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. Ligh...

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Bibliographic Details
Published in:Annual review of medicine 2011-01, Vol.62 (1), p.69-77
Main Author: Rowley, Anne H
Format: Article
Language:English
Subjects:
Antibodies - immunology
Autoimmune diseases
Autoimmune Diseases - epidemiology
Autoimmune Diseases - genetics
Autoimmune Diseases - immunology
Childrens health
Communicable Diseases - epidemiology
Communicable Diseases - genetics
Communicable Diseases - immunology
Coronary Artery Disease - genetics
Coronary Artery Disease - immunology
Disease prevention
Genetic disorders
Genetic Predisposition to Disease - genetics
Genome-Wide Association Study
Hazardous Substances - adverse effects
Humans
Immunoglobulin A - immunology
Inclusion Bodies - genetics
Inclusion Bodies - immunology
Inclusion Bodies - pathology
Japan - epidemiology
Lymphatic system
Male
Mucocutaneous Lymph Node Syndrome - epidemiology
Mucocutaneous Lymph Node Syndrome - genetics
Mucocutaneous Lymph Node Syndrome - immunology
Pediatrics
Phosphotransferases (Alcohol Group Acceptor) - genetics
Phosphotransferases (Alcohol Group Acceptor) - immunology
Polymorphism
Polymorphism, Genetic
Prevalence
T cell receptors
United States - epidemiology
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Summary: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.
ISSN:0066-4219
1545-326X
DOI:10.1146/annurev-med-042409-151944