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Flashcards in Biliary tract diseases Deck (64):
1

What is Primary Biliary Cholangitis?

An autoimmune condition causing granulomatous inflammation

There is progressive destruction of intrahepatic bile ducts

This leads to cholestasis
Which leads to cirrhosis and portal hypertension

2

What are the intrahepatic bile ducts? Where are they?

They are small ducts that run throughout the liver

Transporting bile produced in the hepatocytes through the liver to the larger ducts that take the bile to the gall bladder

3

Describe the journey of bile from its production to its storage to its release into the small intestine?

Produced by hepatocytes

Released into bile canaliculi

Into interlobular bile ducts

Into intrahepatic bile ducts

Released into the R or L hepatic duct

These merge to form the common hepatic duct

Goes into the cystic duct to reach the gall bladder

Stored in gall bladder

Released from gall bladder back down cystic duct

Goes down common bile duct into the duodenum

4

What is cholestasis?

When bile can't flow from the liver to the gall bladder so it builds up and, along with other toxins, causes damage to the liver

5

Which people are most often affected with PBC?

Women between age 40-50

6

What is the difference between Primary Biliary Cirrhosis and Primary Biliary Cholangitis?

They are the same!
Primary Biliary Cholangitis is the new name for it

7

What causes PBC?

Genetic predisposition

But the disease is set off in these people by an environmental trigger

Such as infection or pollution

8

What are the risk factors of developing PBC?

Family history
Being female
Many UTIs
Smoking
Having other autoimmune disease
Past pregnancy

9

What are the clinical features of PBC?

Often found incidentally via LFT blood tests

Pruritus (itching)
Lethargy
Jaundice
Skin pigmentation
Xanthoma

Hepatosplenomegaly

Signs of liver failure:
- ascites
- varices
- hepatic encephalopathy

10

What is Xanthoma?

Deposition of yellowish cholesterol-rich material that can appear anywhere in the body
These can appear on the skin as yellow blob-like lesions

11

What 2 types of cholestasis are there?

Hepatocellular: the hepatocytes aren't making bile

Obstructive: something is blocking the flow of bile

12

What type of cholestasis is PBC?

Obstructive

13

What are the complications of PBC?

Cirrhosis complications:
- Portal hypertension
- Encephalopathy
- Liver failure

Osteoporosis

Malabsorption of fat soluble vitamins

Coagulopathy

Hepatocellular carcinoma

14

Which are the fat soluble vitamins?

A, D, E, K

15

Why does PBC cause malabsorption of fat soluble vitamins?

Because bile is essential for the digestion of lipids
So if no bile, no lipid absorption

So fat soluble vitamins can't be absorbed

16

Why does PBC cause coagulopathy?

Because PBC causes liver damage and cirrhosis

This impairs the liver's ability to make clotting proteins

Fewer clotting proteins = less ability of blood to clot

17

Investigations of PBC?

Blood:
- raised serum alkaline phosphate
- in late disease, raised bilirubin + low albumin
- autoantibodies

USS
- exclude extra-hepatic cholestasis: problems with gall bladder or pancreas

Biopsy:
- not usually needed, but look for granulomata round bile ducts
- look for cirrhosis + scarring

18

Management of PBC?

Treat the symptoms: pruritus, diarrhoea, osteoporosis

Give fat soluble vitamin supplements to accommodate malabsorption

Drug treatment: UDCA

Regular monitoring of liver function and USS

Liver transplant

19

What is UDCA?

Ursodeoxycholic acid

It reduces ascites, jaundice and can improve survival and delay the need for transplant

20

What is the prognosis of PBC?

Not good
Once jaundice develops, less than 2 years

21

What is Primary Sclerosing Cholangitis?

Progressive obliteration, inflammation and narrowing of intra + extra-hepatic ducts

This leads to cholestasis

Eventually leading to strictures, cirrhosis and gallstones

22

What is the difference between PBC and PSC?

PBC: only affects intra-hepatic ducts, no strictures or gallstones. affects women more

PSC: affects intra and extra-hepatic ducts, strictures and gallstones, strong links with cancer, affects men more

23

Which one of PBC and PSC is linked with inflammatory bowel disease?

PSC

Over 50% have both

24

Clinical presentation of PSC?

Pruritus
Fatigue
Pain + rigors

Cirrhosis
Ascending cholangitis
Signs of liver damage
- Jaundice
- Ascites

25

What are the risk factors for PSC?

Male gender
HLA-A1

Also having IBD, most commonly UC

26

What are the complications of PSC?

Liver failure
Complications associated with this: portal hypertension, encephalopathy

Cancer:
- cholangiocarcinoma
- gall bladder adenocarcinoma
- liver
- colon

27

What is cholangiocarcinoma?

Bile duct cancer

28

Investigations of PSC?

Bloods:
- raised alkaline phosphate
- raised bilirubin
- ANCA (autoantibody)

ERCP:
- do check for extra + intra-hepatic bile duct involvement

Biopsy:
- shows cholangitis: fibrous scarred bile ducts

29

What is ANCA?

Anti-neutrophil cytoplasmic antibody

An autoantibody, linked with autoimmune disease

30

Why is it important to do an ERCP on patients with suspected PSC?

Distinguishes between PSC and PBC

ERCP will show up whether there is extra-hepatic bile duct involvement

If there is suspect PSC
If not suspect PBC

31

Management of PSC?

