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Liposorber® LA-15 system for LDL apheresis in resistant nephrotic syndrome patients

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Abstract

Background

Steroid-resistant nephrotic syndrome (SRNS) is a major cause of stage 5 chronic kidney disease (CKD 5) in children. LDL apheresis (LDL-A) is now FDA approved for the treatment of pediatric focal segmental glomerulosclerosis (FSGS). Effective management of hyperlipidemia with LDL-A in SRNS patients may prevent progression of kidney disease and lead to remission. We report a case series of patients who received LDL-A for treatment of SRNS

Methods

We describe five children with SRNS who were treated with 12 sessions of LDL-A. Partial remission (PR) is defined as urine protein to creatinine ratio (UPC) of 0.2–2 (g/g) or decrease in UPC ≥ 50%, and complete remission (CR) is defined as UPC < 0.2 (g/g).

Results

One patient achieved CR and three achieved PR. One patient did not respond to therapy. The earliest that a patient achieved PR was at treatment #10 and some did not respond until after LDL-A was completed. Those who responded stayed in either CR or PR for extended periods of time. LDL-A was successful at significantly reducing LDL (p < 0.001), total cholesterol (p < 0.001), and triglyceride (p < 0.001).

Conclusions

LDL-A was able to significantly decrease the lipid levels in these patients and induce CR and PR in the majority. The current study confirms previous studies showing those with a higher glomerular sclerosis burden were less likely to respond. LDL-A should be considered in patients with treatment-resistant SRNS and should be considered before there is a high burden of glomerular sclerosis to provide the best chance of success.

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Data availability

The datasets generated during and analyzed during the current study are available from the corresponding author on reasonable request.

Code availability

Not applicable.

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Authors and Affiliations

Authors

Contributions

KT designed the study. KT and MFA performed data acquisition. KT carried out data analysis. KT and MFA performed literature review. KT and MFA prepared the manuscript. All authors were involved in reviewing the manuscript.

Corresponding author

Correspondence to Mohammad Al-mousily.

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Approved by the Medical University of South Carolina Institutional Review Board.

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Conflict of interest

Dr. Twombley receives funding from Kaneka for the FDA post-approval study.

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Al-mousily, M., Nicoara, O., Selewski, D.T. et al. Liposorber® LA-15 system for LDL apheresis in resistant nephrotic syndrome patients. Pediatr Nephrol 37, 585–592 (2022). https://doi.org/10.1007/s00467-021-05211-8

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  • DOI: https://doi.org/10.1007/s00467-021-05211-8

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