Cirrhosis Flashcards

1
Q

Definition

A

Implies irreversible liver damage resulting from the necrosis of liver cells followed by fibrosis and nodule formation.

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2
Q

Three key characteristics

A
  1. chronic hepatic necrosis
  2. chronic inflammatory process with fibrosis
  3. nodules
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3
Q

Aetiology

A

Vitamin

  • vascular
  • infective - hep C and B
  • Trauma
  • Autoimmune - ALD - PBC, Autoimmune hepatitis
  • Metabolic (drugs, toxins, alcohol) - alcoholic steatohepatits, and NASH
  • Iatrogenic
  • Neoplastic
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4
Q

Metabolic- Alcoholic steatohepatitis

A
  • 10-20% alcoholics affected
  • characterised by steatosis(retention of lipids), inflammation and fibrosis
  • Clinical - AST, ALT, fever, hepatomegaly and jaundice
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5
Q

Metabolic - Non-alcoholic steatohepatitis (NASH)

A
  • metabolic syndrome (diabetes, obesity)
  • characterised by steatosis, inflammation and fibrosis
  • cirrhosis takes longer to develop than alcoholic steatohepattis
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6
Q

Infective -Hep C virus

A
  • chronic >6 months
  • transmission: blood contact
  • Asymptomatic (silent)
  • diagnosis - Hep C ABs or Viral RNA - cannot detect antigen
  • treat with interferons
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7
Q

Infective hepatits B virus

A
  • Chronic > 6 months
  • transmission - blood, seme, vagina
  • vaccine is available to prevent
  • diagnose with HbsAg
  • treat with interferons
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8
Q

Primary Biliary cirrhosis definition

A

Autoimmune disease of the liver causing fibrosis and scarring of the biliary tract - Intrahepatic ducts

cholestasis - bile blockage

Mixed portal inflammation with granulomas (florid duct lesion

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9
Q

Primary bilary cirrhosis Clinical features

A
  • middle aged women
  • mainly asymptomatic
  • Fatigue
  • itching
  • jaundice
  • hepatomegaly
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10
Q

Investigations PBC

A

Bilirubin is secreted an eliminatinated in bile but as bile duct flow os blocker, Bilrubin will enter the bloodstream and cause jaundice

  • Alk phosph, bilirubin, chol increases in blood
  • diagnosis - anti-mitochondrial antibodies
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11
Q

Treatment PBC

A

ursodeoxycholic acid

liver transplant

osteoporosis prophylaxis

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12
Q

Primary sclerosing cholangitis definition

A
  • Chronic cholestatic LD
  • characterised by progressive obliterating fibrosis of intra and extra hepatic duts, eventually leading to cirrhosis
  • portal inflammation and fibrosis (periductal onion skinning)
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13
Q

Diagnosis of PSC

A

diagnosis:

  • asymptomatic- routine blood test in patient with IBD
  • symptomatic - pruritis, jaundic or cholangitis
  • P- ANCA -60%
  • MRCP or liver biopsy
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14
Q

PSC clinical features

A

Pruritis +/- fatigue, if advanced ascending cholangitis, cirrhosis and end-stage hepatic failure

middle aged men

uknown cause - assoc with ulcerative collitis

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15
Q

treatment of PSC

A

Treatment - management of complications with eventual transplantation

high risk for cholangiocarcinoma

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16
Q

Management and prognosis

A
  • treat the underlying cause and comolications
  • liver transplant in selected
  • surveillence using endoscopy to screen for varices every 2 years
  • USS to detect HCC
  • prognosis linked to severity
17
Q

Congenital - wilsons disease

A
  • Normally copper absorbed by the stomahc, processed in the liver and excreted in bile
  • mutation in P-Type ATPase (catson transport enzyme) normally transports copper into bile
  • copper then accumulates in liver and brain
  • causes liver disease and neuropschyiatric symptoms
18
Q

Signs of congenital wilsons disease

A
  • Liver disease (Hepatitis, cirrhosis, fulminant liver failure)
  • CNS signs - tremour, dyarthria, dysphagia, dyskinesia
  • mood - depression, personality change
  • cognition- memory decreases; quick to anger
  • Kayser-fleischer rings - cooper in iris
19
Q

