Hepatobiliary disease Flashcards
What is Wilson’s disease?
An autosomal recessive disorder of hepatic copper disposition caused by mutations in the gene ATP7B, located on chromosome 13
Describe the potential complications of Wilson’s disease?
Causes low serum copper concentrations but a build up of copper in the liver
This hepatic retention of copper causes liver injury which can eventually lead to cirrhosis
Describe liver cirrhosis
End stage chronic liver disease, caused by the liver’s response to chronic injury
Characterised by fibrosis and the conversion of normal liver tissue to structurally abnormal nodules
Give the common causes of liver cirrhosis
Alcohol abuse Hepatitis B/C Non alcoholic fatty liver disease - Non alcoholic steatohepatitis (end stage NAFLD) Cryogenic (unknown causes)
What proportion of liver cirrhosis cases are cryogenic (or unknown cause)?
50%
Give some less common causes of liver cirrhosis
Immune-mediated liver disease
- e.g. auto-immune hepatitis, primary biliary cholangitis
Diabetes mellitus
Inherited metabolic disorders
- primary haemochromatosis (excess iron)
- Wilson’s disease (excess copper)
What are the possible early symptoms of cirrhosis?
Fatigue Malaise Anorexia Nausea Weight loss
How may advanced decompensated liver disease present?
Oedema, Ascites Easy bruising (due to lack of clotting factors) Poor concentration and memory Bleeding oesophageal varices Spontaneous bacterial peritonitis
What are the cardinal symptoms of liver failure?
Hepatic encephalopathy
Jaundice
Ascites
Variceal bleeding
Describe the causes of liver failure
Liver failure is a complication of liver injury:
Acute:
- hepatitis due to drugs/alcohol, viruses or other toxins
- bile duct obstruction (bile is toxic to liver cells)
Chronic:
- cirrhosis
List the types of gallstones and describe their composition and/or characteristics
Cholesterol stones - contain mostly cholesterol, are
large, often solitary and radiolucent (transparent to
x-rays)
Pigment stones - black or brown
- black stones can be caused by haemolysis and/or
cirrhosis
- brown stones are the result of stasis and infection
within the biliary system
Mixed stones - composed of calcium salts, pigment and
cholesterol
Describe the general composition of gallstones
Contain a mixture of cholesterol, bile pigments (bilirubin) and phospholipids
What is biliary colic?
“gallbadder attack” - epigastric pain due to a gallstone temporarily blocking a bile duct.
Pain can radiate to shoulder
Usually lasts one to a few hours
What is gallstone ileus?
Obstruction of bowel due to gallstones
What are the potential complications of having gallstones in the bile duct?
Acute pancreatitis Ascending cholangitis (medical emergency) Obstructive jaundice (painful)
Describe the non-operative management of gallstones
Dissolution using oxicolic acid
- dissolve gallstones to reduce their size, allowing
bile to pass
- usually used for patients who are unfit for surgery
What is cholecystitis and what is the most common cause?
Inflammation of the gallbladder (can be acute or chronic)
Most commonly caused by blockage of the cystic duct, usually by gallstones
What is cholangitis?
Inflammation of the biliary tree
Describe the presentation of acute cholecystitis
Continuous epigastric and/or right hypochondric pain
Nausea +/- vomiting
Fever, may have a raised WBC count
Gallbladder may be palpable
What are the potential complications of cholecystitis?
Progression to chronic cholecystitis
Cholangitis
Obstructive (post-hepatic) jaundice
Secondary biliary cirrhosis (if prolonged)
Empyema - gallbladder fills with pus due to obstruction
What physical sign may be present in cases of paracetamol overdose?
Jaundice
What are the aims of treatment in cirrhosis patients?
To delay progression of cirrhosis
To prevent or treat complications