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2a > Biliary tract disease, PBC and PSC > Flashcards

Biliary tract disease, PBC and PSC Flashcards

1
Q

What are gallstones?

A

Small stones, usually formed from cholesterol, that form in the gallbladder.

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2
Q

Give 4 risk factors for gallstones.

A 
The 4 Fs:
Fat (overweight)
Forty
Female
Fertile (pregnancy)
(PTS)
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3
Q

What is biliary colic?

A

Biliary colic is when a gallstone is temporarily impacted in the bladder neck and is dislodged back into the gallbladder.

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4
Q

What is acute cholecystitis?

A

Acute cholecystitis is when a gallstone is stuck in the bladder neck and causes inflammation.

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5
Q

Describe the physiology of bile release from the gallbladder.

A

When fatty acids reach the duodenum, enteroendocrine cells secrete cholecystokinin.
CCK travels through the blood to the gallbladder, causing it to contract and release bile.
CCK also relaxes the sphincter of Oddi, which is where bile is reduced into the duodenum.

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6
Q

Where does biliary colic pain start and radiate to?

A

Dull pain in the right upper quadrant and epigastrium

Radiates to the right shoulder.

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7
Q

What makes biliary pain worse and better?

A

Starts several hours after meal, lasts <6 hours, then the gallstone dislodges back into the gallbladder and the pain goes away. Occurs when lying flat because it is easier for gallstones to get stuck in this position.

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8
Q

Give 3 symptoms of biliary colic.

A

Nausea, vomiting and sweating.

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9
Q

How is acute cholecystitis differentiated from biliary colic?

A

There is inflammation so there is fever.

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10
Q

How is biliary colic diagnosed?

A

Based on recurrent symptoms and confirmed by ultrasound.

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11
Q

Describe the management of biliary colic.

A
  1. Pain and symptom management

2. Cholecystectomy (removal of gallbladder)

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12
Q

Give 3 features of acute cholecystitis.

A

RUQ pain, nausea/vomiting, fever.

lecture

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13
Q

What is Murphy’s sign?

A

The patient inhales while the examiner hooks their fingers under the liver border. The inspiration causes the gallbladder to descend onto the fingers, causing pain if the gallbladder is inflamed. This is a sign of gallbladder disease.

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14
Q

How is acute cholecystitis diagnosed?

A

USS abdomen.
LFTs to exclude bile duct/liver pathology.
(PTS)

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15
Q

Describe the management of acute cholecystitis.

A

IV morphine and fluids
Laparoscopic cholecystectomy.
(PTS)

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16
Q

What is the pathophysiology of ascending cholangitis?

A

Infection and inflammation of the biliary tree (bile/gallstone) secondary to impacted stone or stricture in the common bile duct (choledocholithiasis). Bacteria from the intestine ascend up the biliary tree and are not washed away by bile. (As opposed to cholecystitis which is inflammation of gallbladder only).

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17
Q

What organisms are likely to cause ascending cholangitis?

A

E. Coli, klebsiella, enterococcus.

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18
Q

Give 5 clinical features of ascending cholangitis.

A

Charcot’s triad: RUQ pain, fever, jaundice
Reynold’s pentad:+ hypotension + confusion
(PTS)

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19
Q

Describe the diagnosis of ascending cholangitis.

A

Bloods - microbiological cultures and staining
USS abdo,
MRCP (Magnetic resonance cholangiopancreatography, like MRI) to detect stone

20
Q

Describe the management of ascending cholangitis.

A

Treat symptoms: antibiotics, rehydration.
ERCP: Endoscopic retrograde cholangio-pancreatography, to remove gallstone.
Cholecystectomy to avoid future complications

21
Q

How could ascending cholangitis cause change in mental state and consciousness level?

A

Pressure on the bile duct walls can cause decreased junctional contact between the cells, allowing bacteria and bile to move out into the blood. This causes jaundice, fever and septic shock - the septic shock causes these symptoms so be aware!
(osmosis)

22
Q

What is primary biliary cholangitis?

A

An autoimmune disease of the liver in which bile ducts are damaged, causing cholestasis.
(wiki)

23
Q

Describe the pathophysiology of primary biliary cholangitis.

A

Molecular mimicry theory: bile duct mitochondrial proteins may be similar to those of a pathogen and some people’s immune systems cannot tell the difference (decreased immune tolerance). Attack on intrahepatic bile duct cells by AMAs -> cholestasis (no bile flow) -> fibrosis, cirrhosis and portal hypertension.

24
Q

What causes primary biliary cholangitis?

A

Less of immune tolerance may be due to environmental triggers eg bacteria, pollutants and genetic predisposition.

25
Q

Give 3 symptoms of PBC and why they occur.

