Clinicopathological analysis of primary nephrotic syndrome in children based on 28 years renal biopsy data: a single center

Abstract

Objective To analyze the relationship between pathology and clinical features and the changing tendency of pathologic constitution in children with primary nephrotic syndrome(PNS). Methods A retrospective study was done at pediatric renal biopsy database of the First Affiliated Hospital of Sun Yat-Sen University starting from Jan.1984 to Dec.2011.All of these patients were diagnosed as PNS and under 14 years old.The cases were categorized into 3 periods: period 1(1984 to 1991), period 2(1992 to 2001), and period 3(2002 to 2011). Results A total of 363 patients were investigated in this study.Renal pathology revealed that there were 168(46.3%) patients with minimal change disease(MCD), 77 cases(21.2%) with focal and segmental glomerulosclerosis(FSGS), 76 cases(20.9%) with mesangioproliferative glomerulonephritis(MsPGN), 14 cases(3.9%) with membranoproliferative glomerulonephritis(MPGN), 12 cases(3.3%) with membranous glomerulopathy(MN), 3 cases(0.8%) with chronic sclerosing glomerulonephritis(CSGN), 2 cases(0.6%) with crescentic glomerulonephritis(CrGN) and other 11 cases(3.0%). Two hundred and five cases(56.5%) showed simple NS and 64.9% of them were MCD.One hundred and fifty-eight cases(43.5%) showed nephritic NS in which the most common pathological type was MsPGN(36.4%) in the second period and FSGS(39.6%) in the third period.There were 180 cases(49.6%) that frequently relapse with NS(FRNS) and 49(13.5%) steroid-dependent NS(SDNS). MCD(62.2%) occupied the largest proportion of FR/SDNS, followed by MsPGN(13.3%)and FSGS(13.3%). One hundred and seventy-two cases(47.4%) showed steroid resistant(SR) and 54.1%(93/172 cases) of them were primary steroid resistant(PSR). No significant difference was found between pathological type of PSRNS and secondary steroid resistant NS(SSRNS)(P>0.05). The most common pathological type of SRNS was MsPGN(41.5%) in period 2 and FSGS(41.5%) in period 3.There was no immune complex present in 108 cases(29.8%) in which the main pathological type was MCD(63.9%). Ninety-six cases(26.4%) mainly presented with IgM, of which 47.9% were SRNS and 37.5% were SD/FRNS. Conclusions The main pathological types are MCD, MsPGN and FSGS in children's PNS.There is a relationship between clinic and pathology.FSGS cases have been increasing while MsPGN cases have been decreasing in recent 20 years.It is advised to perform the renal biopsy early for nephritic NS, SD/FRNS and SRNS and repeat the biopsy if necessary. Key words: Nephrotic syndrome, primary; Renal biopsy; Pathology; Child