Decompensated. Liver disease in the ED. St Emlyn’s

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This week I am in Canada, specifically Whistler for the Annual Update in Emergency Medicine CPD conference. This is the second ski resort I’ve done this month, which is odd, because I don’t ski. That said it’s an amazing opportunity to spend time with old and new friends in the spectacular surroundings of the Canadian Coastal Mountains (not Rocky mountains as I have been corrected in the comments).

I’ve got three talks this week, with this one on the management of decompensated liver disease in the ED perhaps being the most important as I think it’s a bit of a neglected area of our practice where small changes can lead to big improvements in a very vulnerable group of patients.

This blog is an update from Gareth Robert’s excellent contribution back in 2019.

Acute vs acute on chronic liver disease.

Acute liver failure patients (ALF) are those that had a previously normal liver and who for whatever reason (e.g. toxins, vascular occlusion), whereas the more common presentation in my practice is for patients to present with liver failure on a background of chronic liver disease (CLD).

The three commonest reasons for CLD in UK practice are alcohol, viruses and non-alcohol fatty liver disease (NAFLD), although there are a whole range of less common conditions such as Wilson’s disease, primary biliary cirrhosis and cancers that can also cause problems. The incidence of liver disease in the UK and in many high income nations is increasing, largely due to the failure to control alcohol consumption and the increase in obesity rates. In other words CLD is on the rise and that means that we are likely to see ever more of these patients passing through our departments. Obesity related liver disease is expected to rise a lot in the next decade with a 95% increase in grade 4 NAFLD (cirrhosis) predicted in Canada from 2019-2030.

Patients with liver disease are common (40% of adults in my part of the world have fatty liver disease) but are usually well compensated. However, as the CLD gets worst and when challenged these patients can develop an acute on chronic decompensation of their hepatic function and that’s when they end up in the ED. Typically acute on chronic liver disease (ACLD) presents with a triad of jaundice, ascites and hepatic encephalopathy +/- features of whatever the precipitating event was (more on this later). Depending on the precipitant the decompensation can be quite dramatic or over several days.

Prognosis

ACLD is a medical emergency. It is surprising to many that the mortality of ACLD is as high as 20%. Previous studies in the UK have identified this high risk group as having significantly poor outcomes, with many preventable deaths. Quite why this happens is not well defined, but I do wonder if there is an element of bias here that I see with a lot of patients in whom a proportion are perceived to be self inflicted (alcohol and obesity are powerful drivers of discrimination amongst healthcare workers), and we need to move beyond that and be better people. This is a group with poor health, in a life threatening state and in whom relatively simple measures can significantly improve outcomes.

Although there are currently no artificial livers available, liver transplantation is an option in some cases, and there is good evidence that lifestyle modification can improve patient outcomes if they survive the ACLD admission.

The bottom line is that ACLD is a very significant event in a patient’s life, but it is not without hope and can in some cases be a turning point for our patients.

Three major causes of CLD. Alcohol, Viruses and NAFLD (often associated with obesity).

Initial assessment

Our initial approach will as always will include a good primary survey, observations and basic investigations. Record and track observations using a NEWS score (or equivalent).

Investigation

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).

Look for and treat the cause

Whilst all patients with ACLD will get supportive measures it’s really important to pursue the reason why your patient has decompensated. Whilst we don’t get an answer in around about 50% of cases we really don’t want to miss something we can treat.

Infections

Any infection can cause decompensation. Patients with CLD are effectively immunocompromised and so are vulnerable to range of infections, but unfortunately this also means that they may not always exhibit the typical signs of infection. Always check the chest x-ray carefully, examine the urine and perform a comprehensive examination of your patient.

Of particular note is spontaneous bacterial peritonitis (SBP) as this is a common reason for decompensation in those patients with ascites. It is thought to be a result of bacterial translocation from the gut (itself a feature of higher levels of inflammation in CLD) with likely haematogenous spread into the peritoneum. SBP may be obvious if your patient has a tense, painful, swollen abdomen, but in roughly 30% of cases there are few if any physical signs. The bottom line is that in any patient with ACLD and ascities you really should perform an ascitic tap and send it to the lab.

It is common for ACLD patients to have abnormal clotting but this must not prevent a tap from being performed. If you use ultrasound to guide your aspiration into a fluid filled area, the risks should be minimal and it’s a really important test for your patient (potentially life-saving). Some historical papers suggested that the INR had to be less than 1.5 to perform an ascitic tap. This is not true – get on with it!

