Revista chilena de pediatría
versión impresa ISSN 0370-4106
BANFI P., Antonio. Kawasaki´s disease. Rev. chil. pediatr. [online]. 2001, vol.72, n.6, pp.487-495. ISSN 0370-4106. http://dx.doi.org/10.4067/S0370-41062001000600002.
Kawasaki´s disease (KD) has been of epidemiological, clinical and laboratory interest since its original description in 1967. The cause of the disease has not been identified but based on epidemiological and clinical evidence the hypothesis is of an infective aetiology. The evidence until now suggests that it is caused by bacterial toxins acting as super-antigens which stimulate T lymphocytes to express the variable gene segments of the T-cell receptor. Certain toxins elaborated by Staphylococcus aureus and Streptococcus pyogenes have properties of super-antigens, and some studies have demonstrated evidence of their role in the pathology of KD. From the pathogenic point of view, the failure to consistently identify an infectious agent along with and anatomopathological and laboratorial evidence of cellular and humoral immunological activation, tend to put KD in the category of autoimmune disease. One of the main problems is that the delay in diagnosis correlates with a poorer prognosis in terms of the sequelaes of the disease, among which coronary artery involvement is highlighted. In the western world it exists the task of implementing an active supervision for KD, which has become the leading cause of acquired cardiopathy, displacing rheumatic heart disease. In Chile it is possible to conceive a notification system for KD taking into account the public and private health care networks of our national health services. Thus an important data base for paedriatric and adult patients could be built. The central dilemma in paedriatrics is the inexistence of a diagnostic test and the possibility of severe sequelae if an opportune diagnosis and early treatment are not instigated. Coronary epithelial damage is the major question in the long term follow up of KD owing to the presence of ischaemic events, myocardial infarction and sudden death in young adults with a history of KD but without aneurysms. It raises the possibility of an accelerated atherosclerosis. Since 15 years ago it exists an effective treatment available, intravenous immumnoglobulin (IVIG) whose largest contribution is the significant reduction in the incidence of coronary aneurysms when administered in the first 10 days. Combined with aspirin it is an effective treatment in the majority of cases. In refractory cases one should prove other drugs that could reduce vascular damage e.g. steroids, pentoxifiline, ulinastatin. For the paedriatrican KD represents a challenge and a delicate management problem, seeing necessary to establish a protocol of observation and follow-up of this disease in Chile. In our judgment the complexity of this problem requires an integrated force of the national hospital services and Health Authorities in order to obtain accurate records, an adequate follow-up and the transference of clinical records to general medicine when the patient become an adult. These measures could put in our grasp the necessary elements in order to manage a problem of undoubted importance
Palabras clave : Kawasaki disease; paedriatrics; coronary aneurysms; miocardial infarct; IVIG; inmunoglobulin.