Cirrhosis Flashcards

1
Q

What are causes of cirrhosis?

A
Alcohol
NAFLD
Hep C and B 
PBC
Autoimmune hepatitis
Haemochromatosis
PSC
Wilson's disease
Alpha 1 anti-trpsin
Budd-Chiari
Methotrexate
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2
Q

How common if NAFLD?

A

The commenest disease in the worls - 30% of the general population
Associated with the metabolic syndrome

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3
Q

Histologociallly what is NASH?

A

A maladaption to oxidate stress causing stearohsis

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4
Q

What is the 2 hit paradigm?

A

1st hit - excess fat accumulation

2nd hit - intrahepatic oxidative stress, lipid peroxidation , TNF-alpha, cytokine cascade

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5
Q

What is the metabolic syndrome?

A
Type 2 diabetes
Obesity
HDL cholesterol lower than 50 mg/dL
Hypertension 
NASH
Triglycerides over 150mg/dL
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6
Q

How is simple stetosis diagnosed?

A

US
No liver outcomes
Increased CV risk
Treatment: weight loss and exercise

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7
Q

How is NASH diagnosed?

A

Diagnosis at present by liver biopsy
Risk of progression to cirrhosis
Treatment: Weight loss and exercise, other experimental treatments

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8
Q

What are the different types of autoimmune liver disease?

A
Primary Biliary Cholangitis
Auto-immune hepatitis
Primary Sclerosing Cholangitis
Alcohol related liver disease
Drug reactions
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9
Q

What is the presentation of primary biliary cholangitis?

A

Middle ages women, usually aasymptomatic but can present with fatigue, itch without rash and exanthelasma/xanthomas

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10
Q

How is PBC diagnosed?

A

Positive AMA
Cholestatic LFTs
Liver biopsy

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11
Q

How is PBC treated?

A

Ureso deoxycholic acid

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12
Q

What is type 2 auto-immune hepatitis?

A

Children and young adults
LKM-1
Exclusive
AMA

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13
Q

What is type 1 auto-immune hepatitis?

A
Adult
ANA
ASMA
SLA severity 
IgG
AMA 
pANCA
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14
Q

What are the extraheaptic manifestations of type 1 auto-immune hepatits?

A
Autoimmune thyroditis
Graves disease 
Chronic UC 
RA
Pernicious anaemia
Systemic sclerosis
ITP
SE
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15
Q

What is the clinical presentation of auto-immune hepatitis?

A
Hepatomegaly 
Jaundice
Stigmata of chronic liver disease 
Splenomegaly
Elevated AST and ALT
Elevated PT
Malaise, fatigue, lethargy, nausea, abdo pain, anoerxia
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16
Q

How is auto-immune hepatits diagnosed?

A

Elevated AST and ALT
Elevated IgG
Presence of autoimmune antibodies
Lvier biopsy

17
Q

What are the differential diagnosis for auto-immune hepatitis?

A
Wilsons disease 
Alpha 1 antitrypsin deficiency
Viral hepaitits
Drug induced liver disease
NASH
PBC, PSC, autoimmune cholangitis
18
Q

What does auto-immune hepatitis look like histologically?

A

Chronic hepatitis with marked piecemeal necoriss and lobular involvement
Numerous plasma cells
Interface hepatitis - hallmark finding

19
Q

How is autoimmunne hepatitis treated?

A

Corticosteroids

Axathioprine

20
Q

What is primary sclerosing cholangitis?

A

Autoimmune destrictuve disease of the large and medium sized bile ducts

21
Q

How is primary sclerosing cholangitis diagnosed?

A

Imagine of biliary tree

Recurrent cholangitis

22
Q

How is PSC trated?

A

Maintain bile flow, monitor for chlangiocarcinoma and colorectal cancer

23
Q

What is haemocrhomatosis?

A

Mono-genetic autosomal recessive disease of iron overload, C282Y or H63D mutations in HFE gene

24
Q

What are the complications of haemochromatosis?

A

Cirrhosis, cardiomyopathy, pancreatic failure, “the bronzed diabetic”

25
Q

How is haemochormatosis treated?

A

Venesection

26
Q

What is wilsons disease?

A

Mono-genetic autosomal recessive disesae

Loss of function or loss of protein mutations in caeruloplasmin

27
Q

What is caeruloplasmin?

A

Copper binding protein, loss of copper regulation with massive tissue deposition of copper, especially liver and basal ganglia

28
Q

What are the clinical signs of wilson’s disease?

A

Neurological - chorea-atheitoid movements
Hepatic - cirrhosis or sub-fulminant liver failure
Kaiser fleisher rings

29
Q

How is wilson’s disease treated?

A

Copper chelation drugs

30
Q

What is alpha 1 anti-trypsin deficiency?

A

Genetic, mutations in the A1At genes, multiple sites, causes variable phenotypes
Protein function lost excess tryiptic activity

31
Q

What are the linical signs of alpha 1 anti-trypsin deficiency?

A

Lung emphysema

Liver deposition of mutant protein, cell damage

32
Q

What is the treatment for alpa 1 anti-trypsin deficiency?

A

Supportive

33
Q

What is budd-chiari?

A

Thrombosis of the hepatic veins, congenital webs, thrombotic tendency, protein C or S deficiency

34
Q

What is the clinical picture of budd-chiari?

A

Acute - jaundice, tender hepatomegaly

Chronic - ascites

35
Q

How is budd-chiari dianogsed?

A

U/S visualisation of hepatic veins

36
Q

How is budd-chiari treated?

A

Tecanalixation or TIPS

37
Q

What is methotrexate?

A

Drug used to treat RA and psoriasis

Dose dependent of liver toxxin as it can cause progressive fibrosis

38
Q

What is cardiac cirrhosis?

A

Secondary to high right heart pressures (incompetent tricuspid valve, congenital, rheumatic fever, constrictive pericarditis)

39
Q

How is cardiac cirrhosis diagnosed and treated?

A

CCF, with too much ascites or liver damage

Treatment is to treat the cardiac condition