SBP 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.

GI bleeding

GI bleeds are common cause of decompensation and evidence of this should be looked for. It may be obvious due to haematemesis or melaena, but in some cases it will be less obvious. Remember to PR the patient and also have a good look at the urea. An unexpectedly raised level may indicate an occult GI bleed.

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. Our gastroenterologists recommend that the patient is given volume resuscitation before Terlipressin is given (as they are worried about gut ischaemia in the hypovolaemic patient). Aim for a MAP of 65mmHg before terlipressin.

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 fewer 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. For patients on anticoagulants then do remember that you can reverse them with Octaplex (warfarin) or specific antidotes for some DOACs (e.g. adnexanet alpha).

Give platelets if <50.

Most patients with oesophageal varices will have CLD, but not all patients with CLD have varices. This can make resuscitation tricky, but if the variceal status is unknown then a low threshold for early endoscopy is indicated, and of course if your patient has known varices then again that needs urgent intervention from your endoscopy teams. Endoscopy should be performed within 12 hours and earlier if clinical indicated (uncontrolled variceal bleed).

Alcohol

Check the patients recent alcohol intake, start a CIWA score and prescribe IV Pabrinex for anyone with a high alcohol intake. 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. In patients with alcohol related hepatitis after prolonged and heavy alcohol use there is a role for steroids (following the STOPAH trial), but you really have to be careful to avoid them in patients who may be septic, so you probably want to rule sepsis out (as far as is practicable) before giving them.

General supportive measures

Whilst we attempt to control the precipitant/source of the acute decompensation we must always provide supportive measures to allow the liver time to recover.

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.

Encephalopathy

The main stay of management of this is to look for a precipitant (GI bleed, constipation, 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). Serum ammonia levels can be performed but are rarely helpful.

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. So my threshold for CT in these patients is pretty low.

Rifaxamin can also be used (a non-absorbable antibiotic) if patients do not respond to laxatives.

You can grade hepatic encephalopathy using this score Hepatic Encephalopathy Grades/Stages https://www.mdcalc.com/calc/674/hepatic-encephalopathy-grades-stages

Venous thrombo-embolism

This is an area where we can really make errors. Patients with ACLD are at high risk of VTE, irrespective of what their INR and other clotting tests show. In practice I see clinicians witholding VTE prophylaxis because the clotting tests are abnormal and this is dangerous. Unless patients are actively bleeding or their platelets are below 50 they should all get VTE prophylaxis with low molecular weight heparin.

Escalation and destination of care

There are some complex decisions to be made about admission to intensive care and ceilings of care with some ACLD patients. As always the patient’s pre-morbid state is vital to consider, but in those with good physiological performance prior to the current precipitating event there should be few if any barriers to ICU admission. For those patients with end stage disease and who are not suitable for transplantation then a more measured approach with appropriate conversations with the patient and their family regarding are required.

I have not found any specific scoring systems to predict ICU admission during an acute episode, although the CLIF-C ACLF (Acute-on-Chronic Liver Failure) score is useful in predicting mortality during an acute episode.

The following scores are useful in determining longer term prognosis and in identifying those suitable for liver transplantation.

  • Child-Pugh score for mortality in CLD https://www.mdcalc.com/calc/340/child-pugh-score-cirrhosis-mortality
  • MELD score. https://www.mdcalc.com/calc/78/meld-score-model-end-stage-liver-disease-12-olderMELD
  • United Kingdom Model for End-Stage Liver Disease (UKELD) https://www.mdcalc.com/calc/10147/united-kingdom-model-end-stage-liver-disease-ukeld

The bottom line

ACLD is a life threatening illness that requires the emergency physician to pursue general supportive management, but also the pursuit and control of the precipitating cause. Early treatment can make a real difference to our patients, and if we can get them through their acute episode there is hope for many through disease modification or transplantation.

