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Y4 Gastrointestinal > Liver disease > Flashcards

Liver disease Flashcards

1
Q

Define what chronic liver disease is

A
  • It is a disease process of the liver lasting over 6 months that involves a process of progressive destruction and regeneration of the liver parenchyma leading to fibrosis and cirrhosis.
  • It consists of a wide range of liver pathologies
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2
Q

What does chronic liver disease eventually result in ?

A

Compensated cirrhosis and then decompensated cirrhosis/chornic liver failure or acute on chronic liver failure

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3
Q

List the different causes of chronic liver disease

A
  • Chronic Alcohol abuse
  • NAFLD
  • Hepatitis C or B
  • Autoimmune disorders - Primary Biliary Cholangitis (PBC), Primary Sclerosing Cholangitis (PSC), Autoimmune Hepatitis
  • Genetic disorders - Haemochromatosis, Wilsons Disease, alpha 1anti-trypsin
  • Budd-Chiari
  • Drugs - Methotrexate, amiodarone, methyldopa
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4
Q

What are the clinical features of liver cirrhosis (compensated and decompensated)?

A

May be none (just increased LFT’s) but as cirrhosis progresses signs eventually show

Chronic liver disease signs:

  • Leuconychia (white nails due to hypoalbunaemia)
  • Clubbing
  • Palmar erythema
  • Duputyrens contracture
  • Jaundice
  • Hepatomegaly
  • Easy burising
  • Weight loss & loss of appetite
  • Fatigue & weakness
  • Spider naevi
  • Xanthelasma
  • Testicular atrophy & hair loss
  • Complications may develop

Then will eventually become decompensated (bascially liver failure):

  • Liver failure Acute –on-chronic; Infection, SIRS
  • Jaundice, Ascites, Encephalopathy, Bruising
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5
Q

Good to look over

A
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6
Q

What clinical sign of cirrhosis is shown here ?

A

Spider naevi

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7
Q

What complications may develop in someone with liver cirrhosis ?

A
  • Hepatic failure - encephalopathy & ascites etc
  • Portal hypertension - ascities, splenomegaly, Portosystemic shunt (oesophageal varices, caput medusae & haemorrhoids)
  • Hepatocellulcar carcinoma
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8
Q

How is liver cirrhosis diagnosed ?

A

Offer transient elastography to diagnose cirrhosis for:

  • People with hepatitis C virus infection
  • Men who drink >50 units of alcohol & women who drink > 35
  • People diagnosed with alcohol-related liver disease.

Offer either transient elastography or acoustic radiation force impulse imaging (whichever is available) to diagnose cirrhosis for people with NAFLD and advanced liver fibrosis (as diagnosed by a score of 10.51 or above using the enhanced liver fibrosis [ELF] test).

2nd line = Consider liver biopsy to diagnose cirrhosis in people for whom transient elastography is not suitable.

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9
Q

What is the general treatment of liver cirrhosis ?

A
  • Avoid alcohol, NSAID’s, sedatives & opiates
  • Colestyramine for pruritus
  • Specific treatment then depending on the cause and complications
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10
Q

What is the only cure for liver cirrhosis ?

A

Liver transplantation

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11
Q

What screening tests are recommended to be done in patients with diagnosed liver cirrhosis ?

A
  1. An upper endoscopy to check for varices in patient’s with a new diagnosis of cirrhosis
  2. Liver US every 6 months (+/- alpha-feto protein) to check for hepatocellular cancer
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12
Q

What is NAFLD?

A

It is a spectrum of disease ranging from:

  • Steatosis - fat in the liver
  • Steatohepatitis - fat with inflammation, non-alcoholic steatohepatitis (NASH), see below
  • Progressive disease may cause fibrosis and liver cirrhosis
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13
Q

What is the primary cause of NAFLD?

A

Obesity

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14
Q

What are the risk factors for development of NAFLD?

A
  • obesity
  • type 2 diabetes mellitus
  • hyperlipidaemia
  • jejunoileal bypass
  • sudden weight loss/starvation
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15
Q

What are the features of NAFLD?

A
  • Usually asymptomatic
  • Hepatomegaly
  • ALT is typically greater than AST
  • Increased echogenicity on ultrasound
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16
Q

Should people at risk of NAFLD be screened for it ?

A

No

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17
Q

If NAFLD is found incidentally (usually on liver US) what should be done ?

A

Enhanced liver fibrosis (ELF) blood test to check for advanced fibrosis

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18
Q

If the ELF blood test shows someone is likely to have advanced liver fibrosis what should be done ?

A
  • 1st line = transient elastography or ARFII
  • 2nd line = liver biopsy
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19
Q

What is the treatment of NAFLD ?

A

Lifestyle changes (particularly weight loss) and monitoring

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20
Q

What is primary biliary cholangitis ?

