RESUMO
Objetivo: la enfermedad de Kawasaki (EK) es una vasculitis frecuente en la infancia que puede producir aneurismas coronarios. Con el objetivo de describir las lesiones coronarias tanto en fase aguda como al año del diagnóstico, realizamos este estudio retrospectivo de 30 niños de 0 a 7 años con EK atendidos en nuestro centro, desde enero de 1990 a febrero de 2010. Resultados: Presentaron afección coronaria 7 pacientes. Cuatro de ellos varones. El 71,4% (n: 5) tenían EK completo. La edad media de inicio de los síntomas fue de 1,55 años (rango: 0,083-3). La mayoría de las lesiones fueron menores tales como la ectasia difusa o el aneurisma pequeño de ambas coronarias (n: 5). Estables al año de seguimiento. Dos varones de 19 días y 7 meses de edad presentaron lesiones mayores que correspondieron a los aneurismas múltiples y un aneurisma gigante persistente. La duración de la fiebre (p=0,04) y el retraso de aplicación de la gammaglobulina (p=0,025) pudo asociarse con afección coronaria. Conclusión: Alto índice de coronariopatías. La mayoría de las lesiones fueron menores tales como las ectasias difusas o los aneurismas pequeños estables al año de evolución. Las lesiones mayores correspondieron a los aneurismas múltiples y un aneurisma gigante persistente. La duración de la fiebre y el retraso en el tratamiento se asoció con mayor compromiso coronario. Recomendamos al médico mantener un alto índice de sospecha de esta enfermedad particularmente en los niños menores de un año de vida.
Objective: Kawasakis disease (KD) is a systemic vasculitis of childhood with a predilection for the coronary arteries. The aim of this study was to describe the coronary lesions of patients with KD bothin the acute phase and after one year. A retrospective study was performedon 30 children with KD from 0 to 7 years of age treated in ourhospital between January 1990 and February 2010. Results: Coronary artery disease was recorded in 7 patients. Medianage at diagnosis was 1.55 years (range: 0.083-3). There were fourboys, 71.4% (n: 5), with typical disease. Most injuries were minor, diffuse ectasia or small aneurysm of both coronary arteries persistedduring follow-up at one year. Two males of 19 days and 7 months ofage developed multiple aneurysms and giant aneurysm respectively. Duration of fever (p=0.04) and delay of diagnosis and treatment with intravenous gamma globulin (p=0.025) were associated with coronary disease. Conclusion: We found a high percentage of coronary artery abnormalities. Mainly ectasia and small aneurysms persistent at one Year of follow. Major lesions were multiple aneurysms and giantaneurysm that were associated with delayed diagnosis and treatment. We recommend clinicians to mantain high levels of suspicion, particularly in infants under one year of age.
Assuntos
Aneurisma Coronário , Síndrome de Linfonodos MucocutâneosRESUMO
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the presence of autoantibodies against intracellular components, the formation of immune complexes, and inflammation in various organs, typically the skin and kidney glomeruli. The etiology of the disease is not well understood but is most likely the result of the interaction between genetic and environmental factors. In order to identify susceptibility loci for SLE, we have performed genome scans with microsatellite markers covering the whole genome in families from Argentina, Italy, and Europe. The results reveal a heterogeneous disease with different susceptibility loci in different family sets. We have found significant linkage to chromosome 17p12-q11 in the Argentine set of families. The maximum LOD score was given by marker D17S1294 in combination with D17S1293, when assuming a dominant inheritance model (Z = 3.88). We also analyzed a repeat in the promoter region of the NOS2A gene, a strong candidate gene in the region, but no association was found. The locus on chromosome 17 has previously been identified in genetic studies of multiple sclerosis families. Several other interesting regions were found at 1p35, 1q31, 3q26, 5p15, 11q23 and 19q13, confirming previously identified loci for SLE or other autoimmune diseases.