Kawasaki Disease
Definition
Kawasaki disease is best known for damage to cardiovascular system. It was first described in Japan in 1967. Also known as the mucotutaneous lymph node syndrome it is, currently, a leading cause of acquired heart disease in the United States.
Pathology
The damage involves all the small and medium size blood vessels. Acute stage of this illness involves a progressive inflammation of the small vessels along with pancarditis with eventual progression into the medium sized muscular arteries which can potentially result in the damage to the walls of the vessels. Coronary artery aneurysms may form in about 25% of affected children. The most serious complication of Kawasaki disease is myocardial infarction. The cause of this disease is under investigation, however, analysis of the T-cell receptor suggests that the cause may involve a conventional antigen, such as S. Aureus.
Clinical Manifestations
The progression of the disease may be subdivided into four stages.
Stage I - lasts 1 to 9 days, acute perivasculitis of small arteries is evident, involves the vasa vasorum of major coronaries, pericarditis, interstitial myocarditis, and endocardial inflammation are present.
Stage II - 12 to 25 days duration, panvasculitis of coronaries, affects intima, media, and adventitia, results in aneurysm and thrombosis formation.
Stage III - 28 to 31 days duration, granulating thrombi and intimal thickening cause partial or total occlusion of coronary arteries.
Stage IV - duration of many years, healing occurs consisting of scarring, calcification, and recanalization of occluded arteries.
Management
Initial therapy during the acute stage of the disease is directed at reducing inflammation, especially in the coronary arterial tree and myocardium. Later treatment is directed towards preventing coronary artery thrombosis by inhibiting platelet aggregation. Treatment is done with intravenous Gamma-globulin (IVGG) ans aspirin.
Some children are candidates for percutaneous transluminal coronary angioplasty (PTCA), bypass grafting, and cardiac transplantation.
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