Biliary/Pancreatic Disease

Question 1

Primary sclerosing cholangitis is

Answer 1

a biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.

Question 2

Primary sclerosing cholangitis associations?

Answer 2

ulcerative colitis
Crohn’s (much less common association than UC)
HIV

Question 3

?% of patients with UC have PSC, ?% of patients with PSC have UC

Answer 3

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

Question 4

PSC bloods

Answer 4

raised bilirubin + ALP

Question 5

PSC sx

Answer 5

cholestasis
jaundice, pruritus
right upper quadrant pain
fatigue

Question 6

PSC ix

Answer 6

endoscopic retrograde cholangiopancreatography (ERCP)

or magnetic resonance cholangiopancreatography (MRCP)

are the standard diagnostic investigations, showing multiple biliary strictures giving a ‘beaded’ appearance

Question 7

PSC which ANCA might be positive

Answer 7

p-ANCA may be positive

Question 8

PSC always use liver biopsy

Answer 8

false

limited role for liver biopsy,

Question 9

PSC what would liver biopsy show

Answer 9

fibrous, obliterative cholangitis often described as ‘onion skin’

Question 10

PSC complications

Answer 10

cholangiocarcinoma (in 10%)

increased risk of colorectal cancer

Question 11

Primary biliary cholangitis is a chronic liver disorder typically seen in middle-aged females (female:male ratio of 9:1)

Answer 11

true

Question 12

Primary biliary cholangitis aetiology is not fully understood although it is thought to be an autoimmune condition

Answer 12

true

Question 13

Primary biliary cholangitis classic presentation

Answer 13

itching in a middle-aged woman

Question 14

Primary biliary cholangitis pathophysiology?

Answer 14

Interlobular bile ducts become damaged by a chronic inflammatory process causing progressive cholestasis which may eventually progress to cirrhosis

Question 15

Primary biliary cholangitis associations

Answer 15

Sjogren’s syndrome (seen in up to 80% of patients)
rheumatoid arthritis
systemic sclerosis
thyroid disease

Question 16

PBC sx

Answer 16

early: may be asymptomatic or fatigue, pruritus
cholestatic jaundice
hyperpigmentation, especially over pressure points
around 10% of patients have right upper quadrant pain
xanthelasmas, xanthomata
also: clubbing, hepatosplenomegaly
late: may progress to liver failure

Question 17

PBC LFTs

Answer 17

raised ALP on routine LFTs

Question 18

PBC diagnosis?

Answer 18

anti-mitochondrial antibodies (AMA) M2 subtype are present in 98% of patients and are highly specific
smooth muscle antibodies in 30% of patients
raised serum IgM

Question 19

PBC mx

Answer 19

first-line: ursodeoxycholic acid
slows disease progression and improves symptoms
pruritus: cholestyramine
fat-soluble vitamin supplementation

Question 20

PBC liver is transplanted is indicated when?

Answer 20

if bilirubin > 100 (PBC is a major indication)

recurrence in graft can occur but is not usually a problem

Question 21

PBC complications

Answer 21

cirrhosis → portal hypertension → ascites, variceal haemorrhage

osteomalacia and osteoporosis

significantly increased risk of hepatocellular carcinoma (20-fold increased risk)

Question 22

Viral hepatitis common symptoms include:

Answer 22

nausea and vomiting, anorexia
myalgia
lethargy
right upper quadrant (RUQ) pain

Question 23

Viral hepatitis Questions may point to risk factors such as

Answer 23

foreign travel or intravenous drug use.

Question 24

Congestive hepatomegaly sx

Answer 24

The liver only usually causes pain if stretched

One common way this can occur is as a consequence of congestive heart failure.

Question 25

Biliary colic sx

Answer 25

RUQ pain, intermittent, usually begins abruptly and subsides gradually. Attacks often occur after eating. Nausea is common.

Question 26

Acute cholecystitis sx

Answer 26

Pain similar to biliary colic but more severe and persistent. The pain may radiate to the back or right shoulder. The patient may be pyrexial and Murphy's sign positive (arrest of inspiration on palpation of the RUQ)

Question 27

Ascending cholangitis sx

Answer 27

An infection of the bile ducts commonly secondary to gallstones. Classically presents with a triad of: fever (rigors are common) RUQ pain jaundice

Question 28

Gallstone ileus sx

Answer 28

Abdominal pain, distension and vomiting are seen.

Question 29

Cholangiocarcinoma sx

Answer 29

Persistent biliary colic symptoms, associated with anorexia, jaundice and weight loss. A palpable mass in the right upper quadrant (Courvoisier sign), periumbilical lymphadenopathy (Sister Mary Joseph nodes) and left supraclavicular adenopathy (Virchow node) may be seen

Question 30

Acute pancreatitis sx

Answer 30

Usually due to alcohol or gallstones Severe epigastric pain Vomiting is common Examination may reveal tenderness, ileus and low-grade fever Periumbilical discolouration (Cullen's sign) and flank discolouration (Grey-Turner's sign) is described but rare

Question 31

Pancreatic cancer sx

Answer 31

Painless jaundice is the classical presentation of pancreatic cancer. However pain is actually a relatively common presenting symptom of pancreatic cancer. Anorexia and weight loss are common

Question 32

Amoebic liver abscess sx

Answer 32

Typical symptoms are malaise, anorexia and weight loss. The associated RUQ pain tends to be mild and jaundice is uncommon.

