wk 6 6 Aetiologies and Pathophysiology of chronic liver disease

Question 1

t/f chronic liver disease is of a duration greater than 3 months

Answer 1

false 6 months

Question 2

t/f chronic liver disease can present acutely q

Answer 2

true
duration (6mnths) may be sub clinical
signs/symptoms dependent on underlying disease/features of cirrhosis

Question 3

describe the pathology of chronic liver disease

Answer 3

recurrent inflammation and repair with fibrosis and regeneration

Question 4

fibrosis of the liver is due to the activation of hepatic stellate cells, how does this occur

Answer 4

quiescent HSC is activated through hepatocyte Kupffer inflammatory cells

Question 5

TIMP is what turns the activated HSC into an apoptic HSC, what does it do

Answer 5

TIMP - Tissue Inhibitor of Metalloprotinease, determines the degradation of cells through MMP (matrix metalloprotineases)

Question 6

other than alcohol, autoimmune disease and hepatitis, what else can cause Chronic liver disease

Answer 6

NAFLD (non-alcoholic fatty liver disease)
Primary Biliary/sclerosing Cholangitis
Haemochromatosis (iron overload)
WIlsons Disease (copper overload)
Alpha 1 anti-trypsin (pancreatic enzyme)
Budd-Chairi (hepatic portal occlusion)
Methotrexate

Question 7

2 examples of chronic diseases that affect the liver (but arent chronic liver disease)

Answer 7

amyloid 
rotor syndrome (hyperbilirubinaemia)

Question 8

what is the commonest disease in the world

Answer 8

NAFLD (simple steatosis)

Question 9

NAFLD vs NASH

Answer 9

NASH is a type of NALFD

there is Non-alcoholic steatohepatitis, or simple steatosis

Question 10

NALFD initiates due to excess fat accumulation, what does this cause 3

Answer 10

inrtrahepatic oxidative stress
lipid peroxidation
TNF-alpha/cytokine cascade

Question 11

t/f progression to NASH can occur with/without increased expression of ARE-genes

Answer 11

true
with - large inc in ROS leads to greater expresssion
without - multiple factors (Fatty acids) instead cause

both lead to activation of NF–kB

Question 12

how is a simple steatosis diagnosed

Answer 12

ultrasound

Question 13

simple steasosis increases the risk of

Answer 13

cardiovascular disease

Question 14

treatment for simple steatosis

Answer 14

weight loss

exercise

Question 15

in NASH, there is a risk of progression to

Answer 15

cirrhosis

Question 16

difference between primary biliary and primary sclerosing choangitis

Answer 16

primary biliary - interlobular bile ducts affected by granulomatous immune cells, leading to cholangitits

primary sclerosing - progressive inflammation of bile ducts leading to strictures

Question 17

how does PBC present?

Answer 17

middle aged woman 
usually asymptomatic but may feel 
- fatigue
-itch and no rash 
- xanthesalma and xanthomas

Question 18

what antibodies are produced in PBC which allow it to be diagnosed

Answer 18

Antimitochondrial Antibodies (AMAs)

Question 19

other thn AMAs, give 2 ways PBC may be diagnosed

Answer 19

Cholestatic LFTs

Liver Biopsy

Question 20

treatment of PBC

Answer 20

urseo deoxycholic acid

Question 21

t/f auto-immune hepatitis affects woman more than men

Answer 21

true

3:1`

Question 22

auto-immune hepatitis can be subtyped into Type 1 and 2, which one occurs in children/young adults? which is diagnosed through AMA alone? which will see IgG present?

Answer 22

type 2
type 2
type 1

Question 23

clinical presentation of type 1

Answer 23

patient: <40 yrs
hepatosplenomegaly
jaundice
stigmata of chronic liver disease
Elevated AST, ALT, PT (aspartate/alanine aminotransferase) (prothrombin time)
non specific - malaise,nausea,ab pain, anorexia

Question 24

how is type 1 diagnosed

Answer 24

elevated AST,ALT,IgG
rule out wilsons, antitrypsin, vira hep, drug induced liver disease, NASH, PBC/PSC
presence of autoimmune antibodies
liver biopsy

Question 25

what is the hallmark finding of type 1 autoimmune hepatitis histologically

Answer 25

interface hepatitis

also have chronic hepatitis with marked necrosis and lobular involvement with numerous plasma cells

Question 26

which drugs can trigger autoimmune hepatitis

Answer 26

methyldopa 
diclofenac
statins 
minocycline
oxyphenistatin 
nitrofurantoin

Question 27

treatment of autoimmune hepatitis

Answer 27

prednisone (coritcosteroid) + azathioprine (immunosuppressant)

Question 28

what worsens the prognosis in AIH

Answer 28

if present with ascites or hepatic encephalopathy (brain damage due to irremoval of toxins in liver)

Question 29

recurrent cholangitis is most likely

Answer 29

primary sclerosing Cholangitis

Question 30

2 cancers which need to be monitored for in PSC

Answer 30

cholangiocarcinoma

colo-rectal carcinoma

Question 31

how is PSC diagnosed

Answer 31

imaging of biliary tree

Question 32

which inflammatory disease is PSC most typically seen inb

Answer 32

Ulcerative Colitis

Question 33

haemochromatosis is …..

and is treated using

Answer 33

iron overload

maintenance venesection for life, diets reducing iron

Question 34

wilsons disease is due to the poor regulation of deposition of which metal? how is it treated?

Answer 34

copper

copper chelation drugs

Question 35

alpha 1 anti-trypsin defiency causes what

Answer 35

deregulation of neutrophil elastase (leading to emphysema)

Question 36

Budd-chiari is the

Answer 36

thrombosis of hepatic veins

Question 37

3 defiencies which lead to budd-chairi

Answer 37

thrombotic defiency
protein C
protein S

Question 38

acute budd-chairi presents as jaundice and tender hepatomegaly, when woulld it be considered chronci

Answer 38

ascite formation

Question 39

how is budd-chairi diagnosed

Answer 39

Ultrasound of hepatic veins

Question 40

treatment for budd-chairi

Answer 40

recanalisation or TIPS (transjugular Intrahepatic Portosystemic Shunt)

Question 41

methotrexate is used to treat Rheumatoid arthritis and proriasis (skin condition) what can it cause

Answer 41

cirrhosis

treatment - stop drug

Question 42

cardiac cirrhosis is secondaruy to high right heart pressures (incompetent valves, congenital, rheumatic, pericarditis) how does it present and how is it treared

Answer 42

heart failure with ascites/liver impairment

treat underlying cardiac condition