Biliary System Flashcards
define primary biliary cholangitis (PBC)
autoimmune disease associated with progressive destruction of the bile ducts, due to granulomatous inflammation
what is PBC associated with?
rheumatoid arthritis
Sjogren’s syndrome
presentation of PBC
- fatigue
- itch without rash
- xantheslama and xanthomas
diagnosis of PBC
anti-mitochondrial antibodies (AMA) LFTs (cholestatic) liver biopsy (non-caseating granulomas)
management of PBC
urseodeoxycholic acid
transplant (liver)
define primary sclerosing cholangitis (PSC)
autoimmune disease of chronic inflammation of the intra and extra-hepatic bile ducts which leads to bile duct loss
what is PSC associated with?
UC
increased risk of cholangiocarcinoma
presentation of PSC
asymptomatic
itch, rigors
diagnosis of PSC
MRCP/ERCP
LFTS (cholestatic)
management of PSC
- bile flow e.g. stent
- monitor for cholangiocarcinoma and colorectal carcinoma
- control of itch
define haemochromatosis
storage disease of excess iron in the liver
causes of haemochromatosis
primary= genetics secondary= overload from diet
presentation of haemochromatosis
MEALS myocardial: dilated cardiomyopathy and arrhythmias Endocrine: diabetes Arthritis Liver: cirrhosis and HCC Skin: grey
diagnosis of haemochromatosis
liver biopsy with Perls stain (blue)
management of haemochromatosis
phlebotomy: removal of blood stimulates the bone marrow to make new red blood cells using up iron in storage
define Wilson’s disease
this is an autosomal recessive disease that leads to abnormal copper metabolism
presentation of Wilson’s disease
kayser-fleischer rings at corneal limbus
Parkinson-like symptoms
diagnosis of Wilson’s
low serum caeruloplasmin
management of Wilson’s
chelating agents e.g. penicillamine
define alpha1 antitrypsin deficiency
autosomal recessive disease which produces a lack of this inhibitor causing emphysema and cirrhosis
define cholelithiasis
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)
presentation of cholelithiasis
- asymptomatic
- biliary colic (RUQ pain radiates to shoulder)
- itch
- nausea
obstruction indicated by pale stools, jaundice and abdominal pain
risks of cholelithiasis
cholangitis
pancreatitis
gallstone ileus
diagnosis of cholelithiasis
ultrasonography
US
MRCP/ERCP
LFTs
management of cholelithiasis
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
define cholecystitis
inflammation of the gall bladder
presentation of cholecystitis
obstructive jaundice
RUQ pain radiating to shoulder
tender abdomen
fever, nausea
diagnosis of cholecystitis
Murpheys sign (pressure on RUQ pain on inspiration)
bloods - neutrophils
US gall bladder wall is thickened and pericystic fluid
management of cholecystitis
- cholecystectomy
- metronidazole
define cholangitis
inflammation of the biliary tree
presentation of cholangitis
Charcot’s triad: fever, jaundice and abdominal pain
pruritus
diagnosis of cholangitis
bloods
abdominal CT with contrast
MRCP
PTC (X-ray with contrast)
management of cholangitis
ceftriaxone and metronidazole biliary decompression (ERCP with sphincterotomy and stent)
what is cholangiocarcinoma associated with?
UC
PSC
presentation of cholangniocarcinoma
obstructive jaundice
weight loss
lethargy
diagnosis of cholangiocarcinoma
ultrasound
LFTs
ERCP/MRCP
management of cholangiocarcinoma
liver resection (chemo, embolisation, biliary drainage)
transplant
biliary stent ERCP/PTC (palliation)
what causes obstructive jaundice
stones cancer in pancreas head PBC PSC cholangiocarcinoma
presentation of obstructive jaundice
jaundice dark stools pale urine itch biliary colic
diagnosis of obstructive jaundice
bloods LFTs ANA or ASMA MRCP/ERCP EUS for pancreatic carcinoma
what triggers biliary colic?
fat entering the digestive system causes CCK (choleystokinin) secretion from the duodenum which triggers contraction of the gall bladder
how long for cholecystectomy once cholecystitis has developed?
6 -12 weeks later to reduce inflammation of the biliary tree or within 72 hours of inflammation
antibiotics in cholecystitis if surgery unfit
metronidazole
what is post-cholecystectomy syndrome?
changes in bowel flow after gall bladder removal causes diarrhoea, indigestion, RUQ pain, nausea, intolerance to fatty food and flatulence
commonest organisms for cholangitis
e. coli
klebsiella
enterococcus
cholangitis- what to use if unsuitable for ERCP
PTC (percutaneous trans-hepatic cholangiography)
uses of ERCP
visualisation sphincterotomy clearing stones inserting stents balloon dilatation taking biopsies
complications of ERCP
bleeding
cholangitis
pancreatitis
