Hepatobiliary disease Flashcards

1
Q

What is Wilson’s disease?

A

An autosomal recessive disorder of hepatic copper disposition caused by mutations in the gene ATP7B, located on chromosome 13

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2
Q

Describe the potential complications of Wilson’s disease?

A

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

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3
Q

Describe liver cirrhosis

A

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

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4
Q

Give the common causes of liver cirrhosis

A
Alcohol abuse
Hepatitis B/C
Non alcoholic fatty liver disease
  - Non alcoholic steatohepatitis (end stage NAFLD)
Cryogenic (unknown causes)
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5
Q

What proportion of liver cirrhosis cases are cryogenic (or unknown cause)?

A

50%

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6
Q

Give some less common causes of liver cirrhosis

A

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)

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7
Q

What are the possible early symptoms of cirrhosis?

A
Fatigue
Malaise
Anorexia
Nausea
Weight loss
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8
Q

How may advanced decompensated liver disease present?

A
Oedema, Ascites
Easy bruising (due to lack of clotting factors)
Poor concentration and memory
Bleeding oesophageal varices
Spontaneous bacterial peritonitis
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9
Q

What are the cardinal symptoms of liver failure?

A

Hepatic encephalopathy
Jaundice
Ascites
Variceal bleeding

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10
Q

Describe the causes of liver failure

A

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

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11
Q

List the types of gallstones and describe their composition and/or characteristics

A

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

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12
Q

Describe the general composition of gallstones

A

Contain a mixture of cholesterol, bile pigments (bilirubin) and phospholipids

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13
Q

What is biliary colic?

A

“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

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14
Q

What is gallstone ileus?

A

Obstruction of bowel due to gallstones

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15
Q

What are the potential complications of having gallstones in the bile duct?

A
Acute pancreatitis
Ascending cholangitis (medical emergency)
Obstructive jaundice (painful)
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16
Q

Describe the non-operative management of gallstones

A

Dissolution using oxicolic acid
- dissolve gallstones to reduce their size, allowing
bile to pass
- usually used for patients who are unfit for surgery

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17
Q

What is cholecystitis and what is the most common cause?

A

Inflammation of the gallbladder (can be acute or chronic)

Most commonly caused by blockage of the cystic duct, usually by gallstones

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18
Q

What is cholangitis?

A

Inflammation of the biliary tree

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19
Q

Describe the presentation of acute cholecystitis

A

Continuous epigastric and/or right hypochondric pain
Nausea +/- vomiting
Fever, may have a raised WBC count
Gallbladder may be palpable

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20
Q

What are the potential complications of cholecystitis?

A

Progression to chronic cholecystitis
Cholangitis
Obstructive (post-hepatic) jaundice
Secondary biliary cirrhosis (if prolonged)
Empyema - gallbladder fills with pus due to obstruction

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21
Q

What physical sign may be present in cases of paracetamol overdose?

A

Jaundice

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22
Q

What are the aims of treatment in cirrhosis patients?

A

To delay progression of cirrhosis

To prevent or treat complications

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23
Q

Describe the management and treatment options available for cirrhosis patients

A

Specific treatment for underlying cause
Ensure adequate nutrition, including high protein and micro-nutrient supplements
Abstain from drinking alcohol
Colestyramine to treat pruritus (itching)
- mild pruritus can be treated with antihistamine or
with topical ammonium lactate
Treatment to prevent osteoporosis
Vaccination against hep A, influenza and pneumococci
Liver transplantation - end stage liver disease

24
Q

List the possible features of alcoholic hepatitis

A
Malaise and fatigue
Hepatomegaly
Ascites
Mild liver enzyme abnormalities
Mild fever
In more severe cases there may also be:
Jaundice
Hepatic encephalopathy
Elevated INR due to low levels of clotting factors
Weight loss
GI bleeding
25
Q

Describe the histological appearance of a liver that is affected by alcoholic hepatitis

A

Mallory’s hyaline (clumping of organelles)
Balloon degeneration (hepatocycte cytoplasms become swollen and granular)
Neutrophil infiltration

26
Q

What are the three stages of alcoholic liver disease?

A

Alcoholic fatty liver disease
Alcoholic hepatitis
Cirrhosis

27
Q

List the possible features of alcohol-induced cirrhosis, other than those found in cirrhosis of any cause

A

Peripheral neuropathy
Wernicke’s encephalopathy
Korsakoff’s psychosis (amnesia, confabulation, apathy)
Hypogonadism and feminisation (in men)
AST exceeds ALT
Relatively low ALT due to dietary deficiency of vit-B6

28
Q

What is Wernicke’s encephalopathy

A

An encephalopathy associated with alcohol-induced liver cirrhosis
Features include;
- ataxia
- opthalmoplegia (paralysis of eye muscles)
- confusion
- impaired short-term memory

29
Q

Which class of drugs can be used to treat alcohol withdrawal?

