PCRRT in HUS: Role of peritoneal dialysis Thomas J Neuhaus and GF Laube, JF Falger, EM Rüth, MJ Kemper, O Bänziger University Children’s Hospital, Zurich

Zurich: Local History 1955: Gasser et al: Hemolytic-Uremic Syndromes: HUS 1964: Peritoneal dialysis for acute renal failure: HUS 1970: Hemodialysis and renal transplantation 1979: Continuous PD for chronic renal failure 1995: Continuous veno-venous hemofiltration for ARF

HUS = Hemolytic-Uremic Syndromes Hemolytic microangiopathic anemia: fragmentocytes, LDH , neg. Coombs Thrombocytopenia Uremia: acute renal failure Further symptoms / complications: Central nervous system (seizures, hemorrhages) Hypertension and heart failure Liver / Pancreas (with diabetes mellitus) Eye: retinal bleedings

HUS: D+ and D- D+ = Diarrhea-positive Verotoxin (or Shigatoxin)-producing E. Coli Other bacteria, e.g. Shigella …. („Big Mc disease“) Hemorrhagic colitis: mild – severe, intussusception D- = Diarrhea-negative Pneumococcal infections (T-antigen positive) Inherited and/or recurrent forms: e.g. complement (factor I/H) or vWF-cleavage protease deficiency Others: Pregnancy, drugs … „Many“ cases of unknown cause

Zurich: Epidemiology of HUS HUS: most frequent cause of acute renal failure in our hospital

HUS: Indications for dialysis modality since 1995 (1) Peritoneal dialysis: „in general“ first choice On ICU: CAPD On ward: mainly automated PD Hemodialysis if „older“ patient in „good general condition“ not requiring care in intensive care unit D-HUS and plasma-exchange (PEX) anticipated life-threatening hyperkalemia

HUS: Indications for dialysis modality since 1995 (2) Continuous veno-venous hemo(dia)filtration if „in bad general condition“ (+/- PEX) severe colitis Plasma-exchange (PEX) or plasma infusion if D-HUS and inherited type / complement deficiency suspected D- or D+ HUS with severe central nervous system symptoms, e.g. impaired consciousness, neurological deficit

Acute renal failure and peritoneal dialyis among adults ?! Recent review on „Renal replacement therapy of acute renal failure in ICU adult patients“ … Peritoneal dialysis is not further discussed … because of missing data no significant role 1 study showing a very high mortality ….

HUS: 1995 – 2005 (1) N = 68: 30 males, 38 females Age: median 2.3 years (2 months – 12 years) D+: 52 = 76%: 5 months – 12 years D- : 16 = 24%: 2 months – 10 years 6: pneumoccocal infection, 5 with septicemia 1: acute systemic lupus erythematodes 1: complement I deficiency (Dg: 9 yrs after onset !) 1: familial occurrence (mother / grandmother) 7: unknown cause

HUS: 1995 – 2005 (2) „Extreme“ values median range Creatinine 375 μmol/l (4.3 mg/dl) 50 – 995 Urea 32 mmol/l (192 mg/dl) 6 – 76 Hemoglobin 62 g/l 29 – 108 Platelets 36 G/l 7 – 271 Sodium 132 mmol/l 109 – 142

HUS and dialysis: 54 / 68 (79%) 16: D-HUS 52: D+HUS

HUS and PD: 44 / 54 dialysed (81%) 11: D-HUS 43: D+HUS

Acute PD before 1995: „stiff“ Cook-catheter or „soft“(„peel away“) catheter, inserted with trocar or Tenckhoff since 1995: only Tenckhoff catheter surgically placed by the surgeon (and the nephrologist also in theatre) under general anasthetic; at the same time insertion of central venous line

Acute and chronic PD Tenckhoff catheter: coil 2 sizes: < / > 1 year 1 cuff (glued by ourselves) upward facing

Acute PD on ICU: Fresenius system Lactate (march 2006: bicarbonate) Initial prescription: >10 - 15 ml / kg exchange: every hour 1000 IU Heparin/l 1.36% Glucose no antibiotics run by ICU-nurses

Acute PD on ward: Baxter system mainly automated PD Bicarbonate (Physioneal) Prescription: up to 40 ml / kg exchange: 2 – 4 hours 1000 IU Heparin/l 1.36% Glucose no antibiotics Run by ward / renal nurses

HUS and PD: 44 / 54 with dialysis Start with PD: 41/54 (76%) D+ 35/43 (81%) D- 6/11 (55%) Only PD: 35/54 (65%) D+ 30/43 (70%) D- 5/11 (45%)

HUS and PD: 3 patients: switch to PD from HD: 1 D+, transfer ICU  ward and end-stage renal failure CVVH: 1 D-, transfer ICU  ward HD/PEX: 1 D-, transfer ICU  ward and ESRF

HUS and PD: 6 patients: switch from PD to CVVH : 2 1: D+, general deterioration: † 1: D+, rectumperf.  2° peritonitis HD: 2 1: D+, insufficient ultrafiltration despite 3.86% glucose 1: D-, ESRF plus PEX: 2 D+, cerebral involvement: 1 †

HUS and PD: technical aspects Time span between emergency room entry and onset of PD in ICU: median 4 hours (2 – 20) Duration of PD: median 10 days (1 – 35)

HUS and PD: technical complications Peritonitis: n = 9 (all in ICU) Exit-site infection n = 3 Insufficient ultrafiltration: n = 1  switch: HD Catheter obstruction: n = 0 Insufficient dialysis: n = 0  No catheter had to be replaced.

HUS and hemofiltration Only CVVH: 2 1 D+: presentation with epileptic state 1 D-: pneumoccocal septicemia CVVH and PD: 3 CVVH and HD: 2

HUS and hemodialysis / PEX Only HD: 5, all D+ HUS 3: older patients – 12 years – in „good condition“ 1: recurrent intussusception and bowel resection before onset of ARF 1: severe hemorrhagic colitis Plus PEX: 4 2 D-, 2 D+

HUS: clinical complications (1) Hypertension: requiring medication 40 / 68 (59%), 28 / 44 with PD  16 patients with PD: „no medication, only PD“ Cardiomyopathy: 6: impaired ventricular function Pancreatitis: Amylase ↑ 24: but no diabetes mellitus Hepatopathy: Transaminases ↑ 43: but no liver failure

HUS: clinical complications (2) Gastrointestinal tract: n = 4 (all D+) 2 intussusception 1 rectum perforation 1 severe colitis Severe central nervous system: n = 7 4 D+: 3: remission, 1: † 3 D-: 2: sequelae (pneumococcal meningitis, massive hemorrhage), 1: † (SLE) Retinal bleeding: n = 2 (all D+)

HUS: stay in ICU / hospital median: 5 days (0 – 30) Hospital: median: 17 days (1 – 93)

HUS: daily running costs: Pat  20 kg CHF US$ Ratio to PD PD: 2 x 5 l bag: 44 34 1.0 HD: 60 46 1.5 set: 40 concentrate: 20 CVVH: 1 set / 3 days 175 – 210 135 – 160 4.5 set: 225 – 325 4 x 5 l filtrate: 100 HD and CVVH: plus costs of hardware…

Outcome: D+ HUS: n = 52

Outcome: D- HUS: n = 16

Conclusions (1): HUS Incidence: D+ >>> D- (over the last 35 years) 80% require dialysis Outcome: D+ >> D- Patient survival Recovery of renal function

Conclusions (2): HUS – PD Surgically placed Tenckhoff-catheter: Simple technique High efficacy Low frequency of side effects / complications PD in HUS is safe efficient convenient economic