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Level 2 of 6Must-remember

Decompensated chronic liver disease

Assess, manage and stay safe — enough on its own

The card — assess, manage, caution

Assessment— look, ask, measure

  • Know what decompensation means: a patient with cirrhosis is decompensated once they develop ascites, variceal haemorrhage, jaundice or hepatic encephalopathy — and in practice bacterial infection (especially spontaneous bacterial peritonitis) and acute kidney injury belong on the same list, carrying equal prognostic weight and being far more easily missed.
  • Hunt the precipitant, because decompensation is almost never spontaneous: infection (SBP, chest, urinary, skin, line — the commonest single trigger and frequently occult), gastrointestinal haemorrhage, alcoholic hepatitis or continued drinking, dehydration from over-diuresis, diarrhoea or paracentesis without albumin, constipation, hypokalaemia, hyponatraemia, acute kidney injury, sedatives, opioids, antidepressants, antihistamines, NSAIDs and aspirin, high-sodium drugs, recent surgery, dental procedures or TIPS, portal vein thrombosis (10–25% of cirrhotics) and hepatocellular carcinoma (3–5% per year).
  • History to ask for: known liver disease and its cause, alcohol intake and the time of the last drink, every drug including over-the-counter NSAIDs and sedatives, black stool or vomited blood, when the bowels last opened, and a family account of confusion, sleep-wake reversal or personality change (the patient will not report it).
  • Look for the stigmata: multiple spider telangiectasia in the superior vena caval territory (a central arteriole with radiating vessels refilling from the centre), palmar erythema, bounding pulse and warm peripheries, gynaecomastia, testicular atrophy, jaundice, a small shrunken irregular liver, splenomegaly, ascites with shifting dullness, caput medusae, hepatic fetor, bruising, leuconychia, Dupuytren contracture, pigmentation or Kayser-Fleischer rings — but one or two spiders occur in up to 15% of healthy young adults and clubbing alone is not a sign of cirrhosis.
  • Grade the encephalopathy: grade 1 poor concentration, slurred speech, slow mentation, disordered sleep rhythm; grade 2 drowsy but easily rousable, lethargic, occasional aggression, flapping tremor; grade 3 marked delirium, sleepy but responds to voice and pain, gross disorientation, flapping tremor; grade 4 unresponsive to voice, may or may not respond to pain, unconscious.
  • Asterixis — a coarse flapping tremor of the outstretched, dorsiflexed hands — is characteristic but disappears in deep coma; constructional apraxia is tested by asking the patient to copy a five-pointed star.
  • Hepatic encephalopathy does not cause focal neurological signs and seizures are rare: either finding demands an alternative explanation and a CT head.Not available at your setup — CT scan.
  • Capillary glucose immediately and two-hourly: impaired gluconeogenesis makes hypoglycaemia common and it mimics encephalopathy exactly; a value below 4.0 mmol/L is a danger sign, and children with liver disease have small glycogen stores and become hypoglycaemic far faster than adults.