Question 33

Bile-acid malabsorption is a cause of

Answer 33

chronic diarrhoea

Question 34

Bile-acid malabsorption may be primary, due to

Answer 34

excessive production of bile acid, or secondary to an underlying gastrointestinal disorder causing reduced bile acid absorption.

Question 35

Bile-acid malabsorption leads to

Answer 35

steatorrhoea and vitamin A, D, E, K malabsorption.

Question 36

Bile-acid malabsorption secondary causes?

Answer 36

patients with ileal disease, such as with Crohn's. Other secondary causes include: cholecystectomy coeliac disease small intestinal bacterial overgrowth

Question 37

Bile-acid malabsorption ix

Answer 37

the test of choice is SeHCAT nuclear medicine test using a gamma-emitting selenium molecule in selenium homocholic acid taurine or tauroselcholic acid (SeHCAT) scans are done 7 days apart to assess the retention/loss of radiolabelled 75SeHCAT

Question 38

Bile-acid malabsorption mx

Answer 38

bile acid sequestrants e.g. cholestyramine

Question 39

Raised levels of unconjugated bilirubin may occur as a result of

Answer 39

haemolysis, which is to say a pre-hepatic source, for example, autoimmune-mediated haemolytic anaemia. Red blood cell breakdown exposes heme-containing proteins and, as discussed above, these are then processed to form unconjugated bilirubin.

Question 40

Raised levels of conjugated bilirubin can result from

Answer 40

defective excretion of bilirubin, for example, Dubin-Johnson Syndrome, or cholestasis.

Question 41

Jaundice starts to appear when bilirubin reaches an excess of

Answer 41

35umol/l

Question 42

Gallstones ix

Answer 42

abdominal ultrasound and liver function tests

Question 43

Biliary colic mx

Answer 43

If imaging shows gallstones and history compatible then laparoscopic cholecystectomy

Question 44

Acute cholecystitis mx

Answer 44

Imaging (USS) and cholecystectomy (ideally within 48 hours of presentation) (2)

Question 45

Gallbladder abscess mx

Answer 45

Imaging with USS +/- CT Scanning Ideally, surgery although subtotal cholecystectomy may be needed if Calot's triangle is hostile In unfit patients, percutaneous drainage may be considered

Question 46

Cholangitis mx

Answer 46

Fluid resuscitation Broad-spectrum intravenous antibiotics Correct any coagulopathy Early ERCP

Question 47

Gallstone ileus mx

Answer 47

Laparotomy and removal of the gallstone from small bowel, the enterotomy must be made proximal to the site of obstruction and not at the site of obstruction. The fistula between the gallbladder and duodenum should not be interfered with.

Question 48

Acalculous cholecystitis mx

Answer 48

If patient fit then cholecystectomy, if unfit then percutaneous cholecystostomy

Question 49

Risks of ERCP(1)

Answer 49

Bleeding 0.9% (rises to 1.5% if sphincterotomy performed) Duodenal perforation 0.4% Cholangitis 1.1% Pancreatitis 1.5%

Question 50

Pancreatic cancer is often diagnosed late as

Answer 50

it tends to present in a non-specific way

Question 51

Over 80% of pancreatic tumours are ?

Answer 51

adenocarcinomas

Question 52

Over 80% of pancreatic tumours typically occur where

Answer 52

at the head of the pancreas.

Question 53

Pancreatic cancer assoc

Answer 53

increasing age smoking diabetes chronic pancreatitis (alcohol does not appear an independent risk factor though) hereditary non-polyposis colorectal carcinoma multiple endocrine neoplasia

Question 54

Pancreatic cancer genetics

Answer 54

BRCA2 gene | KRAS gene mutation

Question 55

Pancreatic cancer sx

Answer 55

classically painless jaundice pale stools, dark urine, and pruritus patients typically present in a non-specific way with anorexia, weight loss, epigastric pain loss of exocrine function (e.g. steatorrhoea) loss of endocrine function (e.g. diabetes mellitus) atypical back pain is often seen

Question 56

cholestatic liver function tests seen in pancreatic cancer

Answer 56

true

Question 57

Trousseau sign

Answer 57

``` pancreatic cancer migratory thrombophlebitis (Trousseau sign) is more common than with other cancers ```

Question 58

Pancreatic cancer ix

Answer 58

ultrasound has a sensitivity of around 60-90% high-resolution CT scanning is the investigation of choice if the diagnosis is suspected imaging may demonstrate the 'double duct' sign - the presence of simultaneous dilatation of the common bile and pancreatic ducts

Question 59

Pancreatic cancer mx

Answer 59

less than 20% are suitable for surgery at diagnosis a Whipple's resection (pancreaticoduodenectomy) is performed for resectable lesions in the head of pancreas. Side-effects of a Whipple's include dumping syndrome and peptic ulcer disease adjuvant chemotherapy is usually given following surgery ERCP with stenting is often used for palliation