Journal
ANNUAL REVIEW OF MEDICINE, VOL 62, 2011
Volume 62, Issue -, Pages 69-77Publisher
ANNUAL REVIEWS
DOI: 10.1146/annurev-med-042409-151944
Keywords
IgA; intracytoplasmic inclusion bodies; ITPKC; coronary artery aneurysms; vasculitis; pediatrics
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Funding
- NHLBI NIH HHS [R01 HL063771-09A1, R01 HL063771, R01 HL063771-10, R01 HL063771-11, R01 HL63771] Funding Source: Medline
- NATIONAL HEART, LUNG, AND BLOOD INSTITUTE [R01HL063771] Funding Source: NIH RePORTER
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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.
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