Aetiologies and Pathophysiology of chronic liver disease

Question 1

What is chronic liver disease?

Answer 1

Duration greater than 6 months
Outcome - progression to cirrhosis

Pathology - recurrent inflammation and repair with fibrosis and regeneration

Question 2

Name some causes of chronic liver disease?

Answer 2

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

Question 3

What can chronic liver disease lead to?

Answer 3

Cirrhosis

Question 4

What is NAFLD?

Answer 4

Fatty liver or stet-hepatitis in absence of other cause

Question 5

Describe the 2 hit paradigm pathogenesis of NAFLD?

Answer 5

First hit - excess fat accumulation

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

Question 6

Describe how you might treat simple steatosis?

Answer 6

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

Question 7

How would you treat NASH (non alcoholic steatohepatitis)?

Answer 7

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

Question 8

Name some auto-immune liver diseases?

Answer 8

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

Question 9

What does auto-immune mean?

Answer 9

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

Question 10

Describe the presentation of PBC?

Answer 10

Middle ages women
Usually asymptomatic

Fatigue
Itch without a rash
Xanthesalmas and xanthomas

Question 11

How do you diagnose PBC?

Answer 11

2 of 3 of….

Positive AMA
Cholestatic LFTs
Liver biopsy

Question 12

What is the treatment for PBC?

Answer 12

Urseodeoxycholic acid

Question 13

Who is more likely to develop auto-immune hepatitis?

Answer 13

Women

Question 14

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

Answer 14

40%

Question 15

Describe type 1 auto-immune hepatitis?

Answer 15

Females more common

Assoc with extra hepatic manifestions

Question 16

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

Answer 16

Hepatomegaly
Jaundice
Stigmata of chronic liver disease

Elevated AST, ALT, PT

Question 17

How do you diagnose auto-immune hepatitis?

Answer 17

Rule out other causes
Liver biopsy
Presence of autoimmune antibodies

Question 18

How do you treat auto-immune hepatitis?

Answer 18

Corticosteroids

Azathioprine

Question 19

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

Answer 19

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

Question 20

What is primary sclerosing cholangitis?

Answer 20

Autoimmune destructive disease of large and medium sized bile ducts.

Progressive fibrosis and obliteration of the biliary tracts

Question 21

What does cholangitis mean?

Answer 21

Inflammation of the bile ducts

Question 22

Who is primary sclerosing cholangitis more common in?

Answer 22

Males and people who also have UC

Question 23

How do you diagnose primary sclerosing cholangitis?

Answer 23

Imaging of the biliary tree

Question 24

What is the treatment of primary sclerosing cholangitis?

Answer 24

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

Question 25

What is haemochromatosis?

Answer 25

Genetic iron overload syndrome

Question 26

What gene mutations are responsible for haemochromatosis?

Answer 26

Mutations in the HFE gene

Question 27

Is wilsons disease a recessive or dominant disorder?

Answer 27

autosomal recessive

Question 28

What causes wilsons disease?

Answer 28

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)

Question 29

What is alpha 1 anti-trypsin deficiency?

Answer 29

Genetic, mutations in the A1AT genes Commonly affects lungs (Emphysema) and liver

Question 30

How do you treat A1AT deficiency?

Answer 30

Supportive

Question 31

What is Budd-Chiari syndrome?

Answer 31

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

Question 32

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

Answer 32

Acute - jaundice - tender hepatomegaly Chronic - ascites

Question 33

How do you diagnose Budd-Chiari?

Answer 33

U/S visualisation of hepatic veins - showing occlusion

Question 34

How do you treat budd Chiari?

Answer 34

TIPS | Recanculisation

Question 35

What is methotrexate usually used to treat?

Answer 35

Rheumatoid arthritis and psoriasis

Question 36

What must you be careful about when using methotrexate?

Answer 36

Dose dependent liver toxin - can cause progressive fibrosis

Question 37

What is cardiac cirrhosis?

Answer 37

Secondary to high right heart pressures

Question 38

What are the clinical symptoms of cardiac cirrhosis?

Answer 38

CCF | Too much ascitites and or liver impairment

Question 39

How do you treat cardiac cirrhosis?

Answer 39

Treat the cardiac condition