  • Judge perfusion against the patient's own physiology: the hyperdynamic circulation means 100/60 mmHg may represent shock in someone whose baseline is 90/50, and blunted baroreceptor reflexes mean the expected tachycardia may be absent — use capillary refill, mental state, lactate and urine output.Not available at your setup — Arterial blood gas. If blood gas/lactate is unavailable, judge perfusion by capillary refill, mental state and urine output alone.
  • Danger signs: haematemesis or melaena; encephalopathy grade 3 or 4 (an unprotected airway); glucose below 4.0 mmol/L; oliguria under 0.5 mL/kg/hour or a creatinine rise of 26.5 micromol/L or more; potassium above 6.0 mmol/L; sodium below 125 mmol/L; hypotension or a rising lactate; new focal neurological signs; a tense tender abdomen with fever.Not available at your setup — Serum electrolytes, Renal function (creatinine/urea).
  • Spontaneous bacterial peritonitis is the great mimic: abdominal pain and fever are absent in roughly one-third of cases, and the only manifestation may be new confusion, a creeping creatinine, unexplained hypotension, or simply that the patient is not right — any deterioration in a patient with ascites is SBP until excluded.
  • Do a diagnostic paracentesis in every patient with new ascites, every patient admitted with a complication of cirrhosis, and every patient with known ascites who deteriorates in any way: 10–20 mL through a 21 G needle in the left lower quadrant, lateral to the rectus sheath and clear of scars and visible collaterals. Coagulopathy is not a contraindication.Doctor / Nurse
  • An ascitic neutrophil count above 250 × 10⁶/L is neutrocytic ascites and is sufficient by itself to start antibiotics; inoculate blood culture bottles at the bedside for an 80–90% culture yield rather than sending a plain container.Not available at your setup — Blood culture.
  • Bedside severity — Child-Pugh: bilirubin under 34 / 34–50 / over 50 micromol/L, albumin over 35 / 28–35 / under 28 g/L, prothrombin time prolonged under 4 / 4–6 / over 6 seconds, and ascites and encephalopathy scored none / mild / marked, one to three points each; grade A is under 7, grade B is 7–9, grade C is 10 or more.Not available at your setup — Liver function tests, Coagulation (PT/INR).
  • Bloods that matter: platelets under 150 × 10⁹/L indicate cirrhosis unless another cause is found; albumin under 28 g/L, sodium under 125 mmol/L and creatinine over 130 micromol/L all mark a poor outlook; the INR measures synthetic function and is not a measure of bleeding risk; alpha-fetoprotein above 200 ng/mL strongly suggests hepatocellular carcinoma.Not available at your setup — Liver function tests, Serum electrolytes, Renal function (creatinine/urea), Coagulation (PT/INR).
  • Consider the mimics of confusion: alcohol withdrawal (tremor, sweating, tachycardia, hallucinations 6–72 hours after the last drink, asterixis absent), Wernicke encephalopathy (ophthalmoplegia and ataxia), subdural haematoma after a fall (focal signs), hypoglycaemia, sepsis-related delirium and drug intoxication — excluded by bedside glucose, a septic screen and the drug history.

