chronic liver disease Flashcards

1
Q

what is the definition of a chronic liver disease

A

one which can lead to cirrhosis

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2
Q

PBC

A

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

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3
Q

PBC epidemiology and classical presentation

A

women middle aged

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

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4
Q

PBC S/S

A

xanthomas

jaundice - appears later

itch without rash

fatigue

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5
Q

PBC Dx

A

2 of AMA, cholestastic LFT and liver biopsy

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6
Q

management PBC

A

Urseodeoxycholic acid

transplant if severe

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7
Q

autoimmune hepatitis

A

resulting inflammation the liver as being attacked by own immune cells

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8
Q

autoimmune hepatitis epidemiology

A

WOMEN >MEN

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9
Q

autoimmune hepatitis o/e

A

hepatomegaly, splenomegaly, jaundice

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10
Q

autoimmune hepatitis s/s

A

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

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11
Q

autoimmune hepatitis dx

A

elevated AST, ALT, IgG

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12
Q

autoimmune hepatitis treatment

A

corticosteroids - prednisolone

immune suppressant - azathioprine

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13
Q

autoimmune hepatitis histology

A

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

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14
Q

autoimmune hepatitis type 1

A

seen in 80%, usually occurs alongside other autoimmune disease

ASMA and ANA postiive

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15
Q

autoimmune hepatitis type2

A

children and young adults

more common in europe than in asia

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16
Q

which type of autoimmune hepatitis more commonly progresses to cirrhosis

A

2

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17
Q

LKM-1 positive in which type of autoimmune hepatitis

A

2

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18
Q

ASMA and ANA in type 2 and 3 autoimmune hepatitis

A

negative

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19
Q

anti-SLA in type 3

A

postive

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20
Q

PSC

A

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

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21
Q

PSC histology

A

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

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22
Q

PSC s/s

A

jaundice and fibrosis

asymptomatic

itch

malabsorption

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23
Q

PSC associated with

A

IBD - UC (40%)

24
Q

PSC dx

A

ERCP

25
Q

PSC more common in

A

MALE

26
Q

PSC treatment

A

maintain blood flow transplant

27
Q

PSC complications

A

colorectal cancer post transplant

cholangiocarcinoma

28
Q

describe the malabsorption PSC

A

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

29
Q

what can you develop post transplant

A

colorectal cancer

30
Q

Haemochromatosis inheritance and genes

A

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

31
Q

primary and secondary haemochromatosis

A

primary - AR condition

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

32
Q

Haemochromatosis complications pancreas and liver

A

PANCREAS

type 1 diabetes and malabsorption

LIVER

iron builds up which creates free radicals which cause cell damage and cirrhosis

33
Q

Haemochromatosis complications skin

A

bronzed skin slate grey

34
Q

Haemochromatosis complications heart

A

cardiomyopathy - arrhythmias

35
Q

Haemochromatosis complications gonads

A

amenorrhoea and testicular atrophy 0 erections

36
Q

Haemochromatosis investigations

A

blood test - inc transferring saturation and ferritin production liver MRI

biopsy

ECHO/ECG for cardiomyopathy

37
Q

Haemochromatosis treatment

A

predispose to carcinoma

Venesection (phlebotomy)and blood letting.

out come depends on genetics and cofactors (alcohol)

38
Q

wilsons disease inheritance

A

recessive

39
Q

wilsons disease

A

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

40
Q

wilsons disease s/s

A

hepatic seen first - cirrhosis, chronic hepatitis, liver failure

neurology - weird movement, depression, labile emotions

eyes - kaiser-flesher rings

41
Q

wilsons disease ix

A

urine - inc free copper

LFT

genetic

MRI

serum ceruloplasmin dec normally but can be falsely high or low

42
Q

wilsons disease tx

A

lifelong copper chelating drugs

43
Q

how do children in wilsons disease usually present

A

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

44
Q

how do adults with wilsons disease usually present

A

CNS signs - tremor, dysarthria, dysphagia

45
Q

copper chelating drug

A

pencillamine

46
Q

Budd Chiari classic presentation

A

young woman on oral contraceptive pill

47
Q

Budd Chiari

A

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

48
Q

Budd Chiari aetiology

A

pill

thrombophilia

leukaemia

obstructive cause e.g. tumour

49
Q

Budd Chiari s/s

A

acute - jaundice, tender hepatomegaly

chronic - ascites

50
Q

Budd Chiari ix

A

high protein ascitic fluid

US, CT, MRI to show occlusion

51
Q

Budd Chiari tx

A

recanalisation or TIPS

thrombolysis

treat ascites

52
Q

a1 - antitrypsin deficiency

A

excess tryptic activity that commonly affects the lungs and liver

53
Q

a1 - antitrypsin deficiency effect on lungs

A

emphysema

54
Q

a1 - antitrypsin deficiency effect on liver

A

hepatocellular cancer cirrhosis HCC

55
Q

a1 - antitrypsin deficiency management

A

supportive

56
Q

what is Methotrexate used in

A

rheumatoid arthritis and psoriasis

57
Q

what can Methotrexate cause

A

progressive liver fibrosis, to treat stop the drug