MedEd liver and biliary disease 2 Flashcards
starting at the liver where bile is produced, describe the anatomy of the biliary tree until it enters the duodeum
in the liver there is the right and left hepatic duct
these join to form the common hepatic duct
the cystic duct joins the common hepatic duct to form the common bile duct
the common bile duct joins the common pancreatic duct to form the common heptopancreatic duct which enters the duodenum
where is bile produced?
in the liver
what part of the biliary tree enters the duodenum?
common hepatopancreatic duct
where does the biliary tree enter the small intestine?
at the duodenum
what is the function of bile?
to emulsify fats to aid digestion
what happens if there is lack of bile?
malabsorption of lipids
malabsorption of fat soluble vitamins ie vitamin A, D, E, K
what vitamins are fat soluble and are therefore only absorbed with fats?
a, d, e, k
what does vitamin a deficiency cause?
night blindness
dry skin and tissues
what does vitamin d deficiency cause?
rickets in children
osteomalacia/porosis in adults
what does vitamin e deficiency cause?
impaired reflexes and co ordination, muscle weakness etcq
what does vitamin k deficiency cause?
bleeding (superficial and deep)
what is beri beri disease a deficiency of?
thiamine/ B1
what is couvoiser’s law?
if there is painless jaundice and a palpable gallbladder it is unlikely to be gallstones and you should suspect pancretic cancer
what cancer should you suspect with couvoisiers sign?
pancreatic
where are most pancreatic cancers found?
head of pancreas
what are rf for pancreatic cancer?
age over 60 smoking obesity T2DM chronic pancreatitis
what are signs and symptoms of pancreatic cancer?
FLAWS loss of exocrine function= steatorrhea loss of endocrine function= diabetes painless jaundice (late) trossaeu sign= due to mucin release from cancer coagulation is triggered causing DVT elsewhere hepatomegaly
what is prognosis for pancreatic cancer and why?
poor as they are often diagnosed late only when it has spread to other organs, mainly the liver
what tissue is most pancreatic cancer of?
exocrine tissue
what ix are done for pancreatic cancer?
bloods- ca19-9 marker
GS biopsy via ERCP or EUS
Ix of choice if high indication of suspection- high resolution CT due to high sensitivity, will show the double duct sign
what tumor marker is associated with pancreatic cancer?
ca 19-9
what is gs ix for pancreatic cancer?
biopsy via ERCP or EUS
what ix is ideally done if there is a high suspicion of pancreatic cancer, what do you see and why is it done?
high resolution CT is done as it has high sensitivity
you might see double duct sign
how is pancreatic cancer managed?
if it hasnt spread can do surgery (only 20% of cases) which is stenting with ERCP or whipple’s resection and adjuvant chemo
what is cholangiocainoma?
cancer arising from the bile ducts
what are the 2 types of cholangiocarcinoma?
intrahepatic
extrahepatic
what are rf for cholangiocarcinoma?
old age
smoking
obesity
chronic inflammation of the bile ducts
what are signs and symptoms of cholangiocarcinoma?
virchows node sister mary joesph nodule FLAWS RUQ pain if extrahepatic: painless jaundice, pale stools, dark urine, pruritus
what is sister mary joseph nodule and when would you see it?
it is metastatic deposits at the umbilicus, you would see in advanced abdominal cancers eg cholangiocarcinomas
in what type of cholangiocarcinoma would you get symptoms of biliary obstruction?
extrahepatic
what ix are done for cholangiocarcinoma? what is gold standard?
GS- ERCP with biopsy
may do USS and MRCP
bloods- CEA and CA19-9, LFTs, clotting studies
how is cholangiocarcinoma managed?
scope for surgery is limited as patients often present late
removal of the bile duct if small and loaclised
partial hepatectomy if intrahepatic
whipple’s procedure if distal
adjuvant chemo and radiotherapy to reduce recurrence
what is hepatocellular carcinoma?
primary malignancy of the hepatocytes
what is the most common type of liver cancer?
hepatocellular carcinoma
what are rf for hepatocellular carcinoma?
cirrhosis- can be due to hepatitis, alcohol overuse, NAFLD, PBC, haemochromatosis, AI hep
what are signs and symptoms of hepatocellular carcinoma? when does it usually present?
presents late
signs of chronic liver failure: jaundice, pruritus, hepatosplenomegaly
often presents with acute decompensation eg acute liver failure or hepatic encephalopathy
what tumor marker is associated with hepatocellular carcinoma?
aFP
what tumor marker is associated with cholangiocarcinoma?
CEA
Ca19-9
what ix are done for hepatocellular carcinoma?
aFP tumor marker
USS imaging
how is hepatocellular carcinoma mananged?
surgical if early stage: resection if non cirrhotic and liver transplant if cirrhotic
non operable: ethanol injections for peripheral lesions, sorafenib (multikinase inhibitor)
how is non operable hepatocellular carcinoma managed?
ethanol injections for peripheral lesions
sorafenib- multikinase inhibitor
what is primary biliary cirrhosis?
an autoimmune condition where there is granuloma deposition in the bile ducts leading to inflammation and destruction of the bile ducts
what are rf for primary biliary cirrhosis?
middle aged females
pmhx of other AI conditions
what are signs and symptoms of primary biliary cirrhosis?
pruritus cholestatic jaundice RUQ pain xanthelasma clubbing hepatosplenomegaly
what ix are done for primary biliary cirrhosis and what is seen?
bloods- AMA M2 antibodies, SMA, raised serum IgM
how is primary biliary cirrhosis managed?
liver transplant if bilirubin is over 100
cholestyramine for pruritus
vitamin A,D,E,K supplements
ursodeoxycholic acid
what is primary sclerosing cholangitis?
