Aetiologies and Pathophysiology of chronic liver disease Flashcards

1
Q

What is chronic liver disease?

A

Duration greater than 6 months
Outcome - progression to cirrhosis

Pathology - recurrent inflammation and repair with fibrosis and regeneration

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2
Q

Name some causes of chronic liver disease?

A
Alcohol 
Hep C 
Primary biliary cirrhosis 
Autoimmune hepatitis 
Hep B
NAFLD (non-alcoholic fatty liver disease)
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3
Q

What can chronic liver disease lead to?

A

Cirrhosis

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4
Q

What is NAFLD?

A

Fatty liver or stet-hepatitis in absence of other cause

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5
Q

Describe the 2 hit paradigm pathogenesis of NAFLD?

A

First hit - excess fat accumulation

Second hit - intrahepatic oxidative stress, lipid per oxidation, TNF-alpha, cytokine cascade

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6
Q

Describe how you might treat simple steatosis?

A
Diagnosis by ultrasound
No liver outcomes
Increased cardiovascular risks
Treatment
***Weight loss & exercise
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7
Q

How would you treat NASH (non alcoholic steatohepatitis)?

A
Diagnosis at present by liver biopsy
Risk of progression to cirrhosis
Treatment
***Weight loss & exercise
Other treatments experimental
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8
Q

Name some auto-immune liver diseases?

A
Primary biliary cholangitis 
Auto-immune hepatitis
Primary sclerosing cholangitis 
Alcohol related liver disease 
Drug reactions
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9
Q

What does auto-immune mean?

A

relating to disease caused by antibodies or lymphocytes produced against substances naturally present in the body.

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10
Q

Describe the presentation of PBC?

A

Middle ages women
Usually asymptomatic

Fatigue
Itch without a rash
Xanthesalmas and xanthomas

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11
Q

How do you diagnose PBC?

A

2 of 3 of….

Positive AMA
Cholestatic LFTs
Liver biopsy

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12
Q

What is the treatment for PBC?

A

Urseodeoxycholic acid

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13
Q

Who is more likely to develop auto-immune hepatitis?

A

Women

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14
Q

Roughly how many people will develop cirrhosis from auto-immune hepatitis?

A

40%

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15
Q

Describe type 1 auto-immune hepatitis?

A

Females more common

Assoc with extra hepatic manifestions

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16
Q

What is the usual clinical presentation of auto-immune hepatitis?

A

Hepatomegaly
Jaundice
Stigmata of chronic liver disease

Elevated AST, ALT, PT

17
Q

How do you diagnose auto-immune hepatitis?

A

Rule out other causes
Liver biopsy
Presence of autoimmune antibodies

18
Q

How do you treat auto-immune hepatitis?

A

Corticosteroids

Azathioprine

19
Q

What is usually the prognosis like with people who have auto-immune hepatitis?

A

40% develop cirrhosis
54% oesophageal varices
13-20% have spontaneous resolution

20
Q

What is primary sclerosing cholangitis?

A

Autoimmune destructive disease of large and medium sized bile ducts.

Progressive fibrosis and obliteration of the biliary tracts

21
Q

What does cholangitis mean?

A

Inflammation of the bile ducts

22
Q

Who is primary sclerosing cholangitis more common in?

A

Males and people who also have UC

23
Q

How do you diagnose primary sclerosing cholangitis?

A

Imaging of the biliary tree

24
Q

What is the treatment of primary sclerosing cholangitis?

A

Maintain bile flow, monitor for cholangiocarcinoma and coli-rectal cancer

25
Q

What is haemochromatosis?

A

Genetic iron overload syndrome

26
Q

What gene mutations are responsible for haemochromatosis?

A

Mutations in the HFE gene

27
Q

Is wilsons disease a recessive or dominant disorder?

A

autosomal recessive

28
Q

What causes wilsons disease?

A

loss of function or loss of protein mutations in ceruloplasmin (copper binding proteins – loss of copper regulation leads to massive tissue deposition of copper, especially in liver and basal ganglia)

29
Q

What is alpha 1 anti-trypsin deficiency?

A

Genetic, mutations in the A1AT genes

Commonly affects lungs (Emphysema) and liver

30
Q

How do you treat A1AT deficiency?

A

Supportive

31
Q

What is Budd-Chiari syndrome?

A

Hyper-coagulation in the hepatic vein causing clotting (thrombosis of the hepatic veins)

congenital webs

32
Q

What might some of the clinical symptoms be in Budd-Chiari be?

A

Acute

  • jaundice
  • tender hepatomegaly

Chronic
- ascites

33
Q

How do you diagnose Budd-Chiari?

A

U/S visualisation of hepatic veins - showing occlusion

34
Q

How do you treat budd Chiari?

A

TIPS

Recanculisation

35
Q

What is methotrexate usually used to treat?

A

Rheumatoid arthritis and psoriasis

36
Q

What must you be careful about when using methotrexate?

A

Dose dependent liver toxin - can cause progressive fibrosis

37
Q

What is cardiac cirrhosis?

A

Secondary to high right heart pressures

38
Q

What are the clinical symptoms of cardiac cirrhosis?

A

CCF

Too much ascitites and or liver impairment

39
Q

How do you treat cardiac cirrhosis?

A

Treat the cardiac condition