Resumo

NOGUERA VALVERDE, Ronald Armando; MADRIGAL CAMPOS, Gilbert  e  CARRANZA PORTOCARRERO, Alfonso.  Primary nephrotic syndrome: pathological and clinical analysis. Rev Cubana Pediatr [online]. 2007, vol.79, n.3. ISSN 0034-7531.

The main histological finding in biopsies from children with primary nephrotic syndrome are minimal lesions sensitive to steroid treatment. There may be other histological steroid-resistent lesions, some of them leading to fatal consequences. Other patients progress with steroid dependence and occurence of some adverse effects. With the objective of describing the corelation among clinical findings, initial response to steroids and the histology in children with primary nephrotic syndrome, the medical histories of 35 patients suffering primary nephrotic syndrome, whose biopsies had been performed at the National Child Hospital of Costa Rica from January, 1993 to December,1997, were prospectively reviewed. Sligt lesions were found in 45,7% of biopsies; diffuse mesangial glomerulonephritis in 28,6%; focal and segmentary glomerulosclerosis in 22.9%; membranous glomerulonephritis in 2,9% and besides, it was observed that hematuria and increased creatinine were significantly associated to initial steroid resistance (p< 0,05). Focal and segmentary glomerulosclerosis clinically manifest with initial steroid resistance and lack of response to cytotoxic drugs. Steroid-dependent patients presented with minimal changes or diffuse mesangial glomerulonephritis in their histology, with favorable response to cytotoxic drugs and good tolerance, and their clinical course changed to unfrequent relapses. Steroid-resistent patients must undergo biopsy as indicated, but in steroid-sensitive patients, this procedure may be neglected and instead, a cytotoxic drug-based treatment may be offered in order to change the clinical course of the disease. To those patients with steroid resistence, other treatment regimes should be provided.

Palavras-chave : nephrotic syndrome; renal biopsy; minimal lesions; focal and segmentary glomerulosclerosis; mesangial proliferative glomerulonephritis; steroids; children.

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