UDCA drug: may improve liver function and protect against colon cancer

Liver transplant

Treat symptoms: pruritus

Screen regularly for cancers of bile duct, gall bladder, liver, colon

32

What is biliary colic?

Pain related to the gall bladder associated with the temporary obstruction of the cystic duct or common bile duct
Usually caused by gallstones

33

Obstruction of which ducts in the biliary system causes biliary colic?

Where are they?

Cystic duct: from gall bladder to
common bile duct

Common bile duct: from joining of cystic duct to duodenum

34

What are the 2 types of gallstones?

Which are more common?

Cholesterol: most common - 80%

Pigment

35

Why do cholesterol gallstones form?

In bile with excess cholesterol

In gall bladders with reduced motility

36

What are pigment gallstones made of and why do they form?

Bilirubin polymers and other bilirubin compounds

So they are seen in people who have high levels of bilirubin:
- haemolysis
- sickle cell disease
- cirrhosis

37

What are the risk factors for developing cholesterol gall stones?

Older age
Being female
Family history
Multiparity: given birth to many children
Obesity
Diabetes
High fat diet

38

Investigations of gallstones?

Evidence from history

Blood:
- increased serum alkaline phosphate
- increased bilirubin
- inflammatory features could indicate cholecystitis

39

What problems can gallstones cause?

Biliary colic
Cholecystitis: inflammation of gall bladder
Cholangitis: inflammation of bile ducts
Pancreatitis

40

How do gallstones cause pancreatitis?

A stone can block the common bile duct or even move into the pancreatic duct

Blocking pancreatic enzymes from leaving the pancreas means they build up and become toxic, damaging the pancreas

41

What are the clinical features of gallstones?

Severe right upper quadrant pain
Pain radiates to back and shoulder

Vomiting
Jaundice

Pyrexia if Cholecystitis has developed

42

What is Cholecystitis?

Inflammation of the gall bladder caused by gallstones

43

What causes acute Cholecystitis?

A stone getting stuck in the neck of the gall bladder or the cystic duct

This causesa build up of pressure in the gall bladder, which then leads to inflammation of it

Very occasionally it occurs without stones

44

What causes chronic Cholecystitis?

After many attacks on the gall bladder by gallstones and acute Cholecystitis it becomes damaged.

45

How does acute Cholecystitis present compared with chronic?

Acute: RUQ pain, radiating to back, jaundice, vomiting, pyrexia, tenderness

Chronic: can be asymptomatic, vague abdominal dysfunction, nausea, fat intolerance

46

Why does fat intolerance occur in Cholecystitis?

Because fatty food stimulates the gall bladder to contract, causing pain if the gall bladder is damaged.

47

Investigations of acute Cholecystitis?

Raised white cells
Abnormal liver function tests

USS:
- shows gallstones
- distended gall bladder
- thickened wall

48

Management of acute Cholecystitis?

Nil by mouth to keep gall bladder from contracting

IV fluids, antibiotics

Analgesia

Cholecystectomy within 48 hours

49

In chronic Cholecystitis do you do a cholecystectomy?

Not unless symptoms indicate to do so

50

What is the pathophysiology of Cholecystitis?

A blockage in the cystic duct or common bile duct

When the gall bladder contracts the bile can't escape so it stretches the gall bladder and increases pressure

Causing pain

Over time the bile becomes toxic and damages the mucosa causing it to release inflammatory cytokines

Bacteria might start to grow

51

Describe what pain occurs in Cholestasis?

Mid epigastric pain to start with

As it progresses it localises in the right upper quadrant

This can radiate to the back and shoulder

52

What is choledocholithiasis?

A stone in the common bile duct

Causes blockage of bile and problems with bladder
ALSO
Acute pancreatitis as pancreatic enzymes can't get through

53

What are the clinical features of choledocholithiasis?

The same as Cholecystitis

54

How do you distinguish choledocholithiasis from Cholecystitis?

Choledocholithiasis: negative Murphy's sign

Cholecystitis: positive

55

What is Murphy's sign?

The patient is instructed to inspire

Doctor presses down on the site of the gallbladder

If the patient stops breathing in (as the gallbladder is tender and, in moving downward, comes in contact with the examiner's fingers) and winces with a 'catch' in breath, the test is considered positive.

In order for the test to be considered positive, the same manoeuver must not elicit pain when performed on the left side.

56

How do you treat choledocholithiasis?

Removal of stone with ERCP or laparoscopy

57

What is ascending cholangitis?

Infection of the biliary tree

58

Why is it called 'ascending' cholangitis?

No particular reason, it is also simply called cholangitis

59

What causes ascending cholangitis?

Choledocholithiasis: stone in common bile duct

Benign biliary stricture as a result of surgery

Chronic pancreatitis

60

Clinical features of ascending cholangitis?

Fever
Jaundice
RUQ pain
Rigors
Skin itching
Pale stools and dark urine

Shock

61

What is Charcot's triad?

A triad of symptoms that are seen in Ascending Cholangitis

1. right upper quadrant pain,
2. jaundice
3. fever

62

Investigation of ascending cholangitis?

Bloods:
- Raised white cells due to infection
- cultures of pathogen
- raised serum bilirubin + alkaline phosphatase

USS:
- dilated common bile duct
- stones visible

ERCP:
- will show the stone
- can get a bile sample

63

What pathogens usually cause ascending cholangitis?

E. coli
Enterococcus faecalis

64

Management of ascending cholangitis?

Resuscitate if in shock

Analgesia
Antibiotics

Relief of obstruction: ERCP