Subject(s)
Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Coronary Aneurysm/complications , Coronary Vessel Anomalies/diagnostic imaging , Mucocutaneous Lymph Node Syndrome/etiology , Mucocutaneous Lymph Node Syndrome/diagnostic imaging , Time Factors , Echocardiography/methods , Sensitivity and Specificity , Echocardiography, Transesophageal/methods , Coronary Vessels/diagnostic imaging , Drug-Eluting StentsABSTRACT
La enfermedad de Kawasaki es una vasculitis febril, aguda y multisistémica, que afecta, principalmente, a niños menores de 5 años. Se describen las características clínicas, la evolución y las consideraciones terapéuticas en un paciente con diagnóstico de enfermedad de Kawasaki completo con manifestaciones multisistémicas graves, dentro de las cuales se resalta el síndrome de activación de macrófagos, que representa una complicación inusual y potencialmente mortal de la enfermedad
Kawasaki disease is a febrile, acute and multisystemic vasculitis that mainly affects children under 5 years of age. We describe the clinical characteristics, evolution and therapeutic considerations in a patient with a diagnosis of complete Kawasaki disease with severe multisystem manifestations, among which stands out the macrophage activation syndrome, which represents an unusual and potentially life-threatening complication of the illness
Subject(s)
Humans , Male , Child, Preschool , Macrophage Activation Syndrome , Meningitis, Aseptic , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/diagnostic imagingABSTRACT
La enfermedad de Kawasaki es una vasculitis sistémica de vasos de mediano calibre, que afecta principalmente a las arterias coronarias, provocando dilatación y aneurismas coronarios en un 15 - 25% de los casos no tratados. El tratamiento recomendado actualmente es el uso de inmunoglobulina intravenosa antes del décimo día de enfermedad, lo cual reduce el riesgo de aneurismas coronarios de un 20% a un 4%. Objetivos: Determinar la relación entre el tratamiento oportuno con inmunoglobulina y el riesgo de presentar alteración coronaria en la ecocardi ografía en pacientes con Enfermedad de Kawasaki. Métodos: Se revisó la base de datos de Cardiología Infantil del hospital, identificando 66 pacientes que presentaron la enfermedad entre los años 1994 y 2014. Se determinó el momento en que se inició el tratamiento con inmunoglobulina y se analizó el hallazgo ecocardiográfico de alteraciones coronarias durante el período de hospitalización. Resultados: 52 niños (79%) recibieron tratamiento precoz, antes de 10 días del comienzo de la enfermedad; en el resto el tratamiento fue tardío. El tratamiento precoz se asoció a un menor riesgo de presentar aneurismas coronarios (5.8% vs 50% de los casos, p<0.01). Conclusión: En la enfermedad de Kawasaki es de suma importancia el diagnóstico y tratamiento oportuno con inmunoglobulina intravenosa para reducir el riesgo de formación de aneurismas coronarios.
Kawasaki’s disease is a systemic vasculitis of the mediumsized blood vessels, primarily affecting the coronary arteries, causing coronary dilation and aneurysms in 15 - 25% of untreated children. Use of Intravenous immunoglobulin within the first 10 days of illness is the recommended treatment, redugin the risk of coronary artery aneurysm develo-pment from 20% to 4%. Objective: To determine the association of early treatment to risk of echocardiographic coronary changes on patients with the disease. Methods and Results: A review of the institutional Pediatric Cardiology Database, allowed the identification of 66 patients who had Kawasaki’s disease between 1994 and 2014. The echocardiographic findings at follow up were related to the time of treatment initiation in relation to the beginning of the disease. Treatment with im-munoglobulin within 10 days was associated to coronary artery aneurysms in 5.8% of patients, as opposed to 50% in those with a delayed initiation of therapy. Conclusión: Early treatment with immunoglobulins (within 10 days of illness) significantly reduces the short term risk of developing coronary aneurysms in patients with Kawasaki’s disease.
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Coronary Aneurysm/prevention & control , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Mucocutaneous Lymph Node Syndrome/drug therapy , Time Factors , Coronary Aneurysm/diagnostic imaging , Echocardiography , Retrospective Studies , Coronary Disease/prevention & control , Mucocutaneous Lymph Node Syndrome/diagnostic imagingABSTRACT
Coronary artery problems in children usually have a significant impact on both short-term and long-term outcomes. Early and accurate diagnosis, therefore, is crucial but technically challenging due to the small size of the coronary artery, high heart rates, and limited cooperation of children. Coronary artery visibility on CT and MRI in children is considerably improved with recent technical advancements. Consequently, CT and MRI are increasingly used for evaluating various congenital and acquired coronary artery abnormalities in children, such as coronary artery anomalies, aberrant coronary artery anatomy specific to congenital heart disease, Kawasaki disease, Williams syndrome, and cardiac allograft vasculopathy.
Subject(s)
Child , Child, Preschool , Female , Humans , Male , Coronary Angiography/methods , Coronary Vessel Anomalies/diagnostic imaging , Coronary Vessels/diagnostic imaging , Diagnostic Imaging , Echocardiography/methods , Heart Defects, Congenital/diagnostic imaging , Heart Diseases/diagnosis , Heart Rate , Magnetic Resonance Angiography/methods , Mucocutaneous Lymph Node Syndrome/diagnostic imaging , Tomography, X-Ray Computed/methodsABSTRACT
Intravascular ultrasound (IVUS) imaging was performed to assess the coronary wall morphology in detail at 22 months after the onset of Kawasaki disease in a girl who had developed coronary aneurysms at 4 yr of age. The sites of persistent aneurysms demonstrated a dilated lumen with a marked symmetrical or asymmetrical thickening of the intima-media complex. This pathologic finding was also present in angiographically normal vessels near an aneurysm but with a mild thickening. Coronary artery calcification was observed at one site in the aneurysms. The sites of normal coronary artery far from an aneurysm showed normal intravascular ultrasound findings with no measurable intima-media complex. Our case indicates that the healing process may continue via cell proliferation, with extension to the proximity of the coronary aneurysms. IVUS is useful to evaluate the coronary wall morphology and may be valuable in the long-term follow-up of coronary lesions due to Kawasaki disease.