One way to remember what to do is to remember WAVES. Good luck.

vb

S

References

  1. Gareth Roberts, “Decompensated Liver Disease and what we can do about it in the ED. St Emlyns,” in St.Emlyn’s, September 12, 2019, https://www.stemlynsblog.org/decompensated-liver-disease-and-what-we-can-do-about-it-in-the-ed-st-emlyns/.
  2. World Health Organisation: European Gateway Health For All Database available at https://gateway.euro.who.int/en/datasets/european-health-for-all-database/
  3. Public Health profile: Liver Disease Publice Helath Engalnd. avaiable at https://fingertips.phe.org.uk/profile/liver-disease/data#page/4/gid/8000063/pat/6/par/E12000002/ati/101/are/E06000009/iid/90892/age/1/sex/4
  4. Younghuan Z, Toa Z, Chenggi Z et al: Identification of reciprocal causality between non-alcoholic fatty liver disease and metabolic syndrome by a simplified Bayesian network in a Chinese population BMJ Open2015; 5(9): e008204. available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4593152/
  5. Measuring the Units: A review of patients with chronic liver disease. NCEPOD 2013 available at: https://www.ncepod.org.uk/2013report1/downloads/MeasuringTheUnits_FullReport.pdf
  6. BSG – BASL Decompensated Cirrhosis Care Bundle – First 24 Hours https://www.bsg.org.uk/resource/bsg-basl-decompensated-cirrhosis-care-bundle.html
  7. Papp, M. , Vitalis, Z. , Altorjay, I. , Tornai, I. , et al (2012), Acute phase proteins in the diagnosis and prediction of cirrhosis associated bacterial infections. Liver Int, 32: 603-611.
  8. CIWA-Ar for Alcohol Withdrawal available at https://www.mdcalc.com/ciwa-ar-alcohol-withdrawal
  9. Sort P, Navasa Arroyo V Algeudier X et al: Effect of Intravenous Albumin on Renal Impairment and Mortality in Patients with Cirrhosis and Spontaneous Bacterial Peritonitis N Engl J Med 1999; 341:403-409
  10. Guardelupe G Chirag P: Acute Kidney Injury in Cirrhosis. Hepatology; 2008,48(6) pp2064-77
  11. Angeli P, Gines P, Wong F, Bernardi M: Diagnosis and management of acute kidney injury in patented with cirrhosis: revised consensus recommendations of the international club of ascites; 2015 Gut  64(4)pp531-537
  12. McCormick P, Mistry P, Kaye G, Burroughs AK.: Intravenous albumin infusion is an effective therapy for hyponatraemia in cirrhotic patients with ascites: Gut 1990, 31; 204-207 available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1378381/pdf/gut00596-0102.pdf
  13. Scott R, Austin A, Kohle N, McIntyre C, et al Acute Kidney Injury is independently associated with death in patients with cirrhosis. cott RA, Austin AS, Kolhe NV, et alAcute kidney injury is independently associated with death in patients with cirrhosis Frontline Gastroenterology 2013;4:191-197.
  14. 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/
  15. Villanueva C, Colombo A, Bosch A, Concepcion M et al Transfusion Strategies for Acute Upper GastroIntestinal Bleeding N Engl J Med 2013; 368:11-21 avaiable at https://www.nejm.org/doi/full/10.1056/NEJMoa1211801
  16. Lin Y, Cheng Y, Lin C, Wang I. Increased risk of subdural hematoma in patients with liver cirrhosis, QJM: An International Journal of Medicine, 2017 110 (12) 815–82
  17. Child-Pugh score for mortality in CLD https://www.mdcalc.com/calc/340/child-pugh-score-cirrhosis-mortality
  18. Hepatic Encephalopathy Grades/Stages https://www.mdcalc.com/calc/674/hepatic-encephalopathy-grades-stages
  19. MELD score. https://www.mdcalc.com/calc/78/meld-score-model-end-stage-liver-disease-12-olderMELD
  20. United Kingdom Model for End-Stage Liver Disease (UKELD) https://www.mdcalc.com/calc/10147/united-kingdom-model-end-stage-liver-disease-ukeld
  21. CLIF-C ACLF (Acute-on-Chronic Liver Failure). https://www.mdcalc.com/calc/10240/clif-c-aclf-acute-chronic-liver-failure
  22. STOPAH trial https://pubmed.ncbi.nlm.nih.gov/25901427/
  23. Burden of nonalcoholic fatty liver disease in Canada, 2019–2030: a modelling study. https://pubmed.ncbi.nlm.nih.gov/32518095/

Cite this article as: Simon Carley, "Decompensated. Liver disease in the ED. St Emlyn’s," in St.Emlyn's, February 27, 2024, https://www.stemlynsblog.org/decompensated-liver-disease-in-the-ed-st-emlyns/.

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