Liver Disease Flashcards
Define acute liver disease.
Acute failure of the hepatic cells to maintain normal function, also called fulminant hepatitis.
Define chronic liver disease.
Chronic disease of the hepatic cells -> decrease in overall liver function.
Explain the aetiology/risk factors for acute liver disease.
Infection: Acute viral hepatitis (A, B); EBV may precipitate infectious ucleosis hepatitis.
Drugs/inadvertent poisoning: Paracetamol, isoniazid, halothane and Amanita phalloides (poisonous mushrooms).
Reye syndrome: There is convincing evidence that aspirin given to patients <14 years of age is associated with an acute non-inflammatory encephalopathy with associated liver damage (especially with concomitant varicella infection). (Microvesicular fatty infiltration of the liver)
Explain the aetiology/risk factors for chronic liver disease.
Viral hepatitis: HBV, HCV (death of hepatocytes at an interface between parenchyma and connective tissue, with infiltration of plasma cells and lymphocytes).
Drugs: NSAIDs, antibiotics (nitrofurantoin), anticonvulsants, paracetamol.
Autoimmune disease of parenchyma (autoimmune hepatitis) or biliary tree (primary sclerosing cholangitis) (Inflammatory cell infiltrate with hepatocellular necrosis).
Wilson disease: Genetic disease of copper metabolism which -> deposition of copper in the liver, brain, kidneys and cornea. (Autosomal recessive disorder with multiple mutations on chromosome 13 -> a reduced synthesis of caeruloplasmin (copper-binding protein) and defective excretion of copper in bile.
Cystic fibrosis
Summarise the epidemiology of acute liver disease.
Acute hepatitis is uncommon in children.
Summarise the epidemiology of HBV in children.
5% of the world’s population has chronic HBV infection. Age at infection determines the rate of progression from acute to chronic infection: 90% in the perinatal period, 20–50% in children aged 1–5 years, and < 5% in adults.
Summarise the epidemiology of HCV in children.
1% worldwide chronic infection, with development of cirrhosis and hepatocellular carcinoma in a number of cases, after an interval of 10–15 years.
Summarise the epidemiology of autoimmune hepatitis in children.
0.1–1.2/100,000. M: F.1 : 4. Peak ages: 10–20 years and 45–70 years.
Summarise the epidemiology of Wilson’s Disease.
1/30,000 live births.
What are the presenting signs and symptoms of acute liver disease?
General: May present within hours with jaundice, encephalopathy, coagulopathy, hypoglycaemia or other electrolyte disturbances.
Encephalopathy:
- Young children: There may be a history of alternating periods of irritability and confusion with drowsiness
- Older children: May have a history of aggression and being unusually difficult.
What are the presenting signs and symptoms of chronic liver disease?
General: Failure to thrive, lethargy, loss of fat and muscle bulk.
GI: Distended abdomen (ascites/hepatosplenomegaly), scrotal swelling, dilated abdominal veins (portal hypertension).
Autoimmune hepatitis: Skin rash, lupus erythematosus, arthritis, haemolytic anaemia or nephritis.
Wilson disease: Kayser–Fleischer rings in the corneas at >7 years, neurological features >12 years such as speech changes, tremor, difficulty with fine motor tasks and gait.
What are appropriate investigations for acute liver disease?
Bloods: Increased bilirubin (although may be normal in the early stages), deranged clotting, increased transaminases (ALT, AST), increased ALP, increased plasma ammonia, and decreased glucose.
ABG sampling: For frequently associated acid/base imbalance.
Viral serology: To detect hepatitis strain. CT/MRI brain: May show cerebral oedema in encephalopathy.
Other: EEG may show acute hepatic encephalopathy.
What are appropriate investigations for chronic liver disease?
HBV/HCV: Serology and surface/core antigen screen.
Autoimmune hepatitis: Hypergammaglobulinaemia (IgG >20 g/l), autoantibodies, smooth muscle cell autoantibodies, antinuclear antibodies, liver/kidney microsomal antibodies.
Wilson disease: Decreased serum caeruloplasmin, decreased serum copper, increased urinary copper, increased hepatic copper.
What is the management for acute liver disease?
Treatment of complications, including the following:
- Hypoglycaemia: Dextrose infusion.
- Sepsis: IV broad-spectrum antibiotics.
- Coagulation defect: FFP and H2-blockers/proton pump inhibitors to prevent gastric bleed. Vitamin K is avoided unless necessary as may mask deterioration in clotting factors, whichis used as an indication for liver transplantation.
- Cerebral oedema: Fluid restriction and diuresis with mannitol.
- Liver transplantation: With worsening clinical, biochemical and clotting profile; prothrombin time is the best marker of liver failure.
What is the management for chronic liver disease?
HBV: Immunisation for at-risk infants. Supportive management . alpha-interferon.
HCV: alpha-interferon. No vaccine is available. Autoimmune hepatitis: 90% of children respond to prednisolone and azathioprine.
Wilson disease: Penicillamine reduces hepatic and CNS copper deposition, zinc reduces copper absorption, pyridoxine prevents peripheral neuropathy. All may require liver transplantation in end-stage liver disease.
