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Boards And Beyond > Biliary Disease > Flashcards

Biliary Disease Flashcards

1
Q

Gallbladder bladder carcinoma is a rare carcinoma
True or false

A

True

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2
Q

What type of carcinoma is a gallbladder carcinoma

A

An adenocarcinoma

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3
Q

What is the strongest risk factor for gallbladder carcinoma

A

Gallstone disease

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4
Q

What kind of inflammation is associated with gallbladder carcinoma

A

Chronic inflammation

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5
Q

Which gallstone disease mostly causes a gallbladder carcinoma

A

A chronic gallstone disease leading to a porcelain gallbladder

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6
Q

What is the usual treatment for a patient with gallstone disease

A

Cholecystectomy

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7
Q

Which bacterial infection is also a risk factor for gallbladder carcinoma

A

Chronic salmonella infection (S. typhi)

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8
Q

What type of jaundice could a gallbladder carcinoma cause

A

Obstructive jaundice

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9
Q

How is a GC diagnosed

A

Ultrasound

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10
Q

What is the treatment for GC

A

Cholecystectomy (to remove the gallbladder and the cancer)

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11
Q

In general a GC has a ……… prognosis (good or poor)

A

Poor

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12
Q

What is a cholangiocarcinoma

A

An adenocarcinoma arising from bile duct epithelial cells
Can arise intra-hepatic or extra-hepatic

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13
Q

What are the extra-hepatic bile ducts

A

Common bile duct
Hepatic duct
Cystic duct

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14
Q

Which epithelial cells line the hepatic bile duct, the common bile duct and the cystic duct

A

Bile duct epithelial cells

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15
Q

What are the presenting symptoms of cholangiocarcinoma

A

Bile duct obstruction
Abdominal pain (RUQ)
Anorexia
Weight loss
Pruritus
Dark urine and clay-colored stools and jaundice
Most patients have no predisposing conditions

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16
Q

Which two key risk factors of cholangiocarcinoma could lead to chronic biliary inflammation

A

Primary sclerosing cholangitis (ulcerative colitis)
Clonorchis sinensis (Chinese liver fluke)

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17
Q

When a patient has things like jaundice and right upper quadrant pain, the first test that’s usually done is an RUG ultrasound because you’re investigating for conditions like …………. In which condition, do you have RUQ pain and jaundice but no sign of common duct stone or pancreatic cancer, especially when a patient has a risk factor like primary sclerosing cholangitis

A

A common bile duct stone or pancreatic cancer

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18
Q

How is a cholangiocarcinoma diagnosed

A

CT
MRI
MRCP
EUS (endoscopic ultrasound)
Depending on whether you think the lesion is intra or extra-hepatic

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19
Q

How are cholangiocarcinomas treated

A

Surgery
Radiation
Chemotherapy

Surgery only for localized disease
Poor prognosis

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20
Q

What is biliary cirrhosis

A

Liver damage secondary to biliary obstruction
Chronic obstruction of bile flow leads to liver damage

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21
Q

What are some things which could cause an obstruction to bile flow

A

Gallstones
Pancreatic cancer
Biliary strictures

22
Q

Biliary strictures could be treated by

A

Stenting of bile ducts

23
Q

What is the former name for primary biliary cholangitis

A

Primary biliary cirrhosis

24
Q

What is primary biliary cholangitis

A

Biliary cirrhosis without extra-hepatic obstruction (no secondary cause of poor blood flow). It is an autoimmune disorder where T-cells attack on bile ducts which cause granules Marius inflammation. Most patients who have this condition have other autoimmune disorders as well

25
Q

What size of bile ducts does a primary biliary cholangitis affect

A

Small to medium sized bile ducts

26
Q

Primary biliary cholangitis is more common in which gender

A

Women

27
Q

What are the most common initial symptoms of primary biliary cholangitis

A

Fatigue and pruritus

28
Q

The fatigue in primary biliary cholangitis is often due to

A

Systemic inflammation

29
Q

The pruritis in primary biliary cholangitis is often due to

A

The poor flow of bile into the intestines so bile acids and bile salts accumulate in the serum and they deposit in the skin which often causes intense itching, which may be severe especially at night (what often brings people to medical attention)

30
Q

The pruritus in a primary biliary cholangitis often precedes development of jaundice so it is not always obvious initially that you’re dealing with a biliary disorder
True or false

