chronic liver disease

Question 1

what is the definition of a chronic liver disease

Answer 1

one which can lead to cirrhosis

Question 2

PBC

Answer 2

disease of the liver where interlobular bile ducts are damaged by chronic autoimmune granulomatous inflammation causing cholestasis which may lead to fibrosis, cirrhosis and portal hypertension

Question 3

PBC epidemiology and classical presentation

Answer 3

women middle aged

classical patent is bus pass collecting women who is tired and itchy

Question 4

PBC S/S

Answer 4

xanthomas

jaundice - appears later

itch without rash

fatigue

Question 5

PBC Dx

Answer 5

2 of AMA, cholestastic LFT and liver biopsy

Question 6

management PBC

Answer 6

Urseodeoxycholic acid

transplant if severe

Question 7

autoimmune hepatitis

Answer 7

resulting inflammation the liver as being attacked by own immune cells

Question 8

autoimmune hepatitis epidemiology

Answer 8

WOMEN >MEN

Question 9

autoimmune hepatitis o/e

Answer 9

hepatomegaly, splenomegaly, jaundice

Question 10

autoimmune hepatitis s/s

Answer 10

asymptomatic Malaise, fatigue, lethargy, nausea, abdo pain, anorexia. Gradual jaundice.

Question 11

autoimmune hepatitis dx

Answer 11

elevated AST, ALT, IgG

Question 12

autoimmune hepatitis treatment

Answer 12

corticosteroids - prednisolone

immune suppressant - azathioprine

Question 13

autoimmune hepatitis histology

Answer 13

Interface Hepatitis w/marked piecemeal necrosis and lobular involvement, plasma cells

Question 14

autoimmune hepatitis type 1

Answer 14

seen in 80%, usually occurs alongside other autoimmune disease

ASMA and ANA postiive

Question 15

autoimmune hepatitis type2

Answer 15

children and young adults

more common in europe than in asia

Question 16

which type of autoimmune hepatitis more commonly progresses to cirrhosis

Answer 16

2

Question 17

LKM-1 positive in which type of autoimmune hepatitis

Answer 17

2

Question 18

ASMA and ANA in type 2 and 3 autoimmune hepatitis

Answer 18

negative

Question 19

anti-SLA in type 3

Answer 19

postive

Question 20

PSC

Answer 20

disease of the bile ducts causing progressive cholestasis with bile duct inflammation and strictures.

Question 21

PSC histology

Answer 21

beaded appearance - onion skinning fibrosis as fibrosis of cells around bile duct causes tightening in some areas and dilatation in others.

Question 22

PSC s/s

Answer 22

jaundice and fibrosis

asymptomatic

itch

malabsorption

Question 23

PSC associated with

Answer 23

IBD - UC (40%)

Question 24

PSC dx

Answer 24

ERCP

Question 25

PSC more common in

Answer 25

MALE

Question 26

PSC treatment

Answer 26

maintain blood flow transplant

Question 27

PSC complications

Answer 27

colorectal cancer post transplant cholangiocarcinoma

Question 28

describe the malabsorption PSC

Answer 28

due to dec amount of bile reaching the small intestine resulting in steatorrhoea and reduced amounts of at soluble vitamins (ADEK)

Question 29

what can you develop post transplant

Answer 29

colorectal cancer

Question 30

Haemochromatosis inheritance and genes

Answer 30

autosomal recessive due to C282Y or H63D mutations in HFE gene - the HFE gene reduces the expression of DMT1 at the brush border of enterocytes

Question 31

primary and secondary haemochromatosis

Answer 31

primary - AR condition secondary - due to iron overload from blood transfusion, iron therapy or diet

Question 32

Haemochromatosis complications pancreas and liver

Answer 32

PANCREAS type 1 diabetes and malabsorption LIVER iron builds up which creates free radicals which cause cell damage and cirrhosis

Question 33

Haemochromatosis complications skin

Answer 33

bronzed skin slate grey

Question 34

Haemochromatosis complications heart

Answer 34

cardiomyopathy - arrhythmias

Question 35

Haemochromatosis complications gonads

Answer 35

amenorrhoea and testicular atrophy 0 erections

Question 36

Haemochromatosis investigations

Answer 36

blood test - inc transferring saturation and ferritin production liver MRI biopsy ECHO/ECG for cardiomyopathy

Question 37

Haemochromatosis treatment

Answer 37

predispose to carcinoma Venesection (phlebotomy)and blood letting. out come depends on genetics and cofactors (alcohol)

Question 38

wilsons disease inheritance

Answer 38

recessive

Question 39

wilsons disease

Answer 39

loss of ceruloplasmin results in excess copper deposited in tissues copper accumulates first in the liver and then other organs e.g. brain

Question 40

wilsons disease s/s

Answer 40

hepatic seen first - cirrhosis, chronic hepatitis, liver failure neurology - weird movement, depression, labile emotions eyes - kaiser-flesher rings

Question 41

wilsons disease ix

Answer 41

urine - inc free copper LFT genetic MRI serum ceruloplasmin dec normally but can be falsely high or low

Question 42

wilsons disease tx

Answer 42

lifelong copper chelating drugs

Question 43

how do children in wilsons disease usually present

Answer 43

present with liver disease 0 cirrhosis chronic hepatitis or sub fulminant hepatic failure

Question 44

how do adults with wilsons disease usually present

Answer 44

CNS signs - tremor, dysarthria, dysphagia

Question 45

copper chelating drug

Answer 45

pencillamine

Question 46

Budd Chiari classic presentation

Answer 46

young woman on oral contraceptive pill

Question 47

Budd Chiari

Answer 47

Hepatic vein obstruction by thrombosis or tumour causes congestive ischaemia and hepatocyte damage.

Question 48

Budd Chiari aetiology

Answer 48

pill thrombophilia leukaemia obstructive cause e.g. tumour

Question 49

Budd Chiari s/s

Answer 49

acute - jaundice, tender hepatomegaly chronic - ascites

Question 50

Budd Chiari ix

Answer 50

high protein ascitic fluid US, CT, MRI to show occlusion

Question 51

Budd Chiari tx

Answer 51

recanalisation or TIPS thrombolysis treat ascites

Question 52

a1 - antitrypsin deficiency

Answer 52

excess tryptic activity that commonly affects the lungs and liver

Question 53

a1 - antitrypsin deficiency effect on lungs

Answer 53

emphysema

Question 54

a1 - antitrypsin deficiency effect on liver

Answer 54

hepatocellular cancer cirrhosis HCC

Question 55

a1 - antitrypsin deficiency management

Answer 55

supportive

Question 56

what is Methotrexate used in

Answer 56

rheumatoid arthritis and psoriasis

Question 57

what can Methotrexate cause

Answer 57

progressive liver fibrosis, to treat stop the drug