A

Often asymptomatic
Fatigue (80%), sleepiness (malabsorption?)
pruritus (deposit of bile products in skin)

26
Q

Give 3 signs of PBC.

A

Jaundice (later), skin pigmentation, xanthelasma/xanthomata (lipid depositions), hepatosplenomegaly.

27
Q

Give 3 risk factors of PBC.

A 
Female (9:1) - AI more common in women
Positive family history
Other AI disease eg Sjorgen's, RA.
Recurrent UTIs
Smoking, hair dye and nail polish (octynoic acid)
Past pregnancy
Typical age at presentation 50 years
(OHCM)
28
Q

What investigations would you do if you suspect PBC?

A
  1. Bloods: Antimitochondrial antibodies, IgM. (+Increased ALP, AST, ALT. Later: raised bilirubin and PTT, low albumin.)
  2. Abdo USS to exclude extrahepatic cholestasis.
29
Q

Give 3 complications of PBC.

A

Cirrhosis complications: portal hypertension; hepatic failure (->coagulopathy)
Fat-soluble vitamin (A,D,E+K) malabsorption: Osteomalacia, coagulopathy.
Osteoporosis (due to malabsorption?)
Risk of hepatocellular carcinoma
(OHCM)

30
Q

Why can PBC cause osteomalacia and how is it treated?

A

Malabsorption of fat-soluble vitamins (ADEK) due to cholestasis and decreased bilirubin in the gut lumen. This also causes coagulopathy. Therefore give fat-soluble vitamin prophylaxis.
(OHCM)

31
Q

What is PSC?

A

Primary sclerosing cholangitis.

32
Q

What is ALT, where is it found and what is it a marker of?

A

Alanine transaminase
Found in high concentrations in hepatocytes
Enters blood following hepatocellular injury.
(geekymedics)

33
Q

What is ALP, where is it found and what is it a marker of?

A

Alkaline phosphatase
In the liver, bile duct and bone tissues
Raised in liver pathology due to increased synthesis in response to cholestasis.

34
Q

What is GGT and what is it suggestive of?

A

Gamma-glutamyl transferase
Raised in response to biliary epithelial damage and bile obstruction as well as alcohol and phenytoin.
Should be measure if ALP is raised. Raused ALP + GGT implies cholestasis.

35
Q

What is AST and what is it a marker of?

A

Aspartate aminotransferase
AST:ALT ratio is used to determine cause of LFT derangement.
ALT>AST –> CKD
AST>ALT –> cirrhosis, acute alcoholic hepatitis.
(geekymedics)

36
Q

Describe the pathophysiology of PSC.

A

PSC is likely to be autoimmune. Inflammation of the intra- and extrahepatic ducts (cholangitis) resulting in scarring, strictures and sclerosis of the ducts. Resulting blockage causes progressive cholestasis.

37
Q

Give 3 symptoms of PSC and explain why they occur.

A
  1. Deposition of bile products in the skin causes pruritus
  2. Fatigue (malasorption?)
  3. Abdominal pain (inflammation pushing on nerves?)
    (often asymptomatic)
38
Q

Give 3 signs of PSC and explain why they occur.

A
  1. Hepatosplenomegaly due to portal hypertension
  2. Portal hypertension - inflammation pushes on and constricts portal veins.
  3. Hepatic failure and cirrhosis due to damage by inflammation.
39
Q

Give 3 risk factors associated with PSC.

A 
Age usually 10-30 (younger than PBC)
IBD especially colitis
Autoimmune hepatitis
Male slightly higher 
HLA-A1, B8, DR3.
40
Q

What investigations would you do for PSC?

A
  1. LFTs: ALP (raised)
  2. Auto-abs: p-ANCA +ve, AMA-ve
  3. Imaging: ERCP/MRCP shows ‘beaded’ appearance of ducts due to inconsistent strictures.
41
Q

Why is ALP raised in PSC?

A

It is produced in response to cholestasis.

42
Q

Describe the management of PSC.

A
  1. Liver transplant for end-stage disease
  2. Pruritis: colestyramine (forms complexes with excess bile acid)
  3. Bacterial cholangitis: antibiotics.
43
Q

What affects prognosis in PSC?

A

5 year graft survival >60%.

Prognosis is worse for PSC with IBD, due to colorectal cancer risk.

44
Q

Give 3 cancers which are more common with PSC.

A

Cholangiocarcinoma (bile duct cancer), gallbladder cancer, liver cancer, colon cancer.
(Do yearly colonoscopy and ultrasound.)

45
Q

Which parts of the bile duct do PBC and PSC each affect?

A

Intra-and extra-hepatic ducts, while PBC only affects the intrahepatic duct.

2a (75 decks)

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