Abstract

Strict therapy protocol, which would be used universally for certain morphological forms of primary nephrotic syndrome, does not exist. The aim of the study was to show the effects of used therapy protocol in treatment of primary nephrotic syndrome at the Institute of Nephrology, Clinical Center University of Sarajevo in period of 2000-2005. The retrospective analysis covered 48 patients (17 women and 31 men) with idiopathic nephrotic syndrome, where pathomorphological changes were proved by kidney biopsy. Minimal change disease was confirmed with 6 (12.5%) patients. All patients were initially treated with corticosteroids with dose of 1 mg/kg of body weight. Five patients were in the group of primary responders (83.3%) with long term total remission, and 1 patient (16.6%) was a primary responder with 3 relapses in 8 months with a therapy of corticosteroids and bolus of cyclophosphamide. Diffuse mesangial proliferative glomerulonephritis was shown in 13 patients (27.1%). Seven patients from this group were treated with corticosteroid therapy (1 mg/kg of body weight for 4 weeks, followed by 0.5 mg/ kg of body weight until therapeutical response was achieved, and finally gradual exclusion of therapy after eight weeks in responsive patients). Six patients were treated with corticosteroids and one-month of bolus cyclophosphamide during half of year (10-5 mg/kg of body weight). Total remission was achieved in 37,9% of the patients. The IgA nephropathy presented with the nephrotic syndrome was shown in 10.4% (5) of the patients. Three patients from this group were treated with corticosteroid therapy (1 mg/kg of body weight for 4 weeks, followed by 0,5 mg/ kg of body weight until therapeutical response was achieved, and finally gradual exclusion of therapy after eight weeks in responsive patients) and.2 patients with corticosteroids and cyclophosphamide (1.5 mg/kg of body weight) during 6 months. Complete remission of nephrotic syndrome from this pathomorphological category was achieved in 2 patients. Membranoproliferative glomerulonephritis was shown in 6 patients (12.5%). All were treated with corticosteroids plus bolus of cyclophosphamide. Partial remission was achieved in one patient. Membranous glomerulonephritis was confirmed in 18 patients (37.5 %). Combined therapy of corticosteroids and bolus of cyclophosphamide was used in 7/18 patients, in 2/18 patients therapy of corticosteroids and per os cyclophosphamide (2 mg/kg of body weight) and in 9/18 patients cyclosporine therapy (3 mg/kg of body weight). Complete remission was achieved in 38.8% of the patients. A high percentage of achieved remissions of primary nephrotic syndrome in adults shows the efficiency of immunomodulating therapy used. Membranoproliferative glomerulonephritis still remains a therapy problem.

Highlights

  • Primary nephrotic syndrome (NS) is a consequence of immunoreactive glomerulopathies of unknown cause and is characterised with massive proteinuria, hypoalbuminaemia, hyperlipidaemia and oedema

  • Minimal change disease (MCD) presents pathohistological base in, of the patients with primary nephrotic syndrome (NS), as well as membranoproliferative glomerulonephritis (MPGN), while IgA nephropathy is presented in the lowest number of the patients with primary nephrotic syndrome (, )

  • The therapy protocols applied in all pathohistological forms of primary nephrotic syndrome were not followed by the deterioration of kidney function

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Summary

Introduction

Primary nephrotic syndrome (NS) is a consequence of immunoreactive glomerulopathies of unknown cause and is characterised with massive proteinuria, hypoalbuminaemia, hyperlipidaemia and oedema. It is presented by various histological appearances of glomerular disease. Thanks to many researchers and analyses, nowadays we have more or less accepted principles of primary nephrotic syndrome treatment ( ). Universal therapeutic protocol, which will be used for every specific morphological form of treatment, doesn’t exist. The aim of this paper was to evaluate the effects of applicable therapy protocols in treating patients with primary nephrotic syndrome at the Institute of Nephrology Clinical Centre University (CCU) in Sarajevo in period from to. The average observation period for each patient was one year

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