Management— do this, in order

  • Airway first in grade 3 or 4 encephalopathy: the airway is not safe — nurse head-up at 30 degrees with suction available and arrange tracheal intubation; if agitation threatens the airway the answer is airway protection, not a benzodiazepine.Doctor / NurseNot available at your setup — Endotracheal intubation kit.
  • Correct hypoglycaemia and give thiamine before glucose: check capillary glucose immediately and two-hourly, and in every patient with an alcohol history give parenteral thiamine before any glucose-containing fluid.
  • Resuscitate promptly but not over-vigorously: balanced crystalloid or 0.9% sodium chloride in 250–500 mL boluses in adults (10–20 mL/kg in children), reassessed after each, with 20% human albumin the preferred expander. Over-rapid fluid removal or replacement is the commonest iatrogenic disaster in this disease.
  • Stop what is harming the patient: diuretics if creatinine or potassium is rising or sodium falling; non-selective beta-blockers in SBP, hypotension or AKI; all NSAIDs, aspirin and aminoglycosides; sedatives; and high-sodium or sodium-retaining preparations (antacids, alginates, effervescent tablets, penicillins, cephalosporins, valproate, glucocorticoids, oestrogens, metoclopramide).
  • Tap the ascites and treat SBP immediately on a neutrophil count above 250 × 10⁶/L, without waiting for culture: cefotaxime 2 g IV every 8 hours (6-hourly if severe) for 5–7 days, or ceftriaxone 1–2 g IV once daily; piperacillin/tazobactam 4.5 g IV every 8 hours or meropenem 1 g IV every 8 hours where multidrug-resistant organisms are likely, including hospital-acquired SBP.Doctor / Nurse
  • Add intravenous albumin in SBP where there is jaundice or renal impairment: 1.5 g/kg on day 1 and 1.0 g/kg on day 3 — this reduces hepatorenal syndrome and improves survival. Stop non-selective beta-blockers once SBP is diagnosed.Doctor / Nurse
  • Variceal haemorrhage — transfuse to a restrictive target of haemoglobin 80 g/L (children 10 mL/kg of packed red cells, then reassess); over-transfusion raises portal pressure and provokes early rebleeding.Not available at your setup — Blood & blood products.
  • Give prophylactic ceftriaxone 1 g IV daily for 5–7 days to every cirrhotic with gastrointestinal bleeding, infected or not: such patients have a more than 50% chance of severe bacterial infection and this reduces it to 10–20% and reduces hospital mortality.
  • Start terlipressin, the only vasoconstrictor proven to reduce mortality: 2 mg IV every 6 hours, reducing to 1 mg every 4 hours after 48 hours if a prolonged course is needed, for up to 5 days; alternatives are somatostatin 250–500 micrograms/hour by infusion or octreotide (children 1 microgram/kg IV then 1–5 micrograms/kg/hour). Add a proton pump inhibitor and vitamin K (phytomenadione) 10 mg IV once if the prothrombin time is prolonged (children 250–300 micrograms/kg, maximum 10 mg), keep nil by mouth, and arrange endoscopic band ligation for oesophageal varices or cyanoacrylate injection for gastric varices.Doctor / NurseNot available at your setup — Infusion pump.
  • Encephalopathy — treat the precipitant and empty the bowel: lactulose 30–60 mL orally every 2 hours until mental status improves or the first bowel movement, then 10–30 mL three times daily titrated to two or three soft stools per day and no more; in children titrate to the same endpoint from about 0.5 mL/kg (up to 30 mL) two or three times daily. Add rifaximin 550 mg orally twice daily if it persists or recurs, or metronidazole 200 mg orally four times daily where rifaximin is unavailable.
  • Never give oral lactulose to a drowsy patient with an unprotected airway — use a lactulose retention or phosphate enema instead.
  • Ascites — reduce sodium in and increase sodium out, slowly: restrict sodium to ≤2 g (about 100 mmol) per day, give spironolactone 100 mg orally once daily increased stepwise to 400 mg daily (children 1–3 mg/kg/day in 1–2 divided doses, maximum 100–200 mg/day), adding furosemide 40 mg orally daily, range 40–160 mg/day (children 0.5–1 mg/kg/dose once or twice daily, maximum 2 mg/kg/day), keeping roughly a 100:40 ratio to preserve potassium.
  • Aim for no more than 0.5 kg weight loss per day without peripheral oedema and 1.0 kg/day with oedema: the maximum rate at which ascites can be mobilised is 500–700 mL in 24 hours, and faster diuresis produces hypovolaemia, acute kidney injury, hepatorenal syndrome and encephalopathy.
  • Tense or refractory ascites — large-volume paracentesis with albumin cover, 6–8 g of albumin per litre removed (about 100 mL of 20% or 25% human albumin solution for every 2–3 litres drained; the same 1 g/kg principle applies in children); albumin is mandatory after removal of ≥5 litres, up to 20 litres may be removed over 4–6 hours, remove the drain after 6–12 hours, and continue diuretics.Doctor / Nurse
  • Every rise in creatinine is an emergency: stop all diuretics, all beta-blockers and every nephrotoxic drug, catheterise and measure hourly urine output, tap the ascites to exclude SBP, and give intravenous albumin 1 g/kg/day (maximum 100 g) for 2 consecutive days; only if there is no response is hepatorenal syndrome diagnosed, treated with terlipressin 1–2 mg IV every 4–6 hours with albumin.Not available at your setup — Renal function (creatinine/urea), Serum electrolytes.
  • Feed the patient: at least 35 kcal/kg/day (actual weight, discounted for oedema and ascites) and protein 1.2–1.5 g/kg/day (ideal body weight), with a late evening snack to shorten the catabolic overnight fast; children need 120–150% of the estimated average requirement for age. Feed enterally by fine-bore nasogastric tube if oral intake is inadequate.
  • Treat alcohol withdrawal with lorazepam or oxazepam rather than chlordiazepoxide or diazepam, whose half-lives lengthen substantially in advanced liver disease, at the smallest effective dose and with parenteral thiamine first.