A

It is a chronic autoimmune liver disorder where interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis and portal hypertension.

Note - used to be called ‘primary biliary cirrhosis’

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21
Q

Who is typically affected by primary biliary cholangitis ?

A

Middle-aged women

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22
Q

What conditions is primary biliary cholangitis associated with ?

A

Autoimmune conditions

  • Sjogren’s syndrome (seen in up to 80% of patients)
  • rheumatoid arthritis
  • systemic sclerosis
  • thyroid disease
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23
Q

How is primary biliary cholangitis diagnosed ?

A
  • 1st line = LFTs will demonstrate a cholestasis (obstructive picture) - Increased ALP & GGT + midly increased ALT & AST
  • 2nd line = A liver screen is done (includes US abdo, autoantibodies tested for & immunoglobulins (IgM will be increased). Sometimes liver biopsy done but rarley needed)

If the liver screen demonstrates AMA or smooth muscle antibodies then PBC diagnosed.

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24
Q

What autoantibody is the hallmark of primary biliary cholangitis ?

A

Antimitochondrial antibodies (AMA)

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25
Q

What are the clinical features of primary biliary cholangitis ?

A

Early:

  • Often asymptomatic (e.g. raised ALP on routine LFTs)
  • Fatigue and pruritus (itch) may then preceed jaundice by many years

Mid:

  • Cholestatic jaundice - this will have itch
  • Skin hyperpigmentation, especially over pressure points
  • xanthelasmas, xanthomata
  • clubbing, hepatosplenomegaly

Late:

  • may progress to liver failure
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26
Q

What are the potential complications of primary biliary cholangitis ?

A
  • Malabsorption of fat soluble vitamins (ADEK): osteomalacia, coagulopathy
  • Sicca syndrome (Sjogren syndrome)
  • Portal hypertension: ascites, variceal haemorrhage
  • Hepatocellular cancer
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27
Q

What should be checked due to the increased risk of hepatocellular cancer in patients with primary biliary cholangitis ?

A

AFP twice per year

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28
Q

What is the treatment of PBC ?

A
  • Pruritus: cholestyramine
  • Fat-soluble vitamin supplementation
  • Ursodeoxycholic acid
  • Liver transplantation e.g. if bilirubin > 100
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29
Q

What is primary sclerosing cholangitis ?

A

A biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.

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30
Q

What conditions is primary sclerosing cholangitis associated with ?

A
  • UC - 4% of patients with UC have PSC, 80% of patients with PSC have UC
  • Crohn’s (much less common association than UC)
  • HIV
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31
Q

What are the clinical features suggestive of primary sclerosing cholangitis ?

A
  • Cholestasis = jaundice and pruritus
  • RUQ pain
  • Fatigue
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32
Q

What complications can develop due to primary sclerosing cholangitis ?

A
  • Ascending cholangitis
  • Cirrhosis and end-stage hepatic failure
  • Cholangiocarcinoma
  • Colorectal cancer
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33
Q

How is primary sclerosing cholangitis diagnosed ?

A

Via MRCP (ERCP less preferred due to being invasive)

  • LFTs - ALP & GGT elevated, AST & ALT mild-moderately elevated
  • AMA negative but may be positive for ANCA, ANA & SMA
34
Q

What is the treatment of primary sclerosing cholangitis ?

A
  • Colestyramine for puritis
  • Liver transplantation is they develop end-stage disease
  • Monitor for cholangiocarcinoma and colorectal cancer
35
Q

What is autoimmune hepatitis ?

A

It is an inflammatory liver disease of unknown cause characterised by suppressor T-cell defects with autoantibodies directed against hepatocyte surface antigens

36
Q

What are the 3 types of autoimmune hepatitis and what are they based on ?

A

Classification is based on the autoantibodies present:

  • Type I = ANA &/or SMA present
  • Type II = Anti-liver/kidney microsomal type 1 antibodies (LKM1) present. ANA & SMA -ve
  • Type III = Soluble liver-kidney antigen (SLA) present. ANA & SMA -ve
37
Q

Who is affected by each of the 3 types of autoimmune hepatitis ?

A
  • Type I affects both adults & children
  • Type II affects children only
  • Type III affects middle aged adults
38
Q

Who is most commonly affected by autoimmune hepatitis ?

A

Young females (10-30)

39
Q

What are the clincal features of autoimmune hepatitis ?

A

40% present with Acute hepatitis: fever, jaundice etc and signs of autoimmune disease e.g. malaise, uticarial rash, polyarthritis, pleurisy, pulmonary infiltration or glomerulonephritis

The remainder may present with signs of chronic liver disease:

  • Hepatomegaly
  • Jaundice
  • Stigmata of chronic liver disease
  • Splenomegaly
  • Elevated AST and ALT, Elevated PT
  • Non-specific symptoms: malaise, fatigue, lethargy, nausea, abdominal pain, anorexia

Amenorrhoea (common)

ANA/SMA/LKM1 antibodies, raised IgG levels

40
Q

How is autoimmune hepatitis diagnosed ?

