Primary nephrotic syndrome during childhood in Turkey

Abstract

Background: Minimal change nephrotic syndrome (MCNS) is the most common cause of primary nephrotic syndrome (NS) during childhood. However, recent studies from different countries have reported an increasing incidence of focal segmental glomerulosclerosis (FSGS) in children.

Methods: This is a retrospective study in which 392 Turkish children who were diagnosed with NS during the last 10 years and were followed for at least 2 years, were evaluated. Mean age of the study group was 4.6 +/- 3.4 years (range 0.9-16 years) and 232 were male and 160 were female.

Results: In total, 280 patients were diagnosed as MCNS with their initial presentations, laboratory features, and clinical course. Kidney biopsy was performed in the remaining 112 children according to current recommendations. The results showed that membranoproliferative glomerulonephritis (MPGN) was the most common histopathologic diagnosis, 38 (34%) of the 112 patients were found to have MPGN. The number diagnosed as FSGS was 26 (23%). A significant difference was found between the age groups for both MPGN and FSGS, the former being more common in children >6 years of age and the latter more frequent in children </=6 years. MCNS was found in only 19% of all biopsies performed and the total incidence of MCNS (presumptive + biopsy proven) was 76%. There was no significant difference between the incidence of different histopathological subtypes before and after 1995.

Conclusion: Minimal change nephrotic syndrome is still the most common primary NS in childhood and MPGN is found to be the most common histopathologic subtype in the present patient population who underwent biopsy.