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Abstract: SA-PO385

Lipoprotein Apheresis in Pediatric Post-Transplant FSGS

Session Information

  • Pediatric Nephrology - III
    November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Kazi, Amber Jasmine, University of Washington School of Medicine, Seattle, Washington, United States
  • Okamura, Daryl M., University of Washington School of Medicine, Seattle, Washington, United States
  • Munshi, Raj P., University of Washington School of Medicine, Seattle, Washington, United States
  • Gordillo, Roberto, University of Washington School of Medicine, Seattle, Washington, United States
  • Dick, Andre, University of Washington School of Medicine, Seattle, Washington, United States
  • Smith, Jodi M., University of Washington School of Medicine, Seattle, Washington, United States
  • Menon, Shina, University of Washington School of Medicine, Seattle, Washington, United States
Background

Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease (ESRD) in adolescents with a recurrence rate of >30% after transplant (txp). Lipoprotein apheresis (LDL-A) has been successfully used for post-txp FSGS recurrence (r-FSGS). It has also been used for drug resistant primary FSGS and de novo FSGS (d-FSGS) post-txp. We aimed to describe the efficacy and safety of LDL-A in pediatric patients with FSGS post-txp.

Methods

Retrospective, single center study at a tertiary care pediatric hospital. Outcomes were complete remission (CR) defined as Upc < 0.2; partial remission (PR) defined as Upc 0.2-2 and ≥ 50% reduction in proteinuria from baseline. All Upc in mg/mg.

Results

Five patients, age range 7-21 yrs (2 females; 4 r-FSGS, 1 d-FSGS) underwent LDL-A. Those with r-FSGS, 3 episodes were immediately post-txp, and 1 after 40 mths. D-FSGS was diagnosed 16 mths post txp in a patient with ARPKD. Two had a relapse requiring a 2nd course of LDL-A. Each course had 12 sessions using Liposorber® LA-15 system over a period of 9 weeks. Of those with r-FSGS, 3 had CR or PR at a median of 4 weeks after starting LDL-A. Patient 2 had >50% reduction in Upc from 34 (pre LDL-A) to a nadir of 3.3. A clinical relapse with increase in Upc to 11 was noted 4 months after 1st LDL-A. A 2nd course resulted in CR. Patient 3 with d-FSGS also had a >50% reduction in Upc from 15 (pre LDL-A) to a nadir of 2.7. A relapse with AKI (Upc 10.3, serum creatinine 7.3mg/dL) was seen after 11 months. A second course of LDL was attempted but patient did not respond, and was transitioned to chronic hemodialysis. No adverse events related to LDL-A were reported. Patient 3 had central line associated thrombus.

Conclusion

We report successful use of LDL-A in 5 pediatric patients. CR/PR was achieved in all with r-FSGS. In d-FSGS, there was significant decrease in proteinuria but no remission. The therapy was well tolerated with the only adverse event secondary to vascular access. More studies are needed to assess its efficacy in primary and d-FSGS post-txp.