A

Benzodiazepines

30
Q

Describe the treatment for alcoholic liver disease

A

Abstinence is key
Supportive care - drug therapy shown to be of little use
- good nutrition
- vitamin supplementation, particularly B6
Prednisolone for moderate-severe hepatitis (although this is controversial)
Treat complications as they arise e.g. infection

31
Q

Describe the potential complications of liver disease (particularly cirrhosis)

A

Anaemia, thrombocytopenia and coagulopathy
Oesophageal varices
Ascites
Spontaneous bacterial peritonitis (from ascites)
Hepatocellular carcinoma
(also note that surgery and general anaesthesia carry greater risk in patients with cirrhosis)

32
Q

How does alcohol damage the liver?

A

Main mechanism is through alcohol-induced oxidative stress, due to:

  • changes in redox state of hepatocytes
  • elevation of iron levels in hepatocytes
  • reactive acetaldehyde derivatives
  • mitochondrial damage
  • reduced cellular antioxidants
  • actions of inflammatory cytokines, adipokines and hormones
33
Q

What causes dark urine and pale stools in a patient presenting with jaundice?

A

Obstructive jaundice

cholestasis due to gallstones or some other obstruction of the bile duct

34
Q

Give three classifications of bile duct obstruction and suggests a few examples for each

A

Luminal obstruction
- gallstones, parasites, iatrogenic causes
Mural obstruction (duct wall)
- congenital atresia, traumatic stricture, sclerosing cholangitis, cholangiocarcinoma
Extramural obstruction
- carcinoma in head of pancreas or ampulla of Vater
- pancreatitis
- tumour of the porta hepatis

35
Q

If a jaundiced patient has normal liver function tests, what is most likely to be causing the jaundice?

A

Pre-hepatic cause, e.g. haemolytic anaemia, pernicious anaemia, congenital deficiency of conjugating enzymes

36
Q

Give two congenital syndromes that can cause pre-hepatic (unconjugated) jaundice

A

Deficiencies of conjugating enzymes:

  • Gilbert’s syndrome
  • Crigler Najjar syndrome
37
Q

What is Budd-Chiari syndrome?

A

Blood-clots completely or partially block blood flow from the liver, either in the small or large hepatic veins.
This can cause hepatomegaly, oedema/ascites, oesophageal varices (which can bleed), RUQ pain. It is a potential cause of acute liver failure and renal impairment.

38
Q

What is “caput medusae” and what can cause it?

A

distended and engorged umbilical veins which are seen radiating from the umbilicus across the abdomen.
Severe portal hypertension causes portal-systemic shunting via the umbilical veins.

39
Q

How do you distinguish between Caput Medusae and collateral veins caused by IVC obstruction?

A

By determining the direction of blood flow in umbilical veins:

Caput Medusae; blood flows towards legs
IVC obstruction; blood flows towards head

40
Q

What pressure is the portal venous system normally under?

A

7-14 mm Hg

41
Q

Give two common causes (worldwide) of portal hypertension

A

UK: liver cirrhosis
Worldwide: schistosomiasis

42
Q

Which biliary disease is a medical emergency?

A

Acute (ascending) cholangitis

43
Q

What is Primary Sclerosing Cholangitis?

A

A rare disease of unknown aetiology characterised by chronic inflammation and fibrosis of the bile duct

44
Q

Which drug can be used to relieve pruritus?

A

cholestyramin

45
Q

What are the complications of primary sclerosing cholangitis?

A

secondary biliary cirrhosis
portal hypertension
cholangitis
10% will develop cholangiocarcinoma

46
Q

What does ERCP stand for?

A

Endoscopic Retrograde Cholangio-Pancreatography

47
Q

Describe how an ERCP is conducted

A

An endoscope placed into the duodenum via the mouth/oesophagus/stomach. In the duodenum, a cannula is passed through the endoscope and through the ampulla of Vater. Contrast is injected and X-rays are taken to study the ducts of the pancreas and liver.

48
Q

Give five organisms that most commonly cause acute cholangitis

A
Coliforms:
- Escherichia coli (most common)
- Klebsiella spp.
- Enterobacter spp
Enterococci
Streptococci
49
Q

What two conditions are necessary to cause acute cholangitis?

A
  1. Bile stasis due to chronic obstruction (e.g. gallstones)

2. Bacterial growth in bile

50
Q

What is the major concern in a patient with acute cholangitis?

A

Risk of developing septicaemia, sepsis and septic shock

51
Q

Describe the typical presentation of acute cholangitis

A

Charcot’s triad:

  1. Jaundice
  2. Fever
  3. Right upper quadrant pain
52
Q

What would you expect to find on imaging of a patient with acute cholangitis?

A

Dilatation of the biliary tree

May see evidence of the underlying cause of obstruction e.g. gallstones, stricture, tumour

53
Q

What empirical antibacterial therapy is generally used in the initial management of acute cholangitis?

A

Broad spectrum
cephalosporin plus metronidazole

If penicillin-allergic: quinolone (e.g. ciprofloxacin or levofloxacin) plus metronidazole

54
Q

What organisms most commonly infect the biliary tract and what antimicrobial agent is used to treat this?

A

Coliforms (rod-shaped Gram-negative non-spore forming bacteria)
Co-amoxiclav (unless penicilin-allergic)

55
Q

What is a PTC scan?

A

Percutaneous transhepatic cholangiography

- radiographic technique employed in visualisation of the biliary tract