A

No lab test is pathognomic so depends on ruling out other causes:

  • Elevated AST and ALT
  • Elevated IgG
  • Presence of autoimmune antibodies
  • Liver biopsy - interface hepatitis and marked piecemeal necrosis and lobular involvement

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
41
Q

What is associated with autoimmune hepatitis ?

A
  • Autoimmune disorders e.g. thyroiditis, graves disease, chronic UC, pernicious anaemia, autoimmune haemolysis, glomerulonephritis etc
  • Hypergammaglobulinaemia
  • and HLA B8, DR3.
42
Q

What is the treatment of autoimmune hepatitis ?

A
  • 1st line = Prednisone + Azathioprine
  • 2nd line = liver transplantation if decompensated cirrhosis or failure to respond to medical therapy
43
Q

Note Hepatitis B&C is covered in the hepatitis deck

A
44
Q

What is hereditary haemochromatosis (HH)?

A

An autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation and deposition in the heart, liver, pancreas, pituitary, adrenals, joints & skin

45
Q

What mutation causes HH?

A

C282Y or H63D mutations in both copies of the HFE gene on chromosome 6

46
Q

What are the clinical features suggestive of HH?

A

Early on - symptoms may be nil or faitgue, arthralgia (esp the hands) & erectile dysfunction

Later on:

  • Bronze skin pigmentation
  • Signs of chronic liver disease
  • Cardiac failure - 2° to dilated cardiomyopathy
  • Diabetes mellitus - 2° iron deposition in pancreas
  • Hypogonadism (2nd to cirrhosis and pituitary dysfunction - hypogonadotrophic hypogonadism)
  • Arthritis (especially of the hands)
47
Q

How is HH diagnosed ?

A
  • 1st = check serum ferritin (>300 in men and >200 in women) and % tranferrin (>50%) & low TIBC - this is suggestive of iron loading
  • 2nd = Diagnosis then made by testing for HFE mutation

Liver biopsy using Perl’s stain only done only if uncertain about iron loading or to assess tissue damage

48
Q

What do joint X-rays in someone with HH typically show ?

A

Chondrocalcinosis

49
Q

What is the treatment of haemachromatosis ?

A
  • 1st = Initially weekly venesections to exhaust iron stores continue this until - serum ferritin <20 µg/L
  • 2nd = Then carry out maintance venesections 3-4 times per year - to keep serum ferritin below 50 µg/l
50
Q

When we discover someone has hereditary haemachromatosis, what is offered to 1st degree family members ?

A

Testing for HFE mutation and iron status checked with serum Ferritin and % transferrin saturation

51
Q

What is wilsons disease ?

A

An autosomal recessive disorder characterised by excessive copper deposition in the tissues (liver & CNS) due to increased copper absorption from the small intestine and decreased hepatic copper excretion.

52
Q

What causes wilsons disease ?

A

Defect in the ATP7B gene located on chromosome 13.

53
Q

When does the presentation of wilsons disease usually arise ?

A
  • Usually between 10 - 25 years.
  • Children usually present with liver disease whereas the first sign of disease in young adults is often neurological disease
54
Q

What are the clinical features suggestive of wilsons disease?

A
  • Liver disease - hepatitis, cirrhosis, liver failure
  • CNS signs: tremor, dysarthria, dysphagia, dyskinesias, dystonias, purposless stereotyped movements e.g. hand clapping, dementia, parkinsonism, micrographia, ataxia/clumsiness
  • Kayser-Fleischer rings
  • renal tubular acidosis (esp. Fanconi syndrome)
  • haemolysis
  • blue lunulae (blue nails)
55
Q

How is wilsons disease diagnosed ?

A

No one test is diagnostic - Copper studies mainly:

  • Reduced serum caeruloplasmin
  • Reduced serum copper (counter-intuitive, but 95% of plasma copper is carried by ceruloplasmin)
  • Increased 24hr urinary copper excretion

May also do:

LFTs, liver biopsy, MRI, slit lamp for KF rings and genetic testing

56
Q

If someone is diagnosed with wilsons disease what should be done ?

A

Genetic testing of 1st degree relatives via analysis of the ATP7B gene

57
Q

What is the treatment of wilsons disease?

A
  • 1st line = penicillamine (chelates copper)
  • 2nd line = trientine hydrochloride (alternative chelating agent)
58
Q

What is Alpha-1 antitrypsin deficiency?

A

A common inherited condition caused by a lack of A1AT normally produced by the liver. It commonly affect the lungs (emphysema) & the liver (cirrhosis & hepatocellular carcinoma)

59
Q

What is the role of A1AT ?

