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Is focal segmental glomerulosclerosis increasing in patients with nephrotic syndrome?
Fábio Fernandes Borges1, Luciana Shiraichi, Marcos Paulo Hippólito da Silva
1Faculdade de Ciências Médicas de Santos, UNILUS, São Paulo, Brazil.
Insights
Focal segmental glomerulosclerosis (FSGS) may be increasing in children with nephrotic syndrome, shifting from minimal change disease. This finding suggests a potential change in pediatric kidney disease pathology with significant clinical implications.
Area of Science:
- Pediatric Nephrology
- Renal Pathology
- Epidemiology
Background:
- Idiopathic nephrotic syndrome in children is traditionally linked to minimal change disease.
- Recent observations suggest a potential rise in focal segmental glomerulosclerosis (FSGS) frequency among pediatric nephrotic syndrome cases, mirroring trends seen in adults.
Purpose of the Study:
- To systematically review the literature and determine if the frequency of FSGS in pediatric nephrotic syndrome is increasing.
- To quantify any observed increase in FSGS occurrence over the past three decades.
Main Methods:
- A systematic review of studies comparing FSGS frequency in two distinct periods over the last 30 years.
- Meta-analysis of pooled data from six eligible studies involving 1,149 pediatric nephrotic syndrome patients.
- FSGS frequency was calculated using two denominators: all nephrotic syndrome patients and only those who underwent kidney biopsy.
Main Results:
- Analysis of 885 patients (denominator: all nephrotic syndrome cases) showed an odds ratio of 2.22 (95% CI = 1.18-4.18) for FSGS in the later period.
- Analysis of 603 patients (denominator: kidney biopsies) yielded an odds ratio of 1.98 (95% CI = 1.12-3.50) for FSGS in the later period.
- Both analyses indicate a statistically significant increase in the occurrence of FSGS.
Conclusions:
- The findings suggest a potential shift in the pathological landscape of childhood nephrotic syndrome, with a notable increase in FSGS.
- This trend has significant clinical implications due to the generally poorer prognosis associated with FSGS compared to minimal change disease.
Abstract:
Idiopathic nephrotic syndrome in children has conventionally been associated with minimal change disease. However, recent reports have conflictingly suggested that the frequency of focal segmental glomerulosclerosis (FSGS) in children might be on the increase, as has occurred in adults. The aim of the present work was to review the medical literature to ascertain whether an increase in the frequency of FSGS is occurring and, if so, to quantify such increase. We reviewed the studies comparing the frequency of FSGS in two consecutive periods over the past three decades (period 1 versus period 2). We pooled the data of the studies and then estimated FSGS frequency in two ways: (a) including in the denominator all patients with nephrotic syndrome and (b) including only patients who had undergone kidney biopsy. Both analyses were aimed to determine the odds ratio of FSGS occurrence in the second period. Six studies fulfilled the inclusion criteria, involving 1,149 patients with nephrotic syndrome. Four studies were used to calculate FSGS frequency, including in the denominator all nephrotic patients (n = 885), yielding an odds ratio of 2.22 (95% CI = 1.18-4.18). The analysis combining five studies with the number of biopsies in the denominator (n = 603) produced an odds ratio of 1.98 (95% CI = 1.12-3.50). These results suggest that a shift in the pathological pattern of nephrotic syndrome in children might be occurring, resulting in an increase in FSGS frequency. This hypothesis has major clinical significance due to the poorer prognosis associated with FSGS.
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