Chronic liver diseases Flashcards

1
Q

how do you define chronic liver disease?

A

= duration of greater than 6 months

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2
Q

what is the outcome of chronic liver disease?

A

= progression to cirrhosis

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3
Q

what is the pathology of chronic liver disease?

A

= recurrent inflammation and repair with fibrosis and regeneration

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4
Q

what are the causes of chronic liver disease?

A
  • alcohol
  • hep C
  • primary biliary cholangitis
  • auto-immune hepatitis
  • hep B
  • NAFLD
  • haemochromatosis
  • primary sclerosis cholaningitis
  • Wilsons disease
  • alpha 1 anti-trypsin
  • budd-chiari
  • methotrexate
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5
Q

what is the histopathology of non-alcoholic fatty liver disease?

A

= maladaption of oxidative stress

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6
Q

what is non-alcoholic fatty liver disease associated with?

A
  • diabetes
  • obesity
  • fatty liver disease
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7
Q

describe the 2 hit pathogenesis in non-alcoholic fatty liver disease?

A

First hit
= excess fat accumulation
= hepatocytes can generate TNFa

Second hit
= intra-hepatic oxidative stress
= lipid per-oxidation
= TNF-alpha, cytokinie cascade 
= lipopolysaccharide
- ischeamia re-perfusion injury
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8
Q

how do you diagnose and treat simple steatosis?

A

= diagnosed by ultrasound

Treatment

  • weigh loss
  • exercise
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9
Q

how is Non-Alcoholic Steatohepatitis diagnosed and treated?

A

= diagnosed by liver biopsy

treatment

  • weight loss
  • exercise
  • other experimental treatment
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10
Q

what are the 3 auto-immune liver diseases?

A

1) primary biliary cholangitis
2) auto-immune hepatitis
3) primary sclerosing cholangiis

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11
Q

what is the typical presentation for primary biliary cholangitis?

A
  • middle aged women
  • asympatomatic/incidental
  • fatigue
  • itch without rash
  • Xanthelasma
  • xanthomas
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12
Q

how do you diagnose primary biliary cholangitis?

A
  • positive AMA
  • cholestatic LFTs
  • liver biopsy
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13
Q

how do you treat primary biliary cholanigits?

A
  • urseo deoxycholic acid
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14
Q

what are the 2 types of auto-immune hepatitis?

A
type 1 
= adult
= ANA 
= ASMA 
= SLA severity 
Type 2 
= children & young adults
= LKM-1
= exclusive
= AMA
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15
Q

what is type 1 auto-immune hepatitis associated with?

A
  • extra-hepatic manifestations
    = auto-immune thyroiditis
    = graves disease
    = chronic UC
- less commonly with; 
= RA
= pernicious anaemia
= systemic sclerosis 
= ITP 
= SLE
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16
Q

how do you present with auto-immune hepatitis?

A
  • hepatomegaly
  • jaundicee
  • stigmata of chronic liver disease
  • splenomegaly
  • elevated AST and ALT
  • elevated PT
  • non-specific symptoms;
    = malaise
    = fatigue
    = lethargy
    = nausea
    = abdominal pain
    = anorexia
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17
Q

how do you diagnose auto-immune hepatitis?

A
  • Elevated AST and ALT
  • Elevated IgG
  • Rule out other causes:
    = Wilsons disease
    = Alpha 1 antitrypsin deficiency
    = Viral hepatitis (A, B, C)
    = Drug induced liver disease (alcohol, minocycline, nitrofurantoin, INH, PTU, methyldopa, etc)
    = NASH
    = PBC, PSC, autoimmune cholangitis
  • Presence of autoimmune antibodies
  • Liver biopsy
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18
Q

what is chronic hepatitis marked by histologically?

A

= piecemeal necrosis and lobular involvement

= numerous plasma cells

19
Q

describe the pathogenesis of interface hepatitis?

A

= Genetically predisposed individual with exposure to an environmental agent triggers the autoimmune pathogenic process
= Genetic predisposing factors:
- HLA-DR3: early onset, severe form
- HLA-DR4: caucasian, late onset, better response to steroids, higher incidence of extrahepatic manifestations
- IgG: part of the IgG molecule (mainly the heavy chain)
- T-Cell receptors

= environmental triggers

= drugs; 
Oxyphenisatin
Methyldopa
Nitrofurantoin
Diclofenac
Minocycline
statins
20
Q

how do you treat interface hepatitis?

A
Corticosteroids
Azathioprine
Children: azathioprine or 6MP
Combination Therapy
Prednisone + Azathioprine
Prednisone: start at 30mg daily and taper down to 15mg at week 4, then maintain on 10mg daily until therapy endpoint
Azathioprine 50-100mg daily
21
Q

what is primary sclerosing cholangitis?

A

Autoimmune destructive disease of large and medium sized bile ducts.

22
Q

what is primary sclerosis cholangitis associated with?

A

= colitis (mainly ulcerative colitis)

23
Q

how do you diagnose primary sclerosin cholangitis?

A

imaging of biliary tree

24
Q

how do you treat primary sclerosing cholangitis?

A

= bile flow
= monitor for cholaniocarcinoma
= colorectal cancer

25
Q

what is haemochromatosis?

A

= autosomal recessive disease of iron overload

  • C282Y
  • H63D
  • mutations in HFE ene
26
Q

what can haemeochromatosis cause?

A
  • cirrhosis
  • cardiomyopathy
  • pancreatic failure
  • the bronzed diabetic
27
Q

how do you treat haemochromatosis?

A

= venesection

28
Q

what is Wilsons disease?

A

= autosomal recessive disorder where there is a loss of function or loss of protein mutation in caeruloplasmin
(copper binding protein, loss of copper regulation massive tissue deposition of copper)

29
Q

how do you presenting with wilsonsn disease?

A

Neurological
= chorea-atheitoid movementts

Hepatic
= cirrhosis
= sub-fulminant liver failure

Kaiser Flisher rings

30
Q

how do you treat Wilsons disease?

A

= copper chelation drugs

31
Q

what is alpha 1 anti-trypsin deficiency?

A

Genetic, Mutations in the A1AT genes, multiple sites, causes variable phenotype
Protein Function lost excess tryptic activity

32
Q

how do you present with alpha 1 anti-trypsin deficiency?

A
  • lung emphsema

- liver deposition of mutant protein, cell damage

33
Q

how do you manage alpha 1 anti-trypsin deficiency?

A

= supportive management

34
Q

what is Budd-chiari syndrome?

A

= thrombosis of hepatic vein
= so blood cant drain out of liver
- congenital webs

35
Q

what deficiency causes budd-chiari syndrome?

A

= Protein C or S deficiency

36
Q

how do you present with budd-chiarir syndrome?

A
  • jaundice
  • tender hepatomegaly
  • ascites
37
Q

how do you diagnose budd-chiari?

A

= U/S visualisation of hepatic veins

38
Q

how do you treat budd-chiari syndrome?

A

= recanalization or TIPS

39
Q

what is methotrexate used to treat?

A

= rheumatoid arthritis and psoriasis

40
Q

what can methotrexate cause?

A
  • fibrosis
41
Q

what is cardiac cirrhosis secondary to?

A

= high right heart pressure

42
Q

what could cause cardiac cirrhosis?

A

Incompetent tricuspid valve
Congenital
Rheumatic fever
Constrictive pericarditis

43
Q

how do you present wit cardiac cirrhosis?

A
  • CCF

- too much ascites or liver impairment

44
Q

how do you treat cardiac cirrhosis?

A

= treat cardiac condition