Retos terapéuticos en el síndrome nefrótico idiopático / Therapeutic challenges in the idiopathic nephrotic syndrome

El diagnóstico, evaluación y tratamiento del síndrome nefrótico en el niño data de los tiempos de Hipócrates. Sin embargo, en la actualidad algunos pacientes con la enfermedad siguen siendo un reto terapéutico para el médico. Nos proponemos examinar en la literatura reciente, distintas propuestas o protocolos de tratamiento y las nuevas drogas que pueden utilizarse en la atención al paciente con esta enfermedad. Los protocolos de tratamiento varían con relativa frecuencia y las drogas, tanto en los síndromes nefróticos recaedores frecuentes, corticodependientes o corticorresistentes, no garantizan en muchas ocasiones la curación del paciente. Cuando fracasan los esteroides se pueden utilizar agentes alquilantes, inhibidores de la calcineurina, antiproliferativos, anticuerpos monoclonales y otros fármacos, pero existen pacientes que no tienen remisión de la proteinuria con ninguno de estos tratamientos. Por sus características evolutivas, algunos pacientes con síndrome nefrótico idiopático siguen siendo un reto para el médico que trata de evitar su evolución hacia la pérdida de la función renal. A pesar de todos los avances en la atención del niño con síndrome nefrótico, desde el descubrimiento de los esteroides, antibióticos, diuréticos e inmunosupresores, en la actualidad no sabemos exactamente cuál es el mejor tratamiento en las formas resistentes del síndrome nefrótico idiopático en niños(AU)

Diagnosis, evaluation and treatment of nephrotic syndrome in children dates from Hypocrate times. However, nowadays some patients with this disease are still a therapeutic challenge for physicians. The aim of this work is to search in recent literature different proposals or treatment protocols, and new drugs that can be used in the care of patients with this disease. Treatment protocols vary with relative frequency and drugs, as well as in frequent relapsing nephrotic, corticodependent or corticoresistant syndromes, do not guarantee in many ocassions the cure of the patient. When steroids fail, alkylanting agents, calcineurin depressants, antispreading, monoclonal antibodies and other drugs can be ussed; but there are patients who do not have remission of proteinuria with any of these treatments. Due to their evolutive characteristics, some patients with idiopathic nephrotic syndrome are still a challenge for the physicians who try to avoid its evolution toward the loss of renal function. Although all the advances in the care of children with nephrotic syndrome due to the discover of steroids, antibiotics, diuretics and immunosupressive drugs, nowadays we do not exactly know which is the best treatment for the resistant types of idiopathic nephrotic syndrome in children(AU)

Relative safety of steroid sparing agents in frequently relapsing, steroid dependent and steroid resistant nephrotic syndrome in pediatric patients

Background: In patients with frequently relapsing nephrotic syndrome (FRNS), steroid-dependent nephrotic syndrome (SDNS) and steroid resistant nephrotic syndrome (SRNS) steroids are either used for prolonged period of time or ineffective. To reduce the degree of steroid dependency and avoid steroid toxicity, several immunosuppressive steroid sparing agents (SPAs) have been proposed to treat these children. The present study tried to study the relative safety of most commonly steroid sparing agent in such children.Methods: A multi-centred, prospective observational study was conducted in paediatric nephrology OPD of two tertiary care hospitals in Kolkata over a period of 24 months. All consecutive children with diagnosed FRNS, SDNS and SRNS who were started on steroid sparing agents were enrolled and followed up for at least 6 months. Records of clinical examination, laboratory tests were collected and measured at the baseline and regular intervals. Safety parameters were noted and statistically analysed.Results: A total 110 patients were screened, examined and enrolled. Levamisole, cyclophosphamide and MMF were commonly used SPAs. Of the two tertiary care hospitals, all the patients of FRNS and SDNS were started with levamisole and SRNS with cyclophosphamide in one set-up, while in the other hospital some SDNS patients were started with cyclophosphamide and SRNS with MMF but without clinically significant outcomes. In comparison with few minor adverse events in MMF group, some serious adverse events were documented in the both cyclophosphamide and levamisole groups.Conclusions: Levamisole being a very efficacious, safe and easily affordable drug, should be used as an initial first line SPA in treating FRNS and SDNS children. The side effect profiles of levamisole and MMF are much more patient friendly.

Beneficial effects of cyclophosphamide on the relapse rate, duration of remission and the clinical state of the disease in patients with frequently relapsing nephrotic syndrome.

Objective To find out the beneficial effects of oral cyclophosphamide in patients with frequently relapsing nephrotic syndrome. Design Prospective study Setting: University paediatric unit, Galle Method Patients presenting with frequently relapsing, steroid sensitive nephrotic syndrome were included. Patients were given an 8 week course of oral cyclophosphamide (2.5mg/kg/day). Number of relapses before and after cyclophosphamide therapy, duration of remission and state of the disease were recorded. Results There were 17 patients included in the study (male 10, female 07). Mean age of onset of the disease was 35.2 months (SD± 4.3). Mean relapse rate before cyclophosphamide therapy was 4.5 per year (SD±1.2). Mean relapse rate after cyclophosphamide therapy was 1.1 per year (SD± 0.9). This decrease in relapse rate was statistically highly significant