Clinicopathological Analysis And Prognosis Of 21 Children With Primary Focal Segmental Glomerulosclerosis

Objective To investigate the clinical and pathological characteristics and to probe into the relationship between the clinical and pathological characteristics and prognosis of primary focal segmental glomerulosclerosis (FSGS) in children.Methods Twenty-one cases of FSGS proven by renal biopsy were retrieved from children's hospital of Chongqing Medical University from June 2003 to June 2008, Renal biopsies were reviewed ,the clinical feature,1aboratory data and pathological features were also studied retrospectively , and their prognosis were followed up.Result⑴Amongst the 21 cases of primary FSGS studied, the ratio of boys to girls was 1.33. The age of initial onset was from 1.25 to 13.33 years, the average age of initial onset was 7.36±3.88 years. The course was from 0.43 to 72 months. The follow-up duration was from 4 to 51 months (mean follow-up duration: 21.53±11.89 months).⑵The clinical manifestation included nephritic syndrome in 15(71%)cases(simple type in 10 and nephritic type in 5), glomerulonephritis in 6(29 % )cases (acute glomerulonephritis in 2 and persistent glomerulonephritis in 4).Of 21 cases, 17 cases (81%)presented with hematuria (2 with gross hematuria and 15 with microscopic hematuria),2 cases (10%) presented with hypertension and 2 cases (10%) presented with renal insufficiency .⑶The pathological classification included FSGS not otherwise specified (NOS) variant in 8 cases(38%),perihilar variant in 4 cases(19%),tip variant in 4 cases(19%),cellular variant in 5 cases(24%) and no collapsing variant .⑷Two patients were lost to follow up, one of which had chronic renal failure in admission, whilst 19 of 21 patients were followed up. At last follow-up, 10 patients (53%) were complete remission, 4 patients (21%) were significant remission, 4 patients (21%) were partial remission and 1 patient (5%) was no remission.⑸The possible prognostic factors were analyzed with stepwise logistic regression analysis. The possible prognostic factors which were analyzed, included the serum creatinine level before renal biopsy, the degree of proteinuria, the rate of glomerular sclerosis and tubulointerstitial lesion .It confirmed that tubulointerstitial lesion was related to prognosis. Tubulointerstitial lesion was a risk factor for prognosis (OR=3.2984).Conclusion⑴The predominant clinical manifestation of primary FSGS was nephritic syndrome in children. Hematuria was common. Hypertension and renal insufficiency were less frequently seen.⑵The NOS variant was the commonest pathological variant seen in primary FSGS. ⑶Most of children with primary FSGS had a good prognosis.⑷Tubulointerstitial lesion was associated with prognosis. The more severe the tubulointerstitial lesions were, the worse the prognosis was.