RESUMEN
C1q nephropathy is a rare glomerulopathy characterized by mesangial deposition of the complement component C1q. These deposits can be isolated or associated with immunoglobulins or complement fractions, which are observed by immunofluorescence or immunohistochemical microscopy. In ultramicroscopy, dense mesangial deposits and alterations of the podocyte are observed. Clinically it presents as a nephrotic syndrome (NS) or by alterations of the urinalysis such as proteinuria and/or hematuria in children and young adults. In light microscopy, it is expressed with a morphological pattern of minimal change disease (MCD), mesangial proliferative glomerulonephritis or focal segmental glomerulosclerosis (FSGS). The NS during its evolution usually evolve in steroid resistance or steroid dependency, often requiring the association of immunosuppressants to obtain remission. We report a 14 years old male with a history of NS and its evolution under various treatments during a 12-year follow-up.
Asunto(s)
Humanos , Masculino , Adolescente , Complemento C1q/metabolismo , Glomerulonefritis/diagnósticoRESUMEN
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.