Biliary Disease Flashcards

(33 cards)

1
Q

What is primary biliary cholangitis?

A

A chronic liver disorder typically seen in middle-aged females, previously referred to as primary biliary cirrhosis.

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2
Q

What is the female to male ratio for primary biliary cholangitis?

A

9:1

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3
Q

What is the suspected aetiology of primary biliary cholangitis?

A

An autoimmune condition

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4
Q

What happens to the interlobular bile ducts in primary biliary cholangitis?

A

They become damaged by a chronic inflammatory process.

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5
Q

What is a classic presentation of primary biliary cholangitis?

A

Itching in a middle-aged woman

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6
Q

Which syndrome is associated with up to 80% of patients with primary biliary cholangitis?

A

Sjogren’s syndrome

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7
Q

List other associations of primary biliary cholangitis.

A
  • Rheumatoid arthritis
  • Systemic sclerosis
  • Thyroid disease
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8
Q

What are early clinical features of primary biliary cholangitis?

A
  • May be asymptomatic
  • Fatigue
  • Pruritus
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9
Q

What are late clinical features of primary biliary cholangitis?

A

May progress to liver failure

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10
Q

What is the significance of anti-mitochondrial antibodies (AMA) in primary biliary cholangitis?

A

Present in 98% of patients and are highly specific.

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11
Q

What imaging is required before diagnosing primary biliary cholangitis?

A

Right upper quadrant ultrasound or MRCP to exclude extrahepatic biliary obstruction.

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12
Q

What is the first-line management for primary biliary cholangitis?

A

Ursodeoxycholic acid

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13
Q

What is used to manage pruritus in primary biliary cholangitis?

A

Cholestyramine

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14
Q

What is a major indication for liver transplantation in primary biliary cholangitis?

A

If bilirubin > 100

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15
Q

What are some complications of primary biliary cholangitis?

A
  • Cirrhosis
  • Portal hypertension
  • Osteomalacia
  • Osteoporosis
  • Increased risk of hepatocellular carcinoma
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16
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.

17
Q

What is the association between ulcerative colitis and primary sclerosing cholangitis?

A

4% of patients with UC have PSC, 80% of patients with PSC have UC.

18
Q

What are common features of primary sclerosing cholangitis?

A
  • Cholestasis
  • Jaundice
  • Pruritus
  • Raised bilirubin + ALP
  • Right upper quadrant pain
  • Fatigue
19
Q

What diagnostic investigations are standard for primary sclerosing cholangitis?

A
  • Endoscopic retrograde cholangiopancreatography (ERCP)
  • Magnetic resonance cholangiopancreatography (MRCP)
20
Q

What is a marker of primary sclerosing cholangitis?

A

P- ANCA may be positive in patients.

21
Q

What complications are associated with primary sclerosing cholangitis?

A
  • Cholangiocarcinoma (in 10%)
  • Increased risk of colorectal cancer
22
Q

What are the adverse effects of chlestyramine?

A

Adverse effects
abdominal cramps and constipation
decreases absorption of fat-soluble vitamins
cholesterol gallstones
may raise level of triglycerides

23
Q

What is the cause of colicky abdominal pain, worse postprandially, worse after fatty foods?

A

Biliary colic

24
Q

What is the cause of Right upper quadrant pain
Fever
Murphys sign on examination?

A

Acute cholecystitis
-> Murphy’s sign is inhalation due to pain in RUQ on palpation

25
What is Mirizzi syndrome?
gallstone becomes impacted in the cystic duct or neck of the gallbladder causing compression of the common hepatic duct, resulting in obstruction and jaundice.
26
What causes right upper quadrant pain and swinging Pyrexia?
Swinging Pyrexia is lcutuations in body temp throughout a day. Caused by gallbladder abscess
27
What is the cause of sepsis, jaundice and RUQ pain?
Cholangitis
28
How is a gall bladder abscess imaged?
Ultrasound or C scan
29
What is the cause of high fever, inflammation of gallbladder without stones and COMORBIDITIY?
Acalculous cholecystitis more typical post surgery or sepsis or trauma. Higher risk of gangrene and perforation compared to calculus cholecystitis
30
What is Bouvert’s syndrome
Formation of a cholecystokinin-enteric fistula with the gallstone bladder and gut, known as Bouvert’s syndrome which causes small bowel obstruction. It presents with Rigbert’s triad
31
What is rigbert’s triad?
Associated with gallstone ileus: Pnuemboilia SBO Calcified gallstone, typically in R iliac fossa
32
What is the M rule for priamry biliary cholangitis?
IgM anti-Mitochondrial antibodies, M2 subtype Middle aged females
33
What is the management