Minggu, 09 November 2008

KAWASAKI SYNDROME

Kawasaki syndrome is a worldwide multisystemic disease initially described by Tomisaku Kawasaki in 1967. It is also known as the "mucocutaneous lymph node syndrome." It occurs mainly in children under age 5 but occasionally in adults, at times in epidemic fashion. Asian children are at higher risk. The epidemiology suggests an infectious origin, though no agent has been identified. While disease is probably not mediated by a bacterial toxin, a staphylococcal toxin may serve as a "superantigen" that interacts with T cells. IgA plasma cell infiltration is noted in the visceral organs, lungs, and coronary arteries of Kawasaki syndrome patients.

The disease is characterized by fever and four of the following for at least 5 days: bilateral nonexudative conjunctivitis, mucous membrane changes of at least one type (injected pharynx, cracked lips, strawberry tongue), extremity changes of at least one type (edema, desquamation, erythema), a polymorphous rash, and cervical lymphadenopathy greater than 1.5 cm.

A major complication is arteritis of the coronary vessels, occurring in about 25% of untreated cases and on occasion causing myocardial infarction. Noninvasive diagnosis can be made with magnetic resonance angiography or transthoracic ultrasound. Factors associated with the development of coronary artery aneurysms are leukocytosis and elevated C-reactive protein. Arteritis of extremity vessels and peripheral gangrene are also reported. Cerebrospinal fluid pleocytosis is reported in one-third of cases. The cause of these complications is also unknown. Differentiation from disseminated adenovirus infection is important and may be facilitated in the future with rapid adenovirus assays.

Management is with aspirin (80-100 mg/kg/d in divided doses with subsequent tapering) and intravenous immune globulin, 2 g/kg over 10 hours. Plasmapheresis may be useful in the up to 10% of cases that are unresponsive to immune globulin. Corticosteroids are used by some in refractory disease. Their role in increasing the likelihood of the development of coronary aneurysms is controversial. Aspirin is used for patients with persisting coronary artery aneurysms, while warfarin is indicated for aneurysms larger than 8 mm in diameter. Regular follow-up by a cardiologist is recommended for patients with coronary artery disease or aneurysms. Success is reported with interventional catheter treatment, including stent implantation in patients with long-term cardiac complications.

Barron KS: Kawasaki disease: etiology, pathogenesis, and treatment. Cleve Clin J Med 2002;69 (Suppl 2):SII69. [PMID: 12086269]
Blanchard JN et al: Recurrent Kawasaki disease-like syndrome in a patient with acquired immunodeficiency syndrome. Clin Infect Dis 2003;36:105. [PMID: 12491210]
Brogan PA et al: Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. Arch Dis Child. 2002;86:286. [PMID: 11919108]
Chang RK: Hospitalizations for Kawasaki disease among children in the United States, 1988-1997. Pediatrics 2002;109:e87. [PMID: 12042581]
Mason WH et al: Kawasaki syndrome. Clin Infect Dis 1999;28:169. [PMID: 10064222]
Document Bibliographic Information:
Location In Book:
CURRENT MEDICAL DIAGNOSIS & TREATMENT - 43rd Ed. (2004)
Infectious Diseases: Viral & Rickettsial - Samuel Shelburne III, MD, & Wayne X. Shandera, MD
VIRAL DISEASES