Abstract: SA-PO675
Efficacy of a Gluten-Free Diet (GFD) in Children with Difficult-to-Manage Nephrotic Syndrome (NS)
Session Information
- Pediatric Glomerular Disease
November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1700 Pediatric Nephrology
Authors
- Trachtman, Howard, NYU Langone Health, New York, New York, United States
- Pehrson, Laura Jane, NYU School of Medicine, New York, New York, United States
- Vento, Suzanne M., NYU Langone Medical Center, New York, New York, United States
- Malaga-Dieguez, Laura, New York University School of Medicine, New York, New York, United States
- Gipson, Debbie S., University of Michigan Mott Children's Hospital, Ann Arbor, Michigan, United States
- Lemley, Kevin V., Childrens Hospital Los Angeles, Pasadena, California, United States
- Dell, Katherine MacRae, Cleveland Clinic Children's, Cleveland, Ohio, United States
- Srivastava, Tarak, Childrens's Mercy Hospital, Kansas City, Missouri, United States
- Kaskel, Frederick J., Children’s Hospital at Montefiore, Bronx, New York, United States
- Meyers, Kevin E.C., The Children Hospital of Philadelphia and University of Pennsylvania, Philadelphia, Pennsylvania, United States
- Faul, Christian, The University of Alabama at Birmingham, Birmingham, Alabama, United States
Background
Zonulin (ZON) increases gut permeability after exposure to gliadin in children with celiac disease. Plasma zonulin levels are increased in children with NS. Protease activated receptor-2, which mediates ZON effect in enterocytes, is present on podocytes. Thus, gluten-induced elevations in ZON may affect glomerular permeability and mediate proteinuria in children with NS.We conducted this study to assess the efficacy of a GFD in controlling disease in children with difficult-to-manage NS.
Methods
This multicenter, open-label trial tested the efficacy of a GFD in children with steroid-responsive, difficult-to-manage NS. The Treatment Period was 6 months. A positive response was defined as ≥50% reduction in relapse rate versus the prior 6 months or discontinuation of ≥1 immunosuppressive medication. The following data were tabulated: age, gender, race/ethnicity, serum creatinine, proteinuria, histopathology if available, and treatment. Serum was collected prior to and at completion of the Treatment Period to assess the effect on the glomerular cytoskeleton in vitro. Data are provided as mean±SD.
Results
14 children (8F:6M) were enrolled, age 7.8±4.6 yr, baseline serum creatinine 0.46±0.12 mg/dl, and Up/c 0.45±0.49 (mg:mg). There were 11 Whites, 1 Black and 3 other racial groups and 2 children were Hispanic/Latino. The underlying disease was MCD in 10 and FSGS in 4 cases. At the end of the Treatment Period, 4 participants had a positive response (2 reduced relapse rate and 2 reduced medication burden), 5 had no benefit (2 withdrew before 6 months), 3 patients are in the 6-month Treatment Period, and 1 child was lost to follow-up. One adolescent had no change in relapse rate but responded to corticosteroids more rapidly on the GFD. Baseline plasma zonulin concentration was 19.4±1.7 vs 13.4±0.9 pg/mL in non-responders (n=4) vs GFD responders (n=2), respectively, P=0.01.
Conclusion
Up to a third of patients with difficult-to-manage NS have a favorable response to implementation of a GFD. An elevated plasma zonulin level may predict a poor response to the maneuver. A trial of this dietary intervention may be warranted in children with frequently relapsing or steroid dependent NS to minimize the need for immunosuppressive agents.
Funding
- NIDDK Support