Hepatomegaly Flashcards

1
Q

What is the epidemiology of primary biliary cholangitis

A
  • Chronic disease of the small intrahepatic ducts, characterised by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation.
  • Fibrosis and secondary effects of retained bile acids
  • Consequent cirrhosis

Female: Male 10:1

>45 yrs

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2
Q

How does primary biliary cholangitis present

A
  • Hx of hypercholesterolaemia
  • Itch
  • Fatigue
  • Hepatomegaly
  • Dry eyes and dry mouth - associated Sjogren’s
  • Sleep disturbance
  • Weight loss
  • Jaundice
  • Ascites
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3
Q

What investigations may you order in suspected primary biliary cholangitis

A

Liver function tests

  • Alk Phos - ++
  • Gamma GT - +
  • Bilirubin, albumin - + - suggestive of impaired synthetic ability of liver
  • AST/ALT - +

Auto-antibodies

  • Anti-mitochondrial antibody
  • ANA
  • ANCA

Imaging

  • MRCP
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4
Q

How do you manage primary biliary cholangitis

A

Ursodeoxycholic acid - bile acid analogue

  • long term therapy

Immunomodulation e.g. prednisolone

Anti-pruritic - colestyramine

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5
Q

What are some complications of primary sclerosing cholangitis

A
  • Acute bacterial cholangitis
  • Cholangiocarcinoma
  • Colorectal cancer
  • Cirrhosis and liver failure
  • Fat soluble vitamin deficiency
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