Terapia con micofelonato de mofetilo en niños con síndrome nefrótico idiopático córtico resistente

INTRODUCCION: La mayoria de los ninos con sindrome nefrotico (SN) responde a tratamiento corticoesteroideo, aunque 15% de los casos son cortico resistentes (CR), y esto constituye un reto clinico. OBJETIVO: Evaluar la respuesta del SNCR al tratamiento inmunosupresor con Micofenolato de Mofetilo (MMF) METODOS: Trabajo prospectivo, incluye 42 pacientes, edades de 3.2 +/- 4.4 anos. Tratamiento previo: 8-12 semanas con prednisona, demostrandose cortico-resistencia. Se administro MMF 600 mgs/m²/dia/ 6-18 meses y prednisona 1-2 mg/kg, con reduccion progresiva en esquema piramidal. Se realizo control clinico periodico y cuantificacion de proteinuria, creatinina, proteinas totales y fraccionadas, colesterol y trigliceridos, cada 1-3 meses. Se practico biopsia renal percutanea en 37 pacientes previamente a iniciar MMF. RESULTADOS: 27 casos (64%) tuvieron remision completa, 9 remision parcial y 6 no respondieron. Promedio de seguimiento: 9.2 ± 4.8 meses. 26 pacientes que completaron 18 meses de tratamiento mostraron, despues de seguimiento adicional de 4.2 meses, que 18 (69%) mantenian su remision y 8 (31%) presentaron recaida completa o parcial. Los casos que remitieron totalmente, presentaron lesiones glomerulares minimas o lesiones mesangiales en 22 casos (85%); 67% de los 9 casos con remision parcial mostro lesiones mesangiales y los 6 casos que no respondieron a la terapia mostraron lesiones complejas: 4 glomeruloesclerosis focal y segmentaria, 1 nefropatia membranosa y 1glomerulonefritis mesangioproliferativa. CONCLUSIONES: este trabajo muestra que el MMF es una buena alternativa para el tratamiento de ninos con SNCR lograndose remision completa o parcial en 86% de los casos, con mantenimiento de la remision en el 70% de los pacientes. INTRODUCTION: The majority of children with idiopathic nephrotic syndrome respond to steroid therapy; however, approximately 15% of cases are steroid resistant, and this is a clinical challenge. OBJECTIVE: To evaluate the response to Mophetyl Mycophenolate (MMF) in children with steroid resistant nephrotic syndrome (SRNS). METHODS: We recruited 42 children with SRNS, ages 3.2 +/- 4.4 years. All patients received at least one course of 8-12 weeks of prednisone with no response; renal biopsy was performed in 37/42 cases. Dose of MMF was 600 mgs/m²/ day for 6-18 months and prednisone was reduced stepwise. Patients were evaluated clinically periodically, and proteinuria, serum creatinine, serum proteins, cholesterol and triglycerides were checked every 1-3 months. RESULTS: 27(64%) patients had complete remission, 9(22%) partial remission and 6(14%) no response. Mean follow up was 9.2 +/4.8 months. Of the responders 85% had either minimal change disease or mesangial nephritis (MGN) (22 cases); all but one of the partially responders had MGN and those with no response corresponded to 4 cases with focal and segmental glomerulosclerosis (FGS), 1 membranous nephropathy and 1 MGN. Evaluation of 26 patients who completed 18 months treatment showed, after an additional 4. 2 months period of follow up, that 18(69%) maintained their remission and 8 relapsed (31%). CONCLUSIONS: This report shows that MMF is a good therapeutic alternative for SRNS, achieving either a complete or partial remission in the majority of cases (86%) and stabilization of the remission in almost 70% of them.