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Gwen - GI and General surgery > Hepato-biliary: Gallbladder and Biliary tree Disease > Flashcards

Hepato-biliary: Gallbladder and Biliary tree Disease Flashcards

1
Q

What is primary sclerosing cholangitis?

A

Chronic progressive cholestatic liver disease, characterised by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts, resulting in diffuse, multi-focal stricture formation

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2
Q

What is primary sclerosing cholangitis associated with?

A
  • IBD
  • HLA-A1; B8, DR3
  • Male
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3
Q

What is the main pathophysiological process going on in primary sclerosing cholangitis?

A

Inflammation and injury of the medium- and large-sized bile ducts leading to fibrosis and multi-focal stricturing of the ducts.

Obstruction of the medium- and large-sized bile ducts leads to progressive fibrosis and ultimately obliteration of the smaller ducts (ductopenia) and bile stasis (cholestasis)

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4
Q

What cancers are associated with primary sclerosing cholangitis?

A
  • Cholangiocarcinoma - bile duct, gallbladder
  • Colon cancer
  • Liver cancer
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5
Q

What autoantibody can be found in the serum of those with primary sclerosing cholangitis?

A

pANCA - 60%

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6
Q

What are symptoms of primary sclerosing cholangitis?

A
  • Pruritus + Fatigue
  • Can have RUQ/epigastric pain
  • Features of jaundice
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7
Q

What are signs of primary sclerosing cholangitis?

A
  • Excoriations (from pruritus)
  • Weight loss - from fat malabsorption/active IBD/ advanced liver disease
  • Jaundice
  • Splenomegaly
  • Ascites
  • Encephalopathy
  • Oesophageal variceal bleeding
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8
Q

What sex does primary sclerosing cholangitis most commonly occur in?

A

Male

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9
Q

What tests would you consider doing in someone suspected to have primary sclerosing cholangitis?

A
  • Bloods - LFTs, serum albumin, FBC, PT, ANCA, ANA
  • MRCP
  • Consider ERCP
  • Abdo US
  • Consider iver biopsy
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10
Q

What might you find on LFT in someone with primary sclerosing cholangitis?

A
  • Elevated GGT
  • Elevated ALP
  • Moderately elevated AST/ALT
  • Hyperbilirubinaemia - conjugated
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11
Q

In someone with PSC, what is the predominating type of bilirubin present if they have hyperbilirubinaemia?

A

Conjugated bilirubin

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12
Q

What might you find when assessing serum albumin in someone with PSC?

A

Normal in early disease, low in advanced liver disease

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13
Q

What might you find on FBC in someone with PSC?

A

Indication of liver synthetic dysfunction due to advanced liver disease

  • Normal, or
  • Thrombocytopaenia +/- anaemia, leukopenia
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14
Q

Why would you perform PT in someone with PSC?

A

Assess liver synthetic function - can be prolonged in someone with lvier failure

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15
Q

What investigations reveal duct anatomy?

A
  • MRCP
  • ERCP
  • CT Abdo
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16
Q

What might you find on liver biopsy in someone with PSC?

A

Fibrous, obliterative cholangitis

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17
Q

How would you manage someone with PSC?

A
  • Supportive - pruritis relief, lifestyle change
  • Consider immunosuppression
  • Consider bisphosphonates - if osteopenic/porotic
  • End stage disease - liver transplantation
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18
Q

What are complications that can arise in PSC?

A
  • Liver failure/cirrhosis
  • Cancer - hepatocellular, cholangiocarcinoma, colon, gallbladder
  • Dominant biliary stricture
  • Bacterial cholangitis
  • Choledocholithiasis
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19
Q

What is primary biliary cirrhosis/cholangitis?

A

Primary biliary cholangitis (PBC) is a chronic disease of the small intrahepatic bile ducts that is characterised by progressive bile duct damage (and eventual loss) occurring in the context of chronic portal tract inflammation.

Fibrosis develops as a consequence of the original insult and the secondary effects of toxic bile acids retained in the liver, resulting ultimately in cirrhosis

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20
Q

What is the difference between PBC and PSC in terms of where it affects the biliary tree?

A
  • PBC - intrahepatic ducts
  • PSC - intra- and extrahepatic bile ducts
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21
Q

What type of inflammation occurs in primary biliary cirrhosis?

A

Autoimmune granulomatous inflammation

22
Q

What sex does primary biliary cirrhosis commonly affect?

A

Females

23
Q

What is the patholphysiology of PBC?

A

Granulomatous inflammation that surrounds and destroys bile ducts is characteristic early in the disease, leading to loss of intra-hepatic bile ducts and cholestasis, which may progress to firbrosis, cirrhosis and portal hypertension.

24
Q

What antibodies are the hallmark of PBC?

A

Antimitochondrial antibodies (AMA) - genetic predisposition causes loss of immune tolerance of self-mitochondrial proteins

25
Q

What are risk factors for the development of PBC?

A
  • Family history
  • Recurrent UTIs
  • Smoking
  • Past Pregnancy
  • Other autoimmune disease
26
Q

What are symptoms of PBC?