Caution— what harms

  • Never sedate the encephalopathic patient: sedation is the enemy, flumazenil is not a treatment for hepatic encephalopathy, and the answer to agitation threatening the airway is intubation, not a benzodiazepine.
  • Never restrict dietary protein: it worsens an already malnourished state and should never be restricted beyond 48 hours; malnutrition affects up to 50% of patients with decompensated cirrhosis and sarcopenia independently predicts infection, encephalopathy and ascites.
  • Never pour oral lactulose into a drowsy patient — use an enema; and never titrate lactulose to diarrhoea, since diarrhoea causes dehydration and hypokalaemia which precipitate further encephalopathy.
  • Do not diurese fast: more than 0.5 kg/day without oedema or 1.0 kg/day with oedema causes hypovolaemia, AKI, hepatorenal syndrome and encephalopathy; stop diuretics if creatinine or potassium rises, sodium falls below 128 mmol/L, or encephalopathy worsens.Not available at your setup — Serum electrolytes, Renal function (creatinine/urea).
  • The INR measures synthetic function, not bleeding risk: prolonged prothrombin time and thrombocytopenia coexist with reduced protein C, protein S and antithrombin and raised von Willebrand factor and factor VIII, so do not transfuse plasma to an INR target in a patient who is not bleeding — give plasma and platelets only for active major haemorrhage, guided by the bleeding rather than the number.Not available at your setup — Coagulation (PT/INR).
  • Do not over-transfuse a variceal bleed: the target is haemoglobin 80 g/L, because a higher haemoglobin raises portal pressure and provokes early rebleeding.
  • Coagulopathy is not a contraindication to diagnostic paracentesis — failing to tap is the error, not tapping.
  • Never assume an absent fever or absent abdominal pain excludes SBP: both are absent in roughly one-third of cases, and the presentation may be nothing more than new confusion or a creeping creatinine.
  • Do not attribute new focal neurological signs to encephalopathy: hepatic encephalopathy does not cause focal signs, and seizures are rare — CT of the head is mandatory for focal signs, a fall, or a decline in consciousness that does not fit.Not available at your setup — CT scan.
  • Do not give NSAIDs, aspirin, aminoglycosides, sedatives, opioids, antidepressants or antihistamines, and stop high-sodium preparations; an NSAID here causes renal failure and gastrointestinal bleeding.
  • Do not attribute a left-sided pleural effusion to ascites: a right-sided effusion may be a hepatic hydrothorax (serum-to-pleural-fluid albumin gradient 11 g/L or more), but a left-sided one needs another explanation.
  • Balloon tamponade is a rescue only: a four-lumen Sengstaken-Blakemore tube achieves haemostasis in up to 90% but causes aspiration pneumonia and oesophageal rupture, demands a protected airway first, and must be left for no more than 12 hours.Doctor / NurseNot available at your setup — Endotracheal intubation kit.
  • Terlipressin: abdominal colic, defecation and facial pallor are expected effects of vasoconstriction, not allergy — do not stop the drug for them; but avoid terlipressin in ischaemic heart disease.
  • Do not miss hypoglycaemia, especially in children: small glycogen stores make it the commonest reversible cause of drowsiness in a child with liver disease, and it mimics encephalopathy exactly. Avoid neomycin for encephalopathy, and do not use vaptans routinely — they do not affect mortality, complications or renal failure.

Refer / escalate

Refer or escalate urgently for haematemesis or melaena, grade 3-4 encephalopathy with an unprotected airway, any rise in creatinine or oliguria under 0.5 mL/kg/hour, potassium above 6.0 mmol/L, sodium below 125 mmol/L, hypotension or rising lactate, new focal signs, or an ascitic neutrophil count above 250 × 10⁶/L; and refer for transplant assessment in Child-Pugh C, MELD ≥20, UKELD ≥49, once ascites appears, or after a first episode of spontaneous bacterial peritonitis.

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