Micophenolate Mofetil (MMF) in the Idiopathic Nephrotic Syndrome (SNI) Resistantance to Different Treatments

RESUMEN: El intento para disminuir los efectos adversos del uso prolongado y de las grandes dosis de esteroides en el tratamiento del síndrome nefrótico idiopático (SNI) corticodependiente o corticoresistente, justifica el uso de inmunosupresores, muchos de los cuales también causan efectos adversos además de producir respuestas variables. El objetivo de este trabajo fue la evaluación retrospectiva de la evolución clínica y de los parámetros de laboratorio en 11 pacientes con SNI (siete corticoresistentes y cuatro corticodependientes), luego de seis meses de tratamiento con MMF. El diagnóstico histopatológico fue glomeruloesclerosis focal y segmentarias (GEFS) en ocho pacientes, glomerulonefritis membranosa (GNM) en dos y glomerulonefritis membranoproliferativa tipo II (GNMP) en uno. El promedio de edad de inicio del SNI fue de 7,8 años (rango 2-14), y el de la edad al inicio del MMF fue de 9,9 años (rango 2,5-16). El MMF se administró en una dosis promedio de 406 mg/m2/12h (rango 186,5-600). Se encontró respuesta parcial al MMF en 4 pacientes y completa en 1; igualmente se observó tendencia a mejor control de la hipertensión arterial y resolución de edemas, manteniendo una función renal estable.ABSTRACT: The attempt to diminish the adverse effects of the prolonged use and the great doses of steroids in the treatment of the idiopathic nephrotic syndrome (SNI) with dependency and/or resistance to steroids, justifies the use of immunosuppressive drug, many of these with variable answers and also adverse effects. The objective of this work was to evaluate the clinical evolution and of the laboratory parameters in the patients with SNI to the 6 months of treatment with Mycophenolate mofetil. The trial included 11 patients with SNI, 7 with resistance and 4 with dependency to steroids, the histopathology diagnosis were: 8 focal and segmental glomeruloesclerosis (GEFS), 2 membranous glomerulonephritis (GNM), 1 membranoproliferative type II glomerulonephritis (GNMP). The average of age of beginning of the idiopathic nephrotic syndrome was 7.8 years (range 2-14), and of the age at the beginning of the MMF range was 9.9 years (2.5-16). Dose average of MMF was: 406 mg/m2/12h (186.5-600). This study shows partial answer to the MMF in 4 patients and completed in 1, also we observed tendency better control of the arterial hypertension, resolution of edemas and maintenance of the stable renal function in all patients

Nutcracker syndrome combined with IgA Nephropathy: a cause of recurrent hematuria and proteinuria. Case report

The nutcracker syndrome is caused by the obstruction of the left renal vein secondary to its compression between the superior mesenteric artery and the aorta. Clinically, this syndrome manifests with pain, hematuria, varicocele or lower urinary tract symptoms. The prevalence of this syndrome is currently unknown; however, the diagnosis of this condition has increased thanks to the availability of non-invasive studies that allow its recognition. On the other hand, IgA nephropathy is the most common type of glomerular disease worldwide. Almost 15% of the causes of hematuria in children are secondary to this condition. The combination of IgA nephropathy and the nutcracker syndrome is rare. In the current literature, few cases have been described. We described the case of a 9-year-old scholar with hematuria and persistent proteinuria in the course of a purpura of Henoch-Schonlein in whom renal biopsy documented IgA nephropathy. However, during follow-up, due to the persistence of hematuria and proteinuria, in addition to other symptoms, additional studies are carried out confirming a Nutcracker syndrome. It is key in patients with persistent symptoms to rule out the association of these two diseases to avoid unnecessary interventions