biliary inflammation of unknown aetiology
inflammation of intra and extra hepatic ducts
who is more likely to get primary sclerosing cholangitis?
male
what are signs and symptoms of primary sclerosing cholangitis?
pruritus
cholestatic jaundice
RUQ pain
fatigue
what ix are done for primary sclerosing cholangitis and what is seen?
bloods- pANCA positive
ERCP/MRCP- beads on a string appearance
biopsy- onion skin fibrosis and obliteration of ducts
how is primary sclerosing cholangitis managed?
screen for tumor markers as they are at higher risk of cholangiocarcinoma
at diagnosis do ca19-9 and USS/MRCP
do ERCP biopsy for any suspicious lesions
do tumor markers every 6 months and imaging every 12 months
patients with what condition are at higher risk of cholangiocarcinoma and have to be screened?
primary sclerosing cholangitis
what is the screening programme for those with primary sclerosing cholangitis?
tumor markers via blood every 6 months
imaging every 12 months
what is wilson’s disease?
an autosomal recessive disorder where there is a build up of copper in the body due to increased absorption and decreased excretion
at what age does wilsons disease usually present?
10-25 yrs
what gene is associated with wilsons disease and what chromosome is it on?
ATP7B
on chromosome 13
what is the inheritance pattern of wilsons disease?
autosomal recessive
what are signs and symptoms of wilson’s disease?
organs involved are brain, liver and eyes
brain: damage to basal ganglia causes parkinsonism, behavioural/pyschic/speech disturbance
liver: hepatitis and cirrhosis
eyes: kaiser fleischer rings
what do kaiser fleischer rings look like?
dark ring encircling the iris
what ix are done for wilson’s disease? what is seen
bloods: raised serum copper, reduced serum caeruloplasmin
urinalysis: increased 24 hr urinary copper excretion
slit lamp examination of the eye
genetic testing
liver biopsy
how is wilsons disease managed?
copper chelation with penicillamine first line
other copper chelation methods inc trientine, hydrochloride, tetrathiomolybdate
what is there a build up of in the body in wilsons disease?
copper
what is haemochromatosis?
a disorder where there is abnormal iron deposition in organs causing oxidative damage
what are the 2 types of haemochromotosis and how do they differ?
primary= inherited disorder due to mutation in HEF gene on chromosome 6 secondary= due to blood transfusion, iron supplementation, disease of erythropoiesis
in what gene is there a mutation in in hereditary haemochromatosis? wat chromosome is it on
C282Y mutation
HFE gene
chromosome 6
who is more likely to get haemochromotosis?
middle aged men
how does haemochromatosis present in women?
later
what are some causes of secondary haemochromatosis?
blood transfusions, iron supplementation, disease of erythropoiesis
what is the triad of symptoms for haemochromatosis?
cirrhosis
bronze pigmentation
diabetes
what are signs and symptoms for haemochromatosis?
cirrhosis diabetes bronze pigmentation lethargy impotence arthralgia
what ix are done for haemochromatosis? what is seen
bloods- raised ferritin, raised transferrin saturation, LFTs raised AST and ALT, FBC normal
genetic testing- mutation in HFE gene
liver biopsy
how is haemochromatosis managed?
depends on the stage:
stage 0= monitor iron labs every 3 yrs
stage 1= monitor iron labs and symptoms every year
stage 2-4= venesection (removal of blood)/ iron chelation therapy
end stage liver disease= liver transplant
general advice= avoid iron and vit C supplements and alcohol
what LFTs are raised in wilsons?
AST and ALT
what is the difference between a cyst and an abscess?
cyst= fluid filled abscess= pus filled
what will a liver cyst/abscess contain?
dead cells, pathogens
it may contain blood
in what patients is liver cyst/abscess more common?
diabetics
what are the 4 types of liver cyst/abscess?
pyogenic cyst
amoebic cyst
hydatid cyst
PCLD
what are signs and symptoms of a liver cyst/abscess?
fever malaise weight loss/anorexia RUQ pain jaundice hepatosplenomegaly ascites hx
what ix are done for liver abscess?
bloods- FBC, CRP, LFTs
stool cultures
aspiration and culture of lesion
imaging eg liver USS, CT or MRI
how is liver abscess/cyst managed?
bacterial- drain and long course abx eg amoxicillin, cipro, metronidazole
amoebic- abx targeting anerobe eg metronidazole
hyatid- sterilise with anti fungals then surgical resection
how do you differentiate primary sclerosing cholagitis and primary biliary cirrhosis based off clinical features
PSC= progressive obstructive jaundice PBC= fatigue and pruritus
how is PBC vs PSC diagnosed?
PBC= AMA antibodies PSC= string of pearls on MRCP and onion skin fibrosis
what condition is primary sclerosing cholangitis associated with?
UC
out of PSC and PBC which increases risk of cholangiocarcinoma?
PSC
what is the difference between PBC and PSC in terms of aetiology?
PBC= granuloma formation PSC= fibrotic destruction of bile ducts
what organisms cause pyogenic liver abscess in kids vs adults?
kids= s aureus adults= e coli
what will US show if there is a pyogenic liver abscess?
fluid filled cavity with hyperechoic walls
what does fluid look like in an amoebic abscess?
odourless and pasty in consistency
what organism commonly causes amoebic liver abscess?
entamoeba histolytica
what symptoms might you get with amoebic liver abscess?
amoebic dysentery which gives you profuse bloody diarrhoea
what are hyatid liver cyst/abscess associated with?
tapeworm echinococcus granulosis
sheep rearing
is huge in size
eosinophilia and abnormal LFTs