A

True

31
Q

How is primary biliary cholangitis diagnosed

A

Elevated alkaline phosphatase (usually to 3-5 times ULN (140 IU/L). In primary biliary cholangitis, alkaline phosphatase level 500+ are common
Mild elevations in the AST and ALT
Rise in bilirubin (associated with poor prognosis because it occurs late)
Anti-mitochondrial antibodies (present in 95% of patients)
Anti-nuclear antibodies seen in ~ 70% (non-specific)
Elevated serum lipids (TC > 1000 can be seen), xanthomas (corneal arcus) may occur
Imaging shows absence of biliary obstruction (because in this condition biliary obstruction is intra-hepatic not extra-hepatic)
Liver biopsy (not often done)

32
Q

What test is the hallmark of primary biliary cholangitis

A

Anti-mitochondrial antibodies

33
Q

What is the gold standard for a primary biliary cholangitis (PBC)

A

Liver biopsy

34
Q

Woman with itching and fatigue
LFT shows markedly elevated Alk Phos
Positive anti-mitochondrial antibodies
What condition may this be

A

Primary biliary cholangitis

35
Q

How is primary biliary cholangitis treated

A

Ursodeoxycholic acid
Liver transplant

36
Q

What is the initial therapy for all patients with primary biliary cholangitis

A

Ursodeoxycholic acid

37
Q

Similar to other bile acids but less toxic to hepaticytes
Replaces endogenous bile acids
Improves LFTd and disease progression
Treatment for PBC
What treatment could this be

A

Ursodeoxycholic acid

38
Q

What is the most common associated disorder of PBC

A

Sjögren’s syndrome

39
Q

What are some common associated disorders of PBC

A

Sjögren’s syndrome
Osteoporosis (poorly understood mechanism, serum calcium usually normal)

40
Q

What is primary sclerosing cholangitis

A

It is an autoimmune disorder which causes inflammation, fibrosis and especially strictures in the biliary tree. This is going to obstruct the flow of bile from the liver into the intestines and cause signs and symptoms of biliary obstruction. It involves both intra and extra-hepatic bile ducts

41
Q

How is primary sclerosing cholangitis differentiated from primary biliary cholangitis

A

Primary sclerosing cholangitis involves both the intra and extra-hepatic bile ducts. Biliary cholangitis is limited to the liver whereas in sclerosing cholangitis much of the problem is outside the liver in the common bile ducts and other elements of the biliary tree. Those conduits because obstructed by strictures

42
Q

Primary sclerosing cholangitis is strongly associated with

A

Ulcerative colitis

43
Q

What are the clinical features of PSC

A

Related to strictures that obstruct bile flow
Symptoms of biliary obstruction (RUQ pain, fatigue, jaundice, pruritus)
Often asymptomatic
Identified by abnormal LFTs on routine labs

44
Q

The hallmark lab finding for PSC is evidence of what condition

A

Cholestasis

45
Q

What are the lab findings in PSC

A

Elevated alkaline phosphatase
Elevated conjugated bilirubin
Usually mildly elevated AST/ALT
When cholestasis on their lab testing identified (listed above), patients are usually referred for biliary imaging and that’s how diagnosis is made
Besides laboratory evidence of cholestasis:
Positive p-ANCA antibodies (up to 80% in patients - also seen in UC)
Elevated IgM levels (up to 50% of patients)
Associated with HLA-DRw53a

46
Q

How do you diagnose PSC

A

Suspected from cholestasis, especially in patients with ulcerative colitis
Ultrasound (often non-diagnostic, may show abnormal bile ducts - gallstone in the common bile duct or a pancreatic tumor)
Cholangiogram (can be done endoscopically through an ERCP or can be done as an MRCP - MRI version of cholangeography - tests shows strictures and dilatations (beading))
Liver biopsy (rarely diagnostic)

47
Q

In which biliary disease is liver biopsy often diagnostic

A

Primary biliary cirrhosis

48
Q

What imaging confirms the diagnosis in a PSC

A

Cholangiogram

49
Q

Why is liver biopsy rarely diagnostic in a PSC

A

The problem is in the bile ducts especially the extra-hepatic bile ducts so in liver biopsy you rarely get a diagnostic result

50
Q

How is PSC treated

A

Endoscopic therapy (dilatation or stenting of strictures in bile ducts)
Liver transplant if patients develops a cirrhosis from recurrent biliary obstructions

51
Q

Patients with which conditions need regular screening for cholangiocarcinoma

A

Primary sclerosing cholangitis

*These patients have chronic inflammation of the bile duct epithelial cells and that puts them at high risk for cholangiocarcinoma

52
Q

Which tumor marker is measured to check for primary sclerosing cholangiocarcinoma

A

CA 19-9

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