Clinical features and prognostic analysis of primary membranous nephropathy in children with positive glomerular M-type phospholipase A2 receptor / 中华实用儿科临床杂志

ABSTRACT

Objective:To analyze the clinical and prognosis of primary membranous nephropathy (PMN) in children with positive glomerular M-type phospholipase A2 receptor (PLA2R).Methods:A total of 69 children diagnosed with PMN by renal biopsy admitted to the Department of Pediatrics of Eastern Theater Command General Hospital from January 2006 to December 2018 were retrospectively analyzed, including 40 males and 29 females, with an average age of 14.86 years.According to the immunofluorescence of renal pathology, they were divided into PLA2R positive group and PLA2R negative group.Pathological features between 2 groups were compared by the t test, Mann- Whitney U test and Chi- square test.Kaplan-Meier method was used to compare the long-term renal survival rate and cumulative remission rate between 2 groups. Results:A total of 69 pediatric PMN patients were included.The po-sitive rates of serum anti-PLA2R antibody and positive expression of PLA2R in renal tissues were 53.6% (37 cases) and 82.6% (57 cases), respectively.The proportion of children with clinical manifestations of large proteinuria [55 cases(96.5% ) vs.9 cases(75.0%), P=0.034] was significantly higher in the PLA2R positive group than that of the PLA2R negative group.Blood urea nitrogen level was significantly higher in the PLA2R positive group than that of PLA2R negative group[1.14(0.93, 1.54) mg/L vs.0.80 (0.44, 1.18) mg/L, P=0.049], while estimate glomerular filtration rate(eGFR) [162.26 (139.81, 185.53) mL/(min·1.73 m 2) vs.199.52 (157.58, 212.01) mL/(min·1.73 m 2), P=0.034] and serum IgG [3.58 (2.50, 5.43) g/L vs.5.14 (4.35, 6.03) g/L, P=0.016] were significantly lower.The cumulative remission rate was significantly higher in the PLA2R negative group than that of PLA2R positive group ( P<0.001). The 24 h urinary protein ≥50 mg/kg ( HR=0.119, 95% CI 0.021-0.595, P=0.010)was an independent risk factor for renal prognosis, and PLA2R( HR=0.263, 95% CI 0.125-0.551, P<0.001) and 24 h urinary protein ≥50 mg/kg ( HR=0.568, 95% CI 0.125-0.551, P=0.041)were independent predictors of urinary protein remission.PLA2R ( HR=1.020, 95% CI 0.698-1.682, P=0.656)was not associated with renal prognosis. Conclusions:The severity of PMN in children with positive PLA2R was higher than that in those with negative PLA2R.The long-term cumulative remission rate of PLA2R negative children with PMN was higher than that of PLA2R positive children.