Enfermedad de Kawasaki

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Abstract

Resumen La enfermedad de Kawasaki es un estado febril agudo, cuya incidencia es de 175 por cada 100.000 niños menores de 5 años. Es una vasculitis con predilección por los vasos de pequeño y mediano calibre, especialmente por las arterias coronarias; sin tratamiento pueden desarrollarse aneurismas coronarios que conllevan riesgo de infarto agudo de miocardio y muerte súbita. Se describe el caso de un niño de 4 años a quien se diagnosticó enfermedad de Kawasaki y aneurismas fusiformes moderados en las arterias coronarias derecha e izquierda. En control a los dos meses desarrolló nuevo cuadro febril con hipercolesterolemia, trombocitosis y alteración de las pruebas hepáticas. En ecocardiograma de control se evidenció aneurismagigante en la coronaria izquierda y mediante angiografía coronaria se detectaron dos aneurismas en la coronaria derecha. En vista de que se consideró un caso de evolución atípica, se realizó revisión de la literatura mundial en enfermedad de Kawasaki y se recomendó diagnóstico y tratamiento precoz para disminuir la morbilidad con compromiso cardiovascular severo y/o la mortalidad.

Translated abstract

Abstract Kawasaki disease is an acute febrile state with an incidence of 175 per 100,000 children under the age of 5. It is a vasculitis with a preference for small and mediumsized vessels, especially for coronary arteries. If untreated, coronary aneurysms may pose the risk of acute myocardial infarction and sudden death. The case of a 4 year-old child who was diagnosed of Kawasaki disease and moderate fusiform aneurysms in the right and left coronary arteries is described. Follow-up visit after two months revealed fever again with hypercholesterolemia, thrombocytosis and abnormal liver testing. Control echocardiogram evidenced a giant aneurysm in the left coronary and a coronary angiography detected two additional aneurysms in the right coronary. As this was considered an atypical progress of the condition, review of global Kawasaki disease literature was carried out and early diagnosis and treatment were recommended to reduce morbidity with severe cardiovascular involvement and/or mortality.

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Most cited references 13

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Guidelines for medical treatment of acute Kawasaki disease: report of the Research Committee of the Japanese Society of Pediatric Cardiology and Cardiac Surgery (2012 revised version).

(2014)

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Pathogenesis and management of Kawasaki disease.

Anne Rowley, Stanford T. Shulman (2010)

Kawasaki disease (KD) is an acute systemic inflammatory illness of young children that can result in coronary artery aneurysms, myocardial infarction and sudden death in previously healthy children. Clinical and epidemiologic features support an infectious cause, but the etiology remains unknown four decades after KD was first identified by Tomisaku Kawasaki. Finding the cause of KD is a pediatric research priority. We review the unique immunopathology of KD and describe the current treatment. New research has led to identification of viral-like cytoplasmic inclusion bodies in acute KD tissues; this finding could lead to identification of the elusive etiologic agent and result in significant advances in KD diagnosis and treatment. Current management of acute KD is based upon prospective, multicenter treatment trials of intravenous immunoglobulin (IVIG) with high-dose aspirin. Optimal therapy is 2 g/kg IVIG with high-dose aspirin as soon as possible after diagnosis during the acute febrile phase of illness, followed by low-dose aspirin until follow-up echocardiograms indicate a lack of coronary abnormalities. The addition of one dose of intravenous pulse steroid has not been shown to be beneficial. For the 10-15% of patients with refractory KD, few controlled data are available. Options include repeat IVIG (our preference), a 3-day course of intravenous pulse methylprednisolone, or infliximab (Remicade). Patients with mild-to-moderate coronary abnormalities should receive an antiplatelet agent such as low-dose aspirin (3-5 mg/kg/day) or clopidogrel (1 mg/kg/day up to 75 mg), and those with giant (approximately 8 mm diameter) or multiple coronary aneurysms should receive an antiplatelet agent with an anticoagulant such as warfarin or low-molecular-weight heparin. Acute coronary obstruction requires acute thrombolytic therapy with a surgical or percutaneous interventional procedure.

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N-terminal pro-brain natriuretic peptide and risk of coronary artery lesions and resistance to intravenous immunoglobulin in Kawasaki disease.

Ken Yoshimura, Takahisa Kimata, Kenji Mine … (2013)

To determine whether the serum N-terminal pro-brain natriuretic peptide (NT-proBNP) can be a useful marker not only to identify the patients with Kawasaki disease (KD) who are at a higher risk of developing coronary artery lesions (CAL), and predict resistance to intravenous immunoglobulin (IVIG).