Abstract: SA-PO679
Pioglitazone (Pio) in Pediatric 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
- Hunley, Tracy E., Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Ng, Kar Hui, National University of Singapore, Singapore, Singapore
- Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
- Hidalgo, Guillermo, Eastern Carolina University, Greenville, North Carolina, United States
Introduction
Thiazolidinediones (TZD) may exert direct protective effects on injured podocytes. PPARγ agonists have been shown to reduce proteinuria in adults with non-diabetic kidney disease. We report our experience with off label, adjuvant use of Pio in pediatric patients with NS.
Case Description
Case 1
A 4 yr old boy was diagnosed with steroid resistant NS (biopsy minimal change). Despite ongoing treatment with prednisone (Pred), lisinopril, tacrolimus, mycophenolate (MMF), losartan, rituximab (Ritux), and Acthar he required twice weekly admissions for diuresis. Pio 15 mg/day was added and increased after 4 wks to 30 mg/day. U P/C decreased from 6 - 8 to 0.2 - 1 mg/mg over the next 7 months and cumulative immunosuppression was decreased considerably.
Case 2
A 13 yo female with obesity was found to have NS (Ur P/C 11 mg/mg, SAlb 1.7 g/dL, Screat 0.56 mg/dL), normal C3, and negative ANA. Biopsy showed collapsing FSGS. Glomerular size was normal. She was treated with Pred 40 mg, valsartan 80 mg, simvastatin 20 mg, and Pio 15 mg daily. After 7 wks, U P/C was 3.8 mg/mg and edema had resolved. Pred was decreased to 20 mg and Pio increased to 30 mg daily. After 6 months of treatment, U P/C was 0.5 mg/mg.
Case 3
A 6½ yo boy was diagnosed with NS and renal dysfunction after 3 months of intermittent edema (4+ proteinuria, SAlb 1.2 g/dL, Screat 0.93 mg/dL [eGFR 51 ml/min/1.73 m2]). He was born at 29 weeks gestation with a single right kidney. After 1 1/2 weeks of Pred 2 mg/kg/day, he was readmitted with anasarca and worse renal dysfunction (Screat 2.02 mg/dL [eGFR 24 ml/min/1.73 m2]. Biopsy showed collapsing FSGS. HIV and parvovirus evaluations were negative. Pred was continued and daily Pio 7.5 mg and pravastatin 20 mg were added. He received 2 doses of Ritux. Renal dysfunction progressed and he required dialysis 3 wks later, 6 ½ wks after presentation. During Pio, blood glucose ranged 93 – 159 mg/dL.
Case 4
A 5 yo boy was diagnosed with steroid sensitive but dependent NS. Addition of MMF did not allow Pred taper which was kept at 30 mg/d. Pio 15 mg daily was added for 16 weeks. During this time, Pred was successfully tapered to 5 mg qod while maintaining remission.
Discussion
In pediatric NS, the addition of Pioglitazone may improve proteinuria and allow reduction in corticosteroid and other immunosuppressant medications. In this case series, no hypoglycemia or other adverse events occurred.