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Pediatr Nephrol. 2019 Nov;34(11):2343-2350. doi: 10.1007/s00467-019-04296-6. Epub 2019 Jun 27.

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

Author information

1
Lucile Packard Children's Hospital, Stanford University School of Medicine, 300 Pasteur Drive, G306, MC 5208, Stanford, CA, 94305, USA. Lokesh.shah.md@gmail.com.
2
Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA.
3
Seattle Children's Hospital, 4800 Sand Point Way NE, Seattle, WA, 98105, USA.
4
Royal Manchester Children's Hospital, Hathersage Road, Manchester, M13 9WL, UK.
5
Alfred I. duPont Hospital for Children, 1600 Rockland Rd, Wilmington, DE, 19803, USA.
6
King's College London, Guy's Hospital, Great Maze Pond, London, SE1 9RT, UK.

Abstract

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.

KEYWORDS:

End-stage renal disease (ESRD); Focal segmental glomerulosclerosis (FSGS); Lipoprotein LDL-apheresis (LDL-A); Plasma exchange (PE); Steroid-resistant nephrotic syndrome (SRNS)

PMID:
31250206
DOI:
10.1007/s00467-019-04296-6

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