title: Kawasaki disease: four case reports of cardiopathy with an institutional and literature review.
abstract: Kawasaki disease (KD) is a systemic vasculitis condition with a relatively unknown etiology. First described in 1967 by Tomisaku Kawasaki in Japan, KD has come to be widely diagnosed in every region of the world. The disease has a high prevalence in children ages 6 months to 5 years, particularly in those of Japanese descent. Patients often present with a high fever, rash, lymphadenopathy, and conjunctival injections, but there is no diagnostic test for KD. This paper presents data from our Kawasaki registry including 99 patients with emphasis on Kawasaki cardiopathy. Three patients died from complications of KD, and 1 patient underwent heart transplant for massive aneurysmal dilatation. The 4 explanted hearts showed a spectrum of pathological findings (acute thrombosis, vasculitis, and myocarditis), and 1 patient showed the long-term sequelae of vasculitis in the form of massive aneurysmal dilatation. Among the survivors, 30% showed aneurysmal dilatation. This paper reviews the most recent information regarding Kawasaki cardiopathy and underlying molecular mechanisms.
authors.affiliation: Department of Pediatrics, Children's Healtcare of Atlanta, Atlanta, GA 30322, USA.
authors.forename: Amir H
authors.initials: AH
authors.lastname: Ashrafi
authors.forename: Jenny
authors.initials: J
authors.lastname: Wang
authors.forename: Christina A
authors.initials: CA
authors.lastname: Stockwell
authors.forename: David
authors.initials: D
authors.lastname: Lloyd
authors.forename: James B
authors.initials: JB
authors.lastname: McAlvin
authors.forename: Pierre
authors.initials: P
authors.lastname: Russo
authors.forename: Bahig M
authors.initials: BM
authors.lastname: Shehata
mesh_terms: D000328:Adult; D002648:Child; D017809:Fatal Outcome; D005260:Female; D006331:Heart Diseases / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications / Q000473:pathology*