[Lipoid nephrosis in children. Development and anatomo-clinical correlation]

Lipoid nephrosis was identified by light, immunofluorescence and electron microscopy in 34 children aged 1 month to 10 years (23 males). These patients were followed up for a mean of 6.8 years (range 2.2 to 14 years). Treatment included oral prednisone (2 mg.kg.day for 4 weeks and then four days a week for 2 months). In steroid dependent or resistant patients oral cyclophosphamide 2.5 mg.kg.day was given for 2 months. Satisfactory responses to steroid therapy were recorded in 73.5% steroid dependence in 23.5% and steroid resistance in 3% of cases respectively. As a whole, 97% of patients responded before 8 week to steroids, cyclophosphamide or both. No morphologic differences were recorded from kidney biopsies among corticoid responders, dependents or resistants, neither between first and second biopsies which were done in four steroid dependent and one steroid resistant children. Slight morphologic differences were observed depending on the time elapsed from initial symptoms to renal biopsy: 39 days (means) when total disappearance of foot processes was seen (5 cases); 11 month (means) in 14 cases with partial pedicular absence and 20 months (means) in 15 children with segmental pedicular absence alternating with zones of normal foot process morphology. The total number of nephrotic episodes was 149, with a mean of 4.3 recurrences per patient, including three children (all girls) whose disease never recurred. Serious infections were detected in 4.7% of recurring episodes. At 5 years follow up 94% of patients were in remission. No deaths occurred among these patients.