Biliary System

Question 1

define primary biliary cholangitis (PBC)

Answer 1

autoimmune disease associated with progressive destruction of the bile ducts, due to granulomatous inflammation

Question 2

what is PBC associated with?

Answer 2

rheumatoid arthritis

Sjogren’s syndrome

Question 3

presentation of PBC

Answer 3

• fatigue
• itch without rash
• xantheslama and xanthomas

Question 4

diagnosis of PBC

Answer 4

anti-mitochondrial antibodies (AMA)
LFTs (cholestatic)
liver biopsy (non-caseating granulomas)

Question 5

management of PBC

Answer 5

urseodeoxycholic acid

transplant (liver)

Question 6

define primary sclerosing cholangitis (PSC)

Answer 6

autoimmune disease of chronic inflammation of the intra and extra-hepatic bile ducts which leads to bile duct loss

Question 7

what is PSC associated with?

Answer 7

UC

increased risk of cholangiocarcinoma

Question 8

presentation of PSC

Answer 8

asymptomatic

itch, rigors

Question 9

diagnosis of PSC

Answer 9

MRCP/ERCP

LFTS (cholestatic)

Question 10

management of PSC

Answer 10

• bile flow e.g. stent
• monitor for cholangiocarcinoma and colorectal carcinoma
• control of itch

Question 11

define haemochromatosis

Answer 11

storage disease of excess iron in the liver

Question 12

causes of haemochromatosis

Answer 12

primary= genetics
secondary= overload from diet

Question 13

presentation of haemochromatosis

Answer 13

MEALS
myocardial: dilated cardiomyopathy and arrhythmias
Endocrine: diabetes
Arthritis 
Liver: cirrhosis and HCC
Skin: grey

Question 14

diagnosis of haemochromatosis

Answer 14

liver biopsy with Perls stain (blue)

Question 15

management of haemochromatosis

Answer 15

phlebotomy: removal of blood stimulates the bone marrow to make new red blood cells using up iron in storage

Question 16

define Wilson’s disease

Answer 16

this is an autosomal recessive disease that leads to abnormal copper metabolism

Question 17

presentation of Wilson’s disease

Answer 17

kayser-fleischer rings at corneal limbus

Parkinson-like symptoms

Question 18

diagnosis of Wilson’s

Answer 18

low serum caeruloplasmin

Question 19

management of Wilson’s

Answer 19

chelating agents e.g. penicillamine

Question 20

define alpha1 antitrypsin deficiency

Answer 20

autosomal recessive disease which produces a lack of this inhibitor causing emphysema and cirrhosis

Question 21

define cholelithiasis

Answer 21

this is stone-like material formed within the biliary system, usually due to bilirubin and cholesterol excess or a lack of bile salts (Crohn’s)

Question 22

presentation of cholelithiasis

Answer 22

• asymptomatic
• biliary colic (RUQ pain radiates to shoulder)
• itch
• nausea
  obstruction indicated by pale stools, jaundice and abdominal pain

Question 23

risks of cholelithiasis

Answer 23

cholangitis
pancreatitis
gallstone ileus

Question 24

diagnosis of cholelithiasis

Answer 24

ultrasonography
US
MRCP/ERCP
LFTs

Question 25

management of cholelithiasis

Answer 25

lifestyle recurrent symptoms then cholecystectomy - 6 -12 weeks to reduce inflammation of the biliary tree or within 72 hours of inflammation if unfit for surgery then ursodeoxycholic acid

Question 26

define cholecystitis

Answer 26

inflammation of the gall bladder

Question 27

presentation of cholecystitis

Answer 27

obstructive jaundice RUQ pain radiating to shoulder tender abdomen fever, nausea

Question 28

diagnosis of cholecystitis

Answer 28

Murpheys sign (pressure on RUQ pain on inspiration) bloods - neutrophils US gall bladder wall is thickened and pericystic fluid

Question 29

management of cholecystitis

Answer 29

- cholecystectomy | - metronidazole

Question 30

define cholangitis

Answer 30

inflammation of the biliary tree

Question 31

presentation of cholangitis

Answer 31

Charcot's triad: fever, jaundice and abdominal pain | pruritus

Question 32

diagnosis of cholangitis

Answer 32

bloods abdominal CT with contrast MRCP PTC (X-ray with contrast)

Question 33

management of cholangitis

Answer 33

``` ceftriaxone and metronidazole biliary decompression (ERCP with sphincterotomy and stent) ```

Question 34

what is cholangiocarcinoma associated with?

Answer 34

UC | PSC

Question 35

presentation of cholangniocarcinoma

Answer 35

obstructive jaundice weight loss lethargy

Question 36

diagnosis of cholangiocarcinoma

Answer 36

ultrasound LFTs ERCP/MRCP

Question 37

management of cholangiocarcinoma

Answer 37

liver resection (chemo, embolisation, biliary drainage) transplant biliary stent ERCP/PTC (palliation)

Question 38

what causes obstructive jaundice

Answer 38

``` stones cancer in pancreas head PBC PSC cholangiocarcinoma ```

Question 39

presentation of obstructive jaundice

Answer 39

``` jaundice dark stools pale urine itch biliary colic ```

Question 40

diagnosis of obstructive jaundice

Answer 40

``` bloods LFTs ANA or ASMA MRCP/ERCP EUS for pancreatic carcinoma ```

Question 41

what triggers biliary colic?

Answer 41

fat entering the digestive system causes CCK (choleystokinin) secretion from the duodenum which triggers contraction of the gall bladder

Question 42

how long for cholecystectomy once cholecystitis has developed?

Answer 42

6 -12 weeks later to reduce inflammation of the biliary tree or within 72 hours of inflammation

Question 43

antibiotics in cholecystitis if surgery unfit

Answer 43

metronidazole

Question 44

what is post-cholecystectomy syndrome?

Answer 44

changes in bowel flow after gall bladder removal causes diarrhoea, indigestion, RUQ pain, nausea, intolerance to fatty food and flatulence

Question 45

commonest organisms for cholangitis

Answer 45

e. coli klebsiella enterococcus

Question 46

cholangitis- what to use if unsuitable for ERCP

Answer 46

PTC (percutaneous trans-hepatic cholangiography)

Question 47

uses of ERCP

Answer 47

``` visualisation sphincterotomy clearing stones inserting stents balloon dilatation taking biopsies ```

Question 48

complications of ERCP

Answer 48

bleeding cholangitis pancreatitis