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LDL Apheresis in Drug Resistant Nephrotic Syndrome

This case study explores the use of LDL apheresis as a treatment option for a 14-year-old girl with drug resistant nephrotic syndrome. The patient underwent multiple treatment approaches including tacrolimus, bezafibrate, MMF, and rituximab, but remained severely nephrotic. LDL apheresis was then initiated, resulting in significant improvements in albumin levels, proteinuria, and cholesterol levels. This case highlights the potential of LDL apheresis as a treatment option for patients with drug resistant nephrotic syndrome.

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LDL Apheresis in Drug Resistant Nephrotic Syndrome

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  1. LDL Apheresis in Drug Resistant Nephrotic Syndrome Rodney D Gilbert

  2. Patient JM • 14 year old girl, presented 06/07/2015 • Severe oedema, hypoalbuminaemia and proteinuria • Diagnosis nephrotic syndrome • No response to steroids

  3. At Southampton • Cushingoid, oedematous • Creat 27 µmol/L • Albumin 20 g/L • U Prot/creat 4029 mg/mmol • Total cholesterol 16.3 mmol/L • Triglycerides 16.2 mmol/L • Renal Biopsy: FSGS (collapsing variety)

  4. Further treatment 1 • Tacrolimus (Blood levels 4.3 – 6.4 µg/L) • Bezafibrate 200 mg bd • Continued twice weekly IV albumin • 2 weeks later • Plasma albumin 15 g/L • UPC 3192 mg/mmol • MMF 1 g bd added • 2 months later • Albumin18 g/L • UPC 1371 mg/mmol • Cholesterol 14.3 mmol/L • TG 7.7 mmol/L

  5. Further treatment 2 • Rituximab 1 g IV x 2 doses • Remained severely nephrotic, fortnightly IV albumin • After 8 months: • Albumin 14 g/L • UPC 1575 mg/mmol • Cholesterol 12.5 mmol/L • TG 6.4 mmol/L

  6. 09/03/2016 • First session on the Liposorber!

  7. LDL apheresis

  8. LDL apheresis • Two sessions per week for 3 weeks • One session per week for 6 weeks • Plasma volumes processed increased to 3,500 ml • Last session: Cholesterol 14.2 -> 5.7 mmol/L • LDL C 1.92 post treatment • Albumin 19 g/L • UPC 1365 • NOT IN REMISSION!

  9. First UK patient treated with LDL apheresis for nephrotic syndrome • A few case reports (no genetics) • 2 small series • 11 patients: 5 complete remission, 2 partial remission • 44 patients: 11 complete remission, 10 partial remission (No genetics) • A few reports of post Tx recurrence patients successfully treated

  10. Keeping the customer stratified! • Genomic and clinical profiling of a national nephrotic syndrome cohort advocates a precision medicine approach to disease management. Kidney Int April 2017 • 187 patients with SRNS • WES: Genetic cause in 26.2% • No response to drugs, rapid deterioration, no recurrence • No genetic cause in 73.8% • Some responded to CNI, rituximab or MMF • Slower deterioration • 47.7% recurrence in graft

  11. Planned LDL Apheresis Study • Adults and children • Patients with SRNS with MCNS or FSGS • Drug resistant • Native kidneys or transplant recurrence • No genetic cause found • Watch this space!

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