Abstract: Objective To explore the clinical-pathologic characteristic, prognosis and efficacy of steroid in acute poststreptococcal glomerulonephritis (APSGN) with the nephrotic-range proteinuria in children. Methods The clinical data of 50 cases of APSGN with nephritic proteinuria in recent 10 years were retrospectively analyzed. Results The median onset age of 50 children ( 35 boys and 15 girls) was 8 . 7 ( 6 . 8 - 10 . 0 ) years. The main clinical manifestations were hypertension ( 43 cases), gross hematuria ( 38 cases), edema ( 37 cases), acute kidney injury ( 34 cases) and hypoproteinemia ( 27 cases). All the 16 children who underwent renal biopsy had endocapillary proliferative glomerulonephritis, 15 had renal tubular lesions of varying degrees, 8 had renal interstitial changes, 7 had extensive foot process fusion, 5 had crescent formation, and 3 had extensive capillary loop compression and narrowing. The quantitative 24-hour urine protein of children with hypertension and gross hematuria is higher than that of children without hypertension and gross hematuria, and the difference is statistically significant (P< 0 . 05 ). The glomerular filtration rate (eGFR) of children with renal interstitial disease was lower than that of children without renal interstitial disease (Z=2.79 , P= 0 . 005 ). Kaplan-Meier survival curve showed that the recovery of renal function in the glucocorticoid treatment group was better than that in the no-treatment group, and the difference was statistically significant (χ 2 = 6 . 11 , P= 0 . 013 ). Conclusion APSGN with nephrotic-range proteinuria can be accompanied by acute kidney injury and obvious tubular interstitial damage, and glucocorticoid is helpful to the recovery of renal function

Key words: acute post streptococcal glomerulonephritis; proteinuria; glucocorticoid; pathology; prognosis