LDL-apheresis-induced remission of focal segmental glomerulosclerosis recurrence in pediatric renal transplant recipients

Background Focal segmental glomerulosclerosis (FSGS) in pediatric patients is typically difficult to treat and will progress to end-stage renal disease (ESRD) in about 10% of cases. Following kidney transplantation, FSGS can recur in up to 56% of renal allografts—with a near 100% recurrence in subse...

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Published in:Pediatric nephrology (Berlin, West) West), 2019-11, Vol.34 (11), p.2343-2350
Main Authors: Shah, Lokesh, Hooper, David K., Okamura, Daryl, Wallace, Dean, Moodalbail, Divya, Gluck, Caroline, Koziell, Ania, Zaritsky, Joshua J.
Format: Article
Language:English
Subjects:
Allografts
Apheresis
Children
Chronic kidney failure
Complications and side effects
Creatinine
Development and progression
End-stage renal disease
Health aspects
Health risk assessment
Kidney diseases
Kidney transplantation
Low density lipoprotein
Low density lipoproteins
Medicine
Medicine & Public Health
Nephrology
Original Article
Patients
Pediatric research
Pediatrics
Proteinuria
Remission
Remission (Medicine)
Risk factors
Sclerosis
Transplants & implants
Urology
What’s New in Renal Transplantation
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Summary:Background Focal segmental glomerulosclerosis (FSGS) in pediatric patients is typically difficult to treat and will progress to end-stage renal disease (ESRD) in about 10% of cases. Following kidney transplantation, FSGS can recur in up to 56% of renal allografts—with a near 100% recurrence in subsequent transplants. Methods Four different pediatric centers across the USA and the UK employed a protocol using LDL-apheresis (LDL-A) and pulse solumedrol to treat recurrent FSGS after transplantation in seven patients. All the patients included in this series demonstrated immediate, or early, recurrence of FSGS, which clinically presented as nephrotic-range proteinuria within hours to days after implantation of the kidney. Results All patients experienced reductions in urinary protein to creatinine ratios resulting in partial or complete remission. All patients demonstrated improvements in their estimated GFRs at their most recent follow-up since LDL-A discontinuation. Conclusions This case series describes the successful treatment, across four different pediatric centers, of seven pediatric patients with recurrent post-transplant FSGS using the Liposorber® LA-15 in combination with pulse solumedrol.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-019-04296-6