The particularities of the nephrotic syndrome in children

Written by Gáll Zsuzsanna, Kiss Éva, Egyed-Zs. Imre, Szabó J. Attila, Cucerea Manuela, Simon Márta, Barabás Annamária, Moréh Zsuzsánna

Introduction: Nephrotic syndrome (NS) is a clinically heterogeneous disease characterized by different histological variants and genetic determinants. NS generally has a favorable long-term prognosis, with excellent glucocorticoid responsiveness, but most suffer at least one relapse. Steroid resistance (SRNS) remains one of the most intractable causes of end-stage renal disease (ESRD) in the first two decades of life. Objectives of study: to study the clinico-histopathologhical profile and outcome of children with NS. Methods: We evaluated clinical features, laboratory data and histopathology of 206 children with nephrotic syndrome seen at the 2nd Pediatrics Clinic from Târgu-Mureş and 1st Pediatrics Clinic from Budapest. Results: From the 206 patients (male:female ratio 1.54, average age at diagnosis 5.05±4.33 years) with NS included in this retrospectiv study, 48.54% presented steroid-sensitive NS, 11.16% steroid-dependent NS and 40.29% SRNS, 12 patient had congential and 9 infantil NS. 127 patients had impure nephrotic syndrome, 60 patients with haematuria and 110 patients with hypertension. Percutanous renal biopsy was performed in 92 cases (44.66%), presenting minimal-change disease in 46.74% and FSGS in 33 patients (35.87%). Genetic testing was performed in 32 patients. ESRD developed in 40 patients (19.41%). Conclusions: The most dominant lesions are MCNS and FSGS. According to literature data the incidence of FSGS and steroid-resistant nephrotic syndrome has increased in the last years, confirmed in our study. ESRD develops in every fourth child with nephrotic syndrome. Prospective studies or at least standardized treatment for complicated cases is urgently needed.


Keywords: nephrotic syndrome, child, focal-segmental glomerulosclerosis

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