A

To protect cells from enzymes such as neutrophil elastase.

60
Q

What genetic mutation causes A1AT deficiency?

A

Defect located on chromosome 14 and the degree of A1AT def is classified as:

  • normal = PiMM
  • homozygous PiSS (50% normal A1AT levels)
  • homozygous PiZZ (10% normal A1AT levels)
61
Q

What are the clinical features of A1AT deficiency ?

A
62
Q

What patients with A1AT def usually manifest disease?

A

Usually those with the PiZZ genotype

63
Q

What are the clinical features suggestive of A1AT defiency ?

A
  • Lungs: dysponea from emphysema (COPD)
  • Liver: cirrhosis and hepatocellular carcinoma in adults, cholestasis jaundice in children
64
Q

What is the classical presentation of someone with A1AT def ?

A

Emphysema (i.e. chronic obstructive pulmonary disease) in patients who are young and non-smokers.

65
Q

How is A1AT def diagnosed ?

A
  • 1st line = serum A1AT concentrations (if low diagnosed)
  • 2nd line if low conc = Genetic testing to see the variation of the faulty A1AT gene a person has

spirometry: obstructive picture to see how severly someone is affected

66
Q

What is the treatment of A1AT def?

A

1st line = no smoking + supportive: bronchodilators, physiotherapy

67
Q

What is budd-chiari syndrome ?

A

This is hepatic vein obstruction caused by thrombosis or a tumour. It results in congestive ischaemia & hepatocyte damage

68
Q

What causes budd-chiairi syndrome ?

A

Hypercoagulative states:

  • polycythaemia rubra vera
  • thrombophilia: activated protein C resistance, antithrombin III deficiency, protein C & S deficiencies
  • pregnancy
  • oral contraceptive pill

Tumours - liver, renal or adrenal

69
Q

What are the clinical features suggestive of budd-chiairi syndrome ?

A
  • Abdominal pain: sudden onset, severe
  • Ascites
  • Tender hepatomegaly
  • Increased ALT
70
Q

How is budd-chiairi syndrome diagnosed ?

A

US with Doppler flow studies

71
Q

What is the treatment of budd-chiairi syndrome ?

A

Recanalization via angioplasty or TIPS

72
Q

What are the clinical features suggestive of methotrexate (or the other drugs) causing liver fibrosis and potentially chronic liver disease?

A
  • Clinical- no signs, monitor fibrosis
  • Tx = stop the drug
73
Q

What is cardiac cirrhosis ?

A

Liver cirrhosis secondary to cardiac dysfunction, common causes include:

  • Incompetent tricuspid valve
  • Congenital
  • Rheumatic fever
  • Constrictive pericarditis
74
Q

What are the clinical features suggestive of cardiac cirrhosis ?

A

CCF, with too much ascites and or liver impairment

75
Q

What is the treatment of cardiac cirrhosis ?

A

Treat the cardiac condition

76
Q

What is alcoholic liver disease?

A

It is the term used to describe the manifestations of alcohol overconsumption, leading to inflammation and scarring of the liver tissue

The spectrum of alcoholic liver disease includes 3 subtypes:

  1. Alcoholic fatty liver disease
  2. Alcoholic hepatitis
  3. Cirrhosis.
77
Q

As alcoholic liver disease progresses what do people develop symptoms/signs of ?

A

Chronic liver disease

78
Q

What are the LFT results suggestive of alcoholic liver disease ?

A

Ratio of AST:ALT is normally >2, a ratio of >3 is strongly suggestive of acute alcoholic hepatitis

  • AST: typically 100-200IU/L
  • ALT: May be normal or only mildly raised, even in severe cases.

GGT raised in association with chronic heavy alcohol comsumption

In alcoholic fatty liver disease (AFLD), normally there is a solitary mild increase in AST and ALT.

79
Q

What is the treatment of alcohol liver disease ?

A
  • Alcohol absitence & weight loss & nutrition (high protein diet)
  • Hep A & B vaccinations
  • Prednisolone given for acute alcoholic hepatitis
  • End-stage ALD (cirrhosis with decompensation), liver transplantation can be considered usually must be alcohol free for >6 months
80
Q

What can drinking during pregnancy cause?

A
  • Foetal alcohol syndrome
81
Q

What are the clinical features of foetal alcohol syndrome?

A
  • Baby may show symptoms of alcohol withdrawal at birth e.g. irritable, hypotonic, tremors

Features:

  • short ­palpebral fissure
  • thin vermillion border/hypoplastic upper lip
  • smooth/absent filtrum
  • learning difficulties
  • microcephaly
  • growth retardation
  • epicanthic folds
  • cardiac malformations

Y4 Gastrointestinal (28 decks)

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