Investigations of wilsons disease

A
  • urine: 2hr copper excretion studies need expert interpretation
  • LFT increase
  • serum cooper
  • serum caeruoplasmin decrease
    • falsely low caeruoplasms: protein defiiency states
    • falsely high caeruoplasmim: acute phase reactant
  • molecular genetic testing
  • slip lamp: KF rings
  • liver biopsy: hepatic copper
20
Q

Management of Wilson disease

A

Diet - avoid eating foods with a high copper content (e,g liver,chocolate, nuts, mushrooms, legumes and shellfish)

Drugs - life long penicillamine

SE: nausea, rash, WCC decrease, Hb down, lateelts decrease

Monitor FBC and urinary CU

Liver transplant

21
Q

Hereditary haemochromatosis definition

A

Inherited disorder of iron metabolism in which increased intestinal iron absoprtoin leads to iron deposition in joints, heart, pancrease and pitaru

22
Q

Congenital haemochromotosis

A
  • Accelerated rate of iron absorption from intestines
  • progressive iron deposition in various tissue
  • hepatic cirrhosis along with
    • cardiomyopathy
    • diabetes
    • arthirits
    • hyperpigmentation
    • hypopituitarism
23
Q

Clinical features

A
  • can be entirely asymptomatic
  • weakness, fatigue, muscle cramps, weight loss, non specific *weight loss, nausea and vomitting
  • liver is hard, irregular and non-tender (liver is smaller)
  • spider naevi- strong indicator of liver diease
24
Q

Genetics of HH

A

HFR gene on chromosome 6

25
Q

Clinical features of HH

A
  1. Early - NIl or tiredness, arthralgia
  2. later - slate-grey skin pigmentation, signs of CLD, hepatomegaly, cirrhosis
  3. endocrinopathies - DM bronze diabetes - iron deposition in pancreas
26
Q

HH investigations

A
  • Bloods - increase LFT
  • increased serum ferritin
  • transferrin saturation >45%
  • glucose - DM

Imaging - chondrocalcinosin

Liver biopsy - Perl stain

ECHO if cardiomyopathy suspected

27
Q

Management of HH

A

Venesection for life

monitor LFTS and diabetes

diet low in iron

28
Q

Autoimmune hepatitis definition

A

autoimmune liver disease of unknown cause characterized by suppressor T-cell defects with autoantibodies directed against hepatocyte surface antigens

29
Q

Clinical features of autoimmune hepatitis

A
  • young or middle aged women
  • 40% - acute hepatitis and signs of autoimmune disease e.g fever, malaise, ruticarial rash
30
Q

Investigations with AIH

A
  • serum bilirubin, AST, ALT and ALK phosp all increase
  • hypergammaglobulinaemia + ve autoantibodies
  • liver biopsy - mononclear infiltrate or portal and periportal areas
  • MRCP - helps exclude PSC
31
Q

Management of autoimmune hepatitis

A
  • immunosuppresant therapy - prednsiolone
  • azathioprine can be used as a steroid-sparing agent to maintain remission
  • liver transplant
32
Q

Alpha 1 anti-trypsin deficency

A

Inherited conformation disease than can be fatal. It commonly affects lung (emphysema) and liver (cirrhosis and HCC) and can present in homozygous or heterozygous forms. A1AT is a glycoprotein and one of a family of serine protease inhibitor made in the liver that control inflammatory cascades. Deficency is called a serpinopathy. It makes up 90% of serum alpa 1 - globulin on elettrophoresis

33
Q

Genetics of alpha 1 antitrypsin

A

Autosomal recessive disorder is found on chromosome 14 carrier frequency of 1:10

Genetic types by electrophoretic mobility

(M)- medium

Slow

very slow

34
Q

Investigations of alpha 1 anti-trypsin

A
  • serum alpha 1 antitrypsin
  • liver biopsy - periodic acid schiff
  • phenotyping by isolectrc focusing
35
Q

Management of alpha 1 anti-trypsin

A
  • GP
  • lung
  • liver specialists ad geneteicists
  • supprotive for emphysema and liver disease may be suffienct for some
  • quit smoking
  • liver transplant