Clinicopathological analysis of the first onset nephritic type of primary nephrotic syndrome in children
Objective To investigate the clinicopathological features of the nephritic type of primary nephrotic syndrome in children.To explore the application value of clinical classification of primary nephrotic syndrome in China and the op-erability of classification criteria.Methods The clinicopathological data of children with first onset primary nephrotic syndrome(nephritic type)admitted to the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou Universi-ty from January 2014 to December 2020 were retrospectively analyzed.Results A total of 103 cases with first onset pri-mary nephrotic syndrome(nephritic type)were enrolled in the study.The classification criteria of nephritic type were 93.2％(96/103)for hematuria(microscopic hematuria and gross hematuria),13.6％(14/103)for hypertension,9.7％(10/103)for renal insufficiency,and 3.9％(4/103)for hypocomplementemia.All 103 cases underwent renal biopsy,and 10 cases underwent genetic testing.The final diagnosis was primary nephrotic syndrome(nephritic type)of 27 cases(26.2％),IgA nephropathy of 71 cases(68.9％),Alport syndrome of 3 cases(including 1 case with IgA ne-phropathy),monogenic steroid-resistant nephrotic syndrome of 1 case(WT1 gene mutation),and electron dense deposition disease(DDD)of 1 case.There were 23 cases(85.2％)with hematuria,including 5 cases(18.5％)with gross hematuria,among the 27 children finally diagnosed with primary ne-phrotic syndrome(nephritic type).Renal pathology revealed that there were 6 cases of minimal change glomerulopathy(MCD),5 cases of focal segmental glomerulosclerosis(FSGS),5 cases of atypical membranous nephropathy(AMN),4 cases of membranoproliferative glomerulonephritis(MPGN),3 cases of mesangial proliferative glomerulonephritis(MsPGN),3 cases of focal proliferative glomerulonephritis(FGN),and 1 case of membranous nephropathy(MN).Followed up to December 2022,5 cases were lost,9 cases achieved clinical cure or complete remission,3 cases had re-current proteinuria,and 10 cases had persistent proteinuria(5 cases of chronic renal insufficiency,including 3 cases of dialysis and 1 case of renal transplantation).Conclusion The main clinical manifestation of the nephritic type of prima-ry nephrotic syndrome in children is accompanied by hematuria.The current classification criteria are difficult to stand-ardize and unify.Most children who were initially diagnosed with primary nephrotic syndrome(nephritic type)were final-ly diagnosed as IgA nephropathy,rather than the nephritic type of primary nephrotic syndrome.Early diagnosis of renal pathology and genetic testing are more important.
NETL
NSTL
万方数据
