Chapter 46 END-STAGE LIVER DISEASE

KEY POINTS

• End-stage liver disease is a complication of cirrhosis that is due to a variety of aetiologies, including chronic viral hepatitis, alcoholic liver disease, non-alcoholic fatty liver disease, autoimmune disease and metabolic disease.

• The manifestations of end-stage liver disease are a consequence of hepatic synthetic failure and portal hypertension.

• Common manifestations of end-stage liver disease include portal hypertensive bleeding, fluid retention and ascites, spontaneous bacterial peritonitis, hepatorenal syndrome and hepatic encephalopathy.

• Patients with cirrhosis should be monitored for manifestations of end-stage liver disease so that appropriate measures can be implemented to prevent and treat complications. This includes screening for oesophageal varices and hepatocellular carcinoma, introduction of prophylactic antibiotics in select circumstances and regular monitoring of renal and liver function. Concomitant therapies should be monitored carefully.

INTRODUCTION

End-stage liver disease is also known as decompensated liver disease. It develops in patients with underlying cirrhosis due to a wide variety of causes, including chronic hepatitis C, chronic hepatitis B, alcoholic liver disease, non-alcoholic fatty disease, autoimmune liver diseases (autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis) and genetic or metabolic liver diseases (alpha₁-antitrypsin deficiency, genetic haemochromatosis, Wilson’s disease). Despite the wide aetiological processes involved in the development of cirrhosis, the manifestations of end-stage liver disease are common to all, and are consequent to the combination of portal hypertension and hepatic synthetic failure (Table 46.1). Extrahepatic manifestations of end-stage liver disease include hepatopulmonary syndrome and portopulmonary hypertension. In addition, the presence of cirrhosis predisposes to hepatocellular carcinoma (HCC), bone disease and malnutrition.

TABLE 46.1 Manifestations of end-stage liver disease

Complications of portal hypertension and portosystemic shunting • Ascites • Spontaneous bacterial peritonitis • Hepatorenal syndrome • Oesophageal and gastric varices • Hepatic encephalopathy • Hepatopulmonary syndrome and portopulmonary hypertension

Consequences of hepatic synthetic failure • Hypoalbuminaemia • Hypoprothrombinaemia and coagulopathy • Hyperbilirubinaemia

ASSESSMENT

History

The essential elements of history taking for patients with end-stage liver disease (Figure 46.1) include:

• Likely aetiology and duration of liver disease:

– History of alcohol intake (age of onset, daily intake, last use)

– History of injection drug use (age of onset, last use)

– Ethnicity and country of birth

– Family history of liver disease

– History of obesity, features of metabolic syndrome.

• Precipitants of recent deterioration:

– Recent alcohol abuse

– Recent illness, including infections, diarrhoea, vomiting, constipation

– Recent medication history, including diuretics, sedatives, analgesics, non-steroidal antiinflammatories

– History of gastrointestinal bleeding

– Nutritional intake, including salt intake, protein intake.

FIGURE 46.1 Assessment of end-stage liver disease.

CT = computed tomography; K = potassium, MRI = magnetic resonance imaging; U = urea.

Physical examination

Assessment of patient with end-stage liver disease (Figure 46.1) should include examination for:

• Palmar erythema

• Finger clubbing (presence indicative of hepatopulmonary syndrome)

• Spider naevi

• Gynaecomastia, loss of body hair, testicular atrophy (in males)

• Cardiovascular assessment (usually reveals hyperdynamic circulation, with warm peripheries and mild tachycardia and hypotension)

• Signs of pulmonary hypertension (may be present in 2%–10% of patients with portal hypertension)

• Hepatosplenomegaly

• Fluid retention (ascites, peripheral oedema, pleural effusion)

• Hernias (periumbilical, inguinal)

• Malnutrition (loss of subcutaneous fat deposits, muscle wasting)

• Signs of hepatic encephalopathy (flapping tremor, cognitive changes)

• Fetor hepaticus.

Laboratory evaluation

In addition to the investigations listed, other specific investigations will be required to determine the cause of liver disease (Figure 46.1).

Haematology

• Full blood count (leucopenia and thrombocytopenia reflect portal hypertension).

• Coagulation profile (prolongation of international normalised ratio [INR] reflects hepatic synthetic failure or inadequate vitamin K absorption).

Serum biochemistry

• Sodium, potassium, urea, creatinine.

• Bilirubin, albumin, liver enzymes.

• Serum alpha-fetoprotein (elevation may reflect HCC, or be related to liver inflammation in chronic viral hepatitis).

Microbiology

• Fluid cultures (blood, urine, ascites, etc, if sepsis suspected, or change in clinical condition).

Evaluation of ascitic fluid

• A diagnostic paracentesis involves the removal of 20–40 mL of ascitic fluid.

• Fluid should be sent for a cell count, albumin, protein and culture (by inoculation into blood culture bottles).

• An ascitic white cell count (WCC) >500 cells/mm³ or neutrophil count >250 cells/mm³ indicates the presence of infection (usually spontaneous bacterial peritonitis).

• A serum to ascites albumin gradient (SAAG) of >11 g/L is consistent with ascites due to portal hypertension, rather than malignancy or infection.

• An ascites protein concentration of <10 g/L is associated with a high risk for spontaneous bacterial peritonitis.

Radiologic investigations

• Ultrasound of upper abdomen: a screening test to identify space occupying lesions in the liver, portal vein patency, the presence of ascites, splenomegaly.

• Triple-phase computed tomography (CT) scan of the abdomen: a more sensitive test for identification of hepatocellular carcinoma (classically displays enhancement on arterial phase, and a defect on portal phase), lymphadenopathy, ascites, vascular shunts.

• Magnetic resonance imaging (MRI): can be used safely in patients with renal impairment. More sensitive than ultrasound for detection of hepatocellular carcinoma.

• Portal vein pressure measurements: used in some specialist centres for quantitation of portal hypertension. Involves hepatic vein cannulation, usually via the right external jugular vein. Clinically significant portal hypertension is a hepatic vein–portal vein pressure gradient (HVPG) >10 mmHg (normal 3–6 mmHg). Varices rarely bleed until HVPG >12 mmHg.

Cardiorespiratory evaluation

Shortness of breath is a common symptom in patients with end-stage liver disease. Specific investigations of the cardiorespiratory systems may include:

• Chest X-ray

• CT chest

• Echocardiography

• Full respiratory function tests

• Arterial blood gases on room air

• If hepatopulmonary syndrome is suspected then:

– Contrast-enhanced ‘bubble’ echocardiography

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