1. Experiences with plasmapheresis in our pediatric dialysis unit L. Koster-Kamphuis, E. Cornelissen, E. Levtchenko, N. van de Kar Dept. of Pediatric Nephrology University Children’s Hospital Nijmegen The Netherlands

2. Introduction Extracorporeal therapy Therapeutic Plasma Exchange (TPE) Replacement fluid –Fresh frozen plasma –Albumin

3. Introduction Vascular access Anticoagulation Adapted machinery Blood priming

4. Introduction Removal of (auto)antibodies, immune complexes Intoxication (protein bound) Removal of protein bound medication Hyper viscosity (Para proteins) > 15000 Dalton

6. Methods Membrane filtration AK 100 with PF 1000 filter PRISMA with TPE 2000 filter Fresh frozen plasma or Albumin Heparin anticoagulation

8. Methods PF 1000TPE 2000 MachineAK 100PRISMA Surface area (m 2 ) 0,150,35 Extracorporeal volume (ml) 90 (23 ml filter + 70-80 ml lines) 90 (set with filter and lines) AccessSingle or double (single pump) double Bloodflow<200 ml/min>100 ml/min

9. Methods Choice of replacement fluid Fresh frozen plasma when substitution of plasma components is needed Albumin solution when removal of the plasma is beneficial due to disease causing factors in the plasma

10. Patients N=16 Age 4 months- 18 years Weight 7-67 kg

14. Patients Atypical HUS (n=7) Recurrent NS after transplantation (n=3) Anti-GBM nephritis (n=1) Wegeners Granulomatosis (n=1) Guillain-Barré (n=1) Myasthenia Gravis (n=1) ADEM (n=1) Progressive necrotizing encephalopathy (n=1)

15. Results Positive effect Atypical HUS Myasthenia Gravis Wegeners granulomatosis Recurrent NS after transplantation (n=2)

16. Results No effect Anti-GBM nephritis Progressive necrotizing encephalopathy

17. Results Possible or some effect Guillain-Barré ADEM Recurrent NS after transplantation (n=1)

18. Complications Allergic reactions Hypocalcaemia Hypotension Access problems Catheter related problems Infection Clotting

19. Conclusion TPE is a useful treatment in infants and children for a wide range of indications TPE is a specific treatment modality that fits in the program of a dialysis unit