A
  • Lethargy
  • Sleepiness
  • Pruritis
  • Jaundice
  • Features of other autoimmune disease - dry eyes
27
Q

What are signs of PBC?

A
  • Jaundice - not commonly seen
  • Skin pigmentation
  • Xanthelasma/Xanthomata
  • Hepatosplenomegaly - indicative of portal HTN
  • Ascites
  • Weight loss
28
Q

How would you investigate for PBC?

A
  • Bloods - LFTs, Serum albumin, AMA, ANA, PT, Cholesterol, TSH
  • Abdo US
  • Liver Biopsy
29
Q

What might you find on LFT in someone with PBC?

A
  • Elevated ALP
  • Elevated GGT
  • Hyperbilirubinaemia
  • Elevated ALT/AST
30
Q

What might you find on investigation of albumin in someone with PBC?

A

May be reduced in liver disease

31
Q

What might you find on investigation of PT in someone with PBC?

A

Prolonged - due to liver disease

32
Q

Why might you consider doing abdo ultrasound in someone with suspected PBC?

A

Excludes obstructive duct lesions - Obstructive lesions must always be excluded radiologically before the diagnosis of primary biliary cholangitis is made.

33
Q

What might you find on liver biopsy in someone with PBC?

A
  • Bile duct lesions - biliary ductular cell disruption within inflamed portal tracts
  • Granulomata formation
  • Bile duct loss (ductopenia) with progressive biliary fibrosis
34
Q

What might you find on investigation of cholesterol in someone with PBC?

A

Hypercholesterolaemia

35
Q

How would you manage PBC?

A

Early disease

  • Symptomatic relief
    • Pruritis - Colestyramine
    • Diarhroea - Codeine
  • Bile acid analogue - Ursodeoxycholic acid
  • Fat soluble vitamin replacement - Vit A, D, K
  • Bisphosphonates - osteoporosis risk

End-stage disease

  • Liver transplantation
36
Q

What are complications of PBC?

A
  • Hepatoma
  • Cirrhosis
  • Osteoporosis
  • Hypercholesterolaemia
37
Q

What is cholestasis?

A

Accumulation of bile in canaliculi between perivenular hepatocytes. The resulting liver injury results in accumulation of pigmented Kupffer cells in sinusoids.

The two basic distinctions are an obstructive type of cholestasis where there is a mechanical blockage in the duct system, and metabolic types which are disturbances in bile formation that can occur because of genetic defects or acquired as a side effect of many medications.

38
Q

What autoimmune diseases are associated with PBC?

A
  • Sjögren’s syndrome
  • Scleroderma
  • Thyroid disease
  • Keratoconjunctivitis sicca
  • Coeliac disease
39
Q

What is a choledochal cyst?

A

Congenital malformation of the bile ducts may occur at all levels of the biliary tree, although most commonly are extrahepatic. The resulting dilatation of the choledochus may be saccular, diverticular or of fusiform configuration

40
Q

How do choledochal cysts present?

A
  • Features of cholangitis in childhood
  • Features of choledocholithiasis in adults
41
Q

Why must choledochal cysts be resected?

A
  • Avoid recurrent biliary complications
  • Reduce risk of chlangiocarcinoma
42
Q

What are causes of benign bile duct strictures?

A
  • Biliary surgery
  • Major trauma RUQ
  • Mirrizi’s Syndrome
  • Pyogenic infection
  • Parasitic infestation
  • PSC
  • Pancreatitis
  • HIV
43
Q

How would you manage benign bile duct stricture?

A

Balloon dilatation + temporary stenting

44
Q

Where in the biliary tree can cholangiocarcinoma occur?

A

Intra or extrahepatic

45
Q

What are causes of cholangiocarcinoma?

A
  • Liver Flukes
  • Alcoholic liver disease
  • PSC
  • Choledocal cyst
  • CAroli’s disease
  • HBV/HCV
  • Diabetes
  • Toxins
46
Q

What are clinical features of cholangiocarcinoma?

A
  • Fever
  • Abdo Pain
  • Obstructive jaundice – no pain
  • Itching
  • Weight loss
47
Q

How are extrahepatic cholangiocarcinomas divided in terms of location?

A
  • Perihilar
  • Distal
48
Q

What is the most common type of cholangiocarcinoma; intra- or extra-hepatic?

A

Extra-hepatic

49
Q

What investigations would you consider doing in someone who you suspected might have cholangiocarcinoma?

A
  • Bloods - LFTs, PT, serum CA 19-9/CA-125
  • Abdo ultrasound
  • Consider CT/MRI abdo/MRCP/ERCP
  • Consider PET scan
  • Biopsy and cytology
50
Q

How would you manage someone with cholangiocarcinoma?

A
  • Surgical resection - only curative option
  • Palliative interventions
51
Q

What palliative procedures can be done to paliate someone with chlangiocarcinoma?

A
  • Surgical bypass
  • Stenting (Percutaneous vs Endoscopic)
  • Palliative radiotherapy
  • Chemotherapy
  • PDT
  • Liver transplant (not standard)

Gwen - GI and General surgery (44 decks)

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