Chronic Liver Failure
Chronic Liver Failure (CLF) is a disease process of the liver that involves progressive destruction and regeneration of the liver parenchyma leading to fibrosis and cirrhosis. Venous flow into the liver decreases due to this, leading to elevated portal pressures. Portal hypertension then leads to splenomegaly, causing anaemia and thrombocytopenia. Ascites, hepatorenal syndrome, hepatopulmonary syndrome, variceal bleeding and hepatic encephalopathy are all recognised complications.
Cirrhotic patients may have stable liver functions for long periods of time, and an acute insult in the presence of advanced fibrosis and decreased functional reserve may lead to development of hepatic decompensation. These patients may develop decompensation in two ways:
The most common is a progressive decompensation resulting in a clinical course of end-stage liver disease.
Acute liver decompensation resulting from a precipitating event such as variceal bleeding or sepsis.
You may also want to view the Acute Liver Failure clinical tool.
NASH (non alcoholic steatosis)
Autoimmune hepatitis, Primary sclerosing cholangitis, Primary biliary cirrhosis
Right heart failure, CCF
Drugs e.g. Amiodarone
Metabolic / genetic e.g. Wilsons, Haemachromatosis.
Remember to ask about
Medications patient taking and degree of compliance
Previous endoscopy and presence of varices
Anorexia, weight loss, pruritus, fever, abdo pain, shortness of breath etc.
Examination – Signs
Vital signs – pulse may be low due to B blockers (for varices), BP may be low due to bleeding / dehydration / sepsis, sats may be low due to pleural effusions / splinting of diaphragm / encephalopathy and drowsiness
Hepatomegaly – may be tender
- FBC: May reveal thrombocytopenia, anaemia, macrocytosis
- Coagulation studies
- Liver function tests: Often elevated levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), low albumin / protein
- Bilirubin level: Elevated
- Serum ammonia level: May be dramatically elevated. Risk of intracranial hypertension highest when sustained level ammonia 150-200µmol/L (250-340 µg/dl)
- Serum glucose level: May be dangerously low
- Lactate: Often elevated
- Creatinine level: May be elevated
- Electrolytes: Na may be low, often dilutional due to fluid accumulation
- Calcium, magnesium, phosphate
- Blood cultures: For patients with suspected infection
- Pregnancy test
- If first presentation of CLD consider full liver screen including hepatitis A, B, C +/- D, EBV, CMV, ANA, anti-smooth muscle antibody, ? 1 antitripsyn, Cu, paracetamol, TFT, immunoglobulins, ceruloplasmin, alpha-feto protein.
Tests to consider
- Hepatic Doppler ultrasound
- CT Abdo
- CT Brain particularly looking for intracranial bleeds
- Echo: hypoxic hepatitis can result from myocardial dysfunction
- Intracranial pressure monitoring.
Management – general
Patients with liver failure often deteriorate quickly. If patients are not unwell enough for resuscitation beds they should be placed in a highly visible bed with a cardiac monitor. For patients who require intubation / inotropic support / ICU care one must assess the appropriateness of these interventions, the reversibility of the situation and the prognosis for the patient. The Child-Pugh score can be used to help determine prognosis.
Management - specific
- Thiamine IV 200mg stat and continue IV 200mg 8 hourly – there is no consensus on route, dose, frequency. Patients with Wernickes encephalopathy may need doses up to 500mg IV
- Multivitamins for nutritional deficiencies, replace electrolytes
- Glucose as have lost gluconeogenic powers – give thiamine prior to glucose load
- Alcohol withdrawal scale and oral diazepam
- Lactulose 20mLs QID
- Education about abstinence
- Work out Maddreys Discriminant Function (MDF) score. Patients with MDF ≥32 have a poor prognosis. Discuss with gastroenterology. They may benefit from:
- Steroids – minimal data but there is a suggestion that steroids have a protective effect - start 40mg/day if no contraindications.
- Anticytokine therapy – pentoxifylline – oral phosphodiesterase inhibitor which decreases production TNF? and other cytokines.
- Indications for paracentesis include abdominal pain and SOB
- Check inr/plt and for relative contraindications like adhesions, bowel obstruction
- Send sample for albumin, cytology, cell count, culture
- If large amount (>6L) to be drained then replace volume with human albumin solution (no evidence for use if less than this). Once paracentesis complete 100mLs 20% albumin / 3l ascites.
- Na restriction (plus water restriction if Na
- Spironolactone +/- frusemide
- Link to Life In The Fast Lane Paracentesis video
Spontaneous Bacterial Peritonitis (SBP)
- SBP is infection of ascitic fluid without clear source such as perforation / abscess (secondary bacterial peritonitis).
- Diagnosis – Ascitic fluid - Polymorph cell count >250cells/mm? or positive fluid culture.
- Typical organisms Klebsiella, E coli, Strep pneumoniae.
- Treatment - If SBP suspected treat empirically with Cefotaxime 2g 8 hourly.
- Stop ? blockers – it has been found non selective ? blockers increase haemodynamic instability, time of hospitalisation, and risks for hepatorenal syndrome and acute kidney injury.
- PO vit K 10mg TDS
- Platelet transfusion if plt
- Active haemorrhage FFP +/- recombinant factor VIIa - discuss with Haematologist
- Lactulose 20mLs QID
- Early intubation if required to protect airway. Prior to this one must first make an assessment about the appropriateness of this intervention.
- Link to grades
See Upper GI bleeding Clinical Tool
- Caused by extreme renal artery vasoconstriction in setting of splanchnic vasoldilation, low cardiac output and low effective plasma volume.
- Plasma expansion with albumin (100mLs 20% HAS) +/- haemodialysis plus vasoactive agents like octreotide or norad.
- Paracentesis has been found to improve renal function in volume resuscitated patients.
Hepato-pulmonary syndrome (HPS)
- Tense ascites can cause pleural effusions and splint the diaphragm however true HPS is a disorder of pulmonary vascular dilatation and shunting.
- Supplemental oxygen mainstay of treatment.
- For refractory hypoxaemia, liver transplant is considered and higher priority given to those with liver failure and HPS.
Further References and Resources
1. Galvin R. et al. (2010) EFNS guidelines for diagnosis, therapy and prevention of Wernicke encephalopathy, European Journal of Neurology, vol 17, no. 12, pp. 1408-1418.
2. Moore, KP. and Aithul, GP. (2006) Guidelines on the Management of ascites in cirrhosis, Gut Journal, vol. 55, suppl 6, vi1-vi12.3.
3. Mandorfer M et al. (2014) Nonselective B blockers increase risk for hepatorenal syndrome and death in patients with cirrhosis and spontaneous bacterial peritonitis, Gastroenterology Journal, vol. 146, no. 7, pp. 1680-1689.
4. O’Shea, R., Darasathy, S., McCullough, A. (2010) AASLD Practice Guidelines: Alcoholic Liver Disease, Hepatology Journal, vol. 51, no. 1, pp. 307-328.