Abstract
Objective: We conducted this study to test the hypothesis that plasma zonulin levels are elevated in pediatric patients with nephrotic syndrome compared to healthy controls.Study Design: Plasma zonulin levels were measured by ELISA in 114 children enrolled in the NEPTUNE study. Clinical and laboratory data were retrieved from the NEPTUNE database.Results: The median age of the patients was 10 (IQR = 5 to 14) years, 59 were male, 64 had minimal change disease, 47 focal segmental glomerulosclerosis, median eGFR was 96 (IQR = 80 to 114) ml/min/1.73 m2, and median urine protein:creatinine ratio was 0.5 (IQR = 0.1 to 3.4) (g:g). The plasma zonulin level was 14.2 ± 5.0 vs. 10.2 ± 2.5 ng/ml in healthy adults in a report using the same assay kit, P = 0.0025. These findings were confirmed in an independent cohort of children with nephrotic syndrome compared to healthy age-matched controls, P = 0.01. Zonulin concentrations did not differ in children with minimal change disease vs. focal segmental glomerulosclerosis, frequently relapsing vs. steroid-dependent vs. steroid-resistant clinical course, and were not influenced by the immunosuppressive treatment regimen. There was no relationship between plasma zonulin levels and the absolute or percentage change in proteinuria from enrollment until the time of the zonulin assay.Conclusion: Plasma zonulin levels are elevated in childhood nephrotic syndrome regardless of level of proteinuria or specific treatment. The cause of the high plasma zonulin levels and whether zonulin contributes to glomerular injury requires further study.
Highlights
Primary nephrotic syndrome (NS) in childhood is a rare condition with an annual incidence of 2–7/100,000 [1]
In an effort to determine if there was a relationship between plasma zonulin levels and the time to the remission, we examined 61 patients who were not in a complete remission and who had had subsequent follow-up data
When the patients were divided into two groups, one with nephrotic-range proteinuria and the other with sub-nephrotic range proteinuria, there was a trend toward a higher plasma zonulin level in the latter group whose median zonulin level was 16.3 ng/ml [interquartile range = 10.5–18.9] compared to the former group of 31 patients with urine protein: creatinine ratio ≥2 g:g whose median zonulin concentration was 11.6 ng/ml [interquartile
Summary
Primary nephrotic syndrome (NS) in childhood is a rare condition with an annual incidence of 2–7/100,000 [1] It is most commonly caused by either minimal change disease (MCD) or focal segmental glomerulosclerosis (FSGS), which are distinguished histologically and clinically by responsiveness to treatment. FSGS is more heterogeneous and may reflect the presence of a circulating permeability factor, a genetic abnormality in the podocyte that leads to proteinuria, or other environmental or infectious etiologies [6, 7]. For both disease entities, there is a pressing need to identify new approaches to treatment that are safe and effective
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