bodies are consistent with aggregates of viral proteins and RNA. Advances in molecular genetic analysis and completion of the Human Genome Project have sparked a worldwide effort to identify genes associated with KD. A polymorphism of one such gene, ITPKC, a negative regulator of T cell activation, confers susceptibility to KD in Japanese populations and increases the risk of developing coronary artery abnormalities in both Japanese and U.S. children. Identification of the etiologic agent and of genes conferring KD susceptibility are the best means of improving diagnosis and therapy and enabling prevention of this important disorder of childhood.
Rowley
Anne H
AH
Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA. a-rowley@northwestern.edu
D000906:Antibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000453:epidemiology / Q000235:genetics / Q000276:immunology; D003141:Communicable Diseases / Q000453:epidemiology / Q000235:genetics / Q000276:immunology; D003324:Coronary Artery Disease / Q000235:genetics / Q000276:immunology; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D055106:Genome-Wide Association Study; D015386:Hazardous Substances / Q000009:adverse effects; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D002479:Inclusion Bodies / Q000235:genetics / Q000276:immunology / Q000473:pathology; D007564:Japan / Q000453:epidemiology; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000453:epidemiology / Q000235:genetics* / Q000276:immunology; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000235:genetics / Q000276:immunology; D011110:Polymorphism, Genetic; D015995:Prevalence; D014481:United States / Q000453:epidemiology
D000906:Antibodies; D015386:Hazardous Substances; D007070:Immunoglobulin A; D017853:Phosphotransferases (Alcohol Group Acceptor); C051038:Inositol 1,4,5-trisphosphate 3-kinase