1 Management of End Stage Liver Disease Dr Peter Lewindon Paediatric Gastroenterologist / Hepatologist Lady Cilento Children’s Hospital and QLTS, Brisbane George Abbott Paediatric Symposium Christchurch August 2015 Congratulations NZ and Christchurch’s favourite son
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Dr Peter Lewindon - University of Otago · Paediatric Liver Transplant - Indication Frequency Diagnosis Frequency Mean Range (% of all transplants) Biliary Atresia 49.1 % 36.3 % -
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Management of End Stage Liver Disease
Dr Peter Lewindon
Paediatric Gastroenterologist / Hepatologist Lady Cilento Children’s Hospital and QLTS, Brisbane
George Abbott Paediatric Symposium
Christchurch August 2015
Congratulations NZ and Christchurch’s favourite son
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FISPGHAN Survey 2008 Paediatric Liver Transplant - Indication Frequency Diagnosis Frequency Mean Range
Responsible 70% circulating BSL within hours of meal Fasting - catabolism / loss LBM / hypoglycemia
ESLD and abnormal INR “auto-anticogulated” ?
INR / Prothrombin Time measure of (reduced) coagulation in patients with normal liver function on Warfarin measure reduced liver synthetic function (factors II, V, VII, X and fibrinogen) in advanced liver disease
CONFUSION ? In ESLD - INR NOT measure coagulation / hemostasis
Steve, Now 15 months of Age Profuse Meleana for past few hours Hb 60 (was 105 last clinic) WCC 4.0 Platelets 51 INR pending (1.8) Pulse 140, poorly perfused
What is the Risk What to do immediately What to do Long Term
50% children with Kasai surviving without transplant have had significant GI Bleed by 5 years Survival after first bleed 50% at 5 year (cf Adult survival at 5 years of 17%)
Immediate Resuscitation IV PPI – Upper GI Bleed, Platelet dysfunction
IV Antibiotic – Sepsis precipitates variceal bleeding
(Burroughs, Lancet 1999) Bacterial infection in up to 65% bleeders Bacterial infection independent predictor of rebleeding SBP common before bleed Antibiotics reduce blood transfusion and rebleeding Improve Survival (immed/LT) by up to 10% IV Octreotide – (3-5mcg/kg/min) – inactive within hours Reduces ‘meal-driven” splanchnic blood flow (Cochrane review, no firm evidence of benefit)
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What next?
“Intestinal Decontamination” Colonic Flora generate Ammonia etc “Clear” with neomycin ? “Suppress” with Lactulose ?
Lactulose - SCFAs – acidic stool- inhibition colonic flora Give 2-6 hourly to provide 4-6 sloppy stools/day
Endoscopy?
Site of Bleeding Gastric erosions Duodenal erosions Oesophageal Varices Portoenterostomy “stomal” varices Gastric Varices Other (capsule endoscopy)
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Oesophageal Varices Should we treat and how ? If Bleeding – EVL “Banding” (If >7kg)
necrosis/perforation? Sclerotherapy (Harder) less effective, strictures
If not?? Yes, if “cherry red” / telangiectasia
Will get worse in ESLD - Waiting List?
And After – Prophylaxis ?
Beta Blockers - Beta 2-splanchnic vasoconstriction Primary - Elective Endoscopy and no bleed Secondary – Post portal hypertension related bleed In Adults, data clearly FOR Propranol in Both 1o and 2o
In Children – No good data
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ESLD-Summary
Many Issues not covered Focussed on Underlying Issues
Nutrition Bleeding Infection
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Timeline of impact of Baveno meetings on variceal bleeding related mortality
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And After – Prophylaxis ?
FOR Propranolol
Anecdotal experience - well tolerated - effective reducing