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Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis
Insights
This study differentiates two types of focal segmental glomerular sclerosis (FSGS) in children with nephrotic syndrome: steroid-resistant (SR) and initially steroid-sensitive (SS) forms. SR-FSGS presents earlier, with more females, hematuria, growth issues, and faster progression to renal failure.
Area of Science:
- Pediatric Nephrology
- Glomerular Diseases
- Renal Pathology
Background:
- Focal segmental glomerular sclerosis (FSGS) is a significant cause of nephrotic syndrome in children.
- Understanding the long-term outcomes and distinct clinical presentations of FSGS is crucial for patient management.
Purpose of the Study:
- To investigate the long-term outcomes of pediatric nephrotic syndrome patients with focal segmental glomerular sclerosis (FSGS).
- To identify and characterize distinct clinical and histological subgroups within FSGS.
- To compare the progression and outcomes of steroid-resistant (SR) versus initially steroid-sensitive (SS) FSGS.
Main Methods:
- Longitudinal follow-up study of 24 children with biopsy-proven FSGS over 3 to 19 years.
- Classification of patients into steroid-resistant (SR) and initially steroid-sensitive (SS) groups based on initial treatment response.
- Comparison of demographic, clinical, and renal outcome data between the SR and SS groups.
- Analysis of kidney transplant outcomes and FSGS recurrence rates.
Main Results:
- Two distinct groups emerged: SR-FSGS (mean age 7.7 years, 11/14 females) and SS-FSGS (mean age 3.5 years, 3/10 females).
- SR-FSGS patients showed higher incidence of hematuria (9/14), greater growth retardation (9/13), and earlier progression to end-stage renal failure (2.3 years vs. 10 years).
- FSGS recurrence in kidney allografts was observed in 17.6% of cases, with similar rates in both SR and SS patient groups.
Conclusions:
- Pediatric FSGS with nephrotic syndrome may represent distinct nosologic entities, particularly SR-FSGS and SS-FSGS with evolution to FSGS.
- Steroid-resistant FSGS in children is associated with earlier onset, distinct demographic features, and more rapid renal function decline.
- These findings highlight the importance of differentiating FSGS subtypes for predicting prognosis and guiding therapeutic strategies.
Abstract:
We studied the long-term outcome of a group of children with the nephrotic syndrome who showed the histological lesion of focal segmental glomerular sclerosis (FSGS) during the course of their illness. Of 25 such children studied, a complete follow-up ranging from 3 to 19 years was available in 24. Two distinct groups could be identified. Patients in the first group were characterized by steroid resistance (SR) from the onset, whereas those in the second group were initially steroid sensitive (SS), and had the histological lesion of minimal change which, over time, evolved into FSGS. SR patients had a mean age of 7.7 +/- 3.7 years compared to SS patients who were 3.5 +/- 2.5 years old (p less than 0.01). There were more females (11 of 14) in the SR group than in the SS group (3 of 10; p less than 0.02). The incidence of hematuria was higher in the SR patients (9 of 14) than SS patients (2 of 10; p less than 0.05). SR patients also exhibited a greater degree of growth retardation at the end of the follow-up period (9 of 13 compared to 1 of 8 SS patients; p less than 0.02). SR patients reached end-stage renal failure earlier (2.3 +/- 1.3 years) than SS patients (10 +/- 5.8 years; p less than 0.01) after the initial biopsy. Of the 13 kidney transplanted into 9 SR patients, recurrence of FSGS was noted in two allografts. Of the 4 kidneys transplanted into 2 SS patients, recurrence was seen in 1. The overall recurrence rate of FSGS in allografts was 17.6%. Our study suggests that the two varieties of FSGS occurring in nephrotic patients may be distinct nosologic entities rather than a single disease with varied manifestations.