Preferred Label : Kawasaki disease;
CISMeF acronym : KD;
Type : Phenotype or locus, molecular basis unknown;
Alternative titles and symbols : Infantile polyarteritis; Mucocutaneous lymph node syndrome; KD;
Description : Kawasaki disease is an acute, self-limited vasculitis of infants and children characterized
by prolonged fever unresponsive to antibiotics, polymorphous skin rash, erythema of
the oral mucosa, lips, and tongue, erythema of the palms and soles, bilateral conjunctival
injection, and cervical lymphadenopathy (Kawasaki, 1967). Coronary artery aneurysms
develop in 15 to 25% of those left untreated (Kato et al., 1975, 1996), making Kawasaki
disease the leading cause of acquired heart disease among children in developed countries.
Treatment with intravenous immunoglobulin (IVIG) abrogates the inflammation in approximately
80% of affected individuals and reduces the aneurysm rate to less than 5%. Cardiac
sequelae of the aneurysms include ischemic heart disease, myocardial infarction, and
sudden death. Epidemiologic features such as seasonality and clustering of cases suggested
an infectious trigger, although no pathogen had been isolated. Several lines of evidence
suggested the importance of genetic factors in disease susceptibility and outcome.
First, the incidence of Kawasaki disease is 10 to 20 times higher in Japan than in
Western countries (Cook et al., 1989). Second, the risk of Kawasaki disease in sibs
of affected children is 10 times higher than in the general population, and the incidence
of Kawasaki disease in children born to parents with a history of Kawasaki disease
is twice as high as that in the general population (Fujita et al., 1989; Uehara et
al., 2003). Hata and Onouchi (2009) reviewed current knowledge on Kawasaki disease,
including epidemiology, genomewide linkage analysis, and molecular genetics.;
Prefixed ID : %611775;
Origin ID : 611775;
UMLS CUI : C0026691;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- DO Cross reference
- False automatic mappings
- ORDO concept(s)
- Semantic type(s)
- UMLS correspondences (same concept)
