Over recent years there has been a rapid increase in the number of patients with chronic liver disease. In fact whilst deaths from cardiovascular disease and cancer are falling, we’ve seen a five-fold increase in deaths from liver disease since the 1970’s.
As a result more and more patients are presenting to the emergency department with decompensated chronic liver disease and more and more and more patients are presenting with complication. Indeed latest figures from the NHS suggest there were 131..2 hospital admissions per 100,000 population directly due to liver disease up from 108 in 2011. This increase is not replicated around Europe however and feels a distinctly British problem. It appears this is being driven by one thing. ALCOHOL
What causes chronic liver disease?
There are essentially three main causes of chronic liver disease; Alcohol, Viruses and non alcoholic fatty liver disease (NAFLD) as result of obesity, hypertension and hypercholesterolaemia; the so called “metabolic syndrome“. Although these causes the majority of chronic disease we probably need to be aware of the other causes such as autoimmune hepatitis and other conditions such as primary biliary cholangitis (it’s not cirrhosis anymore), genetic conditions such as Wilsons disease and haemochromatosis (there are others) as well as drugs. I think it’s important to say here that chronic liver disease is a separate entity to the hyper acute/ acute liver failure we may see in paracetamol overdose in the UK although it’s worth saying that world wide the viral hepatitides are still a leading cause of acute liver failure)
How does it present?
Many people with chronic liver disease are asymptomatic. It takes a seriously cirrhosed liver to cause symptoms. If I’ve a patient with an incidental finding of deranged liver function tests in an otherwise well person, other than counselling about lifestyle changes I would refer these people back to primary care for investigation. The patients I’m worried about in the ED are those who have decompensated. They present with deteriorating liver function, jaundice, encephalopathy or renal dysfunction. There’s often a precipitant to the deterioration. GI bleeding (variceal and non-variceal), infection, alcohol and constipation are common but others such as acute portal vein thrombosis, and transformation to hepatocellular carcinoma need to be considered. On the face of it making the diagnosis seems obvious; look for a Homer (appears drunk, sleepy and yellow) but according to a NCEPOD report in 2013 we aren’t very good at treating it and the report even suggests some of the increased mortality seen in the UK may in part be due to us failing these patients. So when we have a report telling us we’re not very good a managing something we do what every respectable health service does and create a care bundle. That’s exactly what doctors at the Freeman hospital in Newcastle did and their Decompensated Cirrhosis Care Bundle has been adopted by the British Society of Gastroenterology. The care bundle aims to simplify the management of these patients and splits it up in to seven areas. These areas concentrate on assessing how bad things are, looking for a precipitant and then managing these and the sequelae of decompensation. It comes as a checklist and should be completed with the first 6 hours of admission.
Routine blood investigation (FBC, UE, LFT, Coag, Bone profile & magnesium, Glucose and CRP should be taken alongside Blood cultures. A CRP> 10 has been shown to be a good indicator of infection in patients with cirrhosis. As well a blood tests, urine dipstick and chest X-ray should be performed. ALL PATIENTS with ascites should have a ascitic tap and the fluid sent for WCC, Albumin and MC+S. A SAAG (serum-ascites albumin gradient) can be calculated. You would expected to find a high gradient (>11g/L) as this is associated with portal disease (cirrhosis, Budd Chiari, Portal vein thrombosis however if a patient presenting with ascites has a low gradient, that might be more indicative of cancer (although this might not always be the case).
Check the patients recent alcohol intake, start a CIWA score and prescribe IV Pabrinex. Alcohol related hepatitis characterised by rapid onset of jaundice (< 3 months) and liver failure is a leading cause of decompensation and abstinence is the best prognostic factor so ongoing support from an alcohol team is a must.
Look for and treat infection obvs. Spontaneous bacterial peritonitis is defined by a WCC >500 or neutrophil count >250/mm^3 in the paracentesis fluid and should be treated with antibiotics to cover gram negative and positive organism. Human Albumin solution (HAS) should also be prescribed. A study by Sort et al showed that in patients with SBP the administration of HAS reduces the incidence of renal failure and mortality therefore 1.5g/kg of 20% HAS should be administered within the first 6 hours. It’s important to say this is in addition to the HAS that would be given when performing a large volume paracentesis.
Acute Kidney Injury and Hyponatraemia
Around 20% of patients with decompensation present with an AKI. It’s defined by a rise in creatinine from baseline of >26 μmol/L within 48 hours or a rise from baseline ≥50% over the course of a week. The AKI associated with cirrhosis is multifactorial with pre-renal AKI been present in just under half. Other causes such as nephrotoxic drugs and hepatorenal syndrome need to be considered. Stop all diuretics and nephrotoxic drugs. For the medics admitting people if this alongside rehydration doesn’t resolve the renal dysfunction, hepatorenal syndrome is likely. Fluidwise, 0.9% Saline can be used as the primary solution however with large volumes there is a risk of worsening the ascites. An alternative would be 5% HAS which has been shown to improve hyponatraemia in cirrhosis. Aim for a MAP of 80 and a normal UO (0.5mls/kg/hr) and consider early critical care involvement if this is unlikely to be achieved. AKI in decompensated liver disease is an independent predictor of mortality. Inpatient mortality is over 40% for those with AKI 3 so these are really sick individuals who deserve good quality care.
GI bleeds are common cause of decompensation and evidence of this should be looked for. Resuscitate and give Terlipressin. It’s a vasopressin analogue which reduces portal flow and thus variceal bleeding but be cautious in elderly patients and those with PVD as it can cause cardiac and bowel ischaemia. Evidence of coagulopathy should be treated with vitamin K and if the INR >2.0 FFP. A restrictive blood transfusion strategy is associated with better mortality, less bleeding and less complications in GI bleeding. In essence this means not transfusing until the Hb falls below 7 unless we’ve got a major haemorrhage on the go. We should be aiming to transfuse to an Hb >8.0. More can be read on GI bleeding in this blog by @cgraydoc and this other blog post of his which features a link to a podcast on GI bleeding by @EMManchester @docib.
The main stay of management of this is to look for a precipitant (GI bleed, constpation, sepsis) and give 100-120 mls of lactulose a day to ensure the patient is having at least 2 loose stool a day. It’s thought lactulose works in two ways; reducing the pH of the bowel trapping ammonia, and increasing the numbers of colonic lactobacilli (they contain no urease therefore there is less ability for the bowel flora to generate ammonia). In spite of the somewhat scant evidence its use has continued. Consider other causes of confusion too and think about requesting a CT brain; patients with complicated cirrhosis have a four fold increase in both traumatic and atraumatic subdural bleeds.
This is simply a reminded that although these patients might have a coagulopathy, they also have a significant risk of thromboembolic disease and as such should be prescribed LMWH if their platelet count is >50)
There we have it. Decompensated liver disease. 7 things to think about when managing these patients. Get using the care bundle.
- World Health Organisation: European Gateway Health For All Database available at https://gateway.euro.who.int/en/datasets/european-health-for-all-database/
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- Kim HR, Lee YS, Yim HJ, et al. Severe ischemic bowel necrosis caused by terlipressin during treatment of hepatorenal syndrome. Clin Mol Hepatol. 2013;19(4):417–420. doi:10.3350/cmh.2013.19.4.417 available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3894442/
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