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Year 3 > MedEd liver and biliary disease 2 > Flashcards

MedEd liver and biliary disease 2 Flashcards

1
Q

starting at the liver where bile is produced, describe the anatomy of the biliary tree until it enters the duodeum

A

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

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2
Q

where is bile produced?

A

in the liver

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3
Q

what part of the biliary tree enters the duodenum?

A

common hepatopancreatic duct

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4
Q

where does the biliary tree enter the small intestine?

A

at the duodenum

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5
Q

what is the function of bile?

A

to emulsify fats to aid digestion

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6
Q

what happens if there is lack of bile?

A

malabsorption of lipids

malabsorption of fat soluble vitamins ie vitamin A, D, E, K

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7
Q

what vitamins are fat soluble and are therefore only absorbed with fats?

A

a, d, e, k

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8
Q

what does vitamin a deficiency cause?

A

night blindness

dry skin and tissues

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9
Q

what does vitamin d deficiency cause?

A

rickets in children

osteomalacia/porosis in adults

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10
Q

what does vitamin e deficiency cause?

A

impaired reflexes and co ordination, muscle weakness etcq

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11
Q

what does vitamin k deficiency cause?

A

bleeding (superficial and deep)

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12
Q

what is beri beri disease a deficiency of?

A

thiamine/ B1

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13
Q

what is couvoiser’s law?

A

if there is painless jaundice and a palpable gallbladder it is unlikely to be gallstones and you should suspect pancretic cancer

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14
Q

what cancer should you suspect with couvoisiers sign?

A

pancreatic

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15
Q

where are most pancreatic cancers found?

A

head of pancreas

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16
Q

what are rf for pancreatic cancer?

A 
age over 60
smoking 
obesity
T2DM
chronic pancreatitis
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17
Q

what are signs and symptoms of pancreatic cancer?

A 
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
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18
Q

what is prognosis for pancreatic cancer and why?

A

poor as they are often diagnosed late only when it has spread to other organs, mainly the liver

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19
Q

what tissue is most pancreatic cancer of?

A

exocrine tissue

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20
Q

what ix are done for pancreatic cancer?

A

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

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21
Q

what tumor marker is associated with pancreatic cancer?

A

ca 19-9

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22
Q

what is gs ix for pancreatic cancer?

A

biopsy via ERCP or EUS

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23
Q

what ix is ideally done if there is a high suspicion of pancreatic cancer, what do you see and why is it done?

A

high resolution CT is done as it has high sensitivity

you might see double duct sign

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24
Q

how is pancreatic cancer managed?

A

if it hasnt spread can do surgery (only 20% of cases) which is stenting with ERCP or whipple’s resection and adjuvant chemo

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25
Q

what is cholangiocainoma?

A

cancer arising from the bile ducts

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26
Q

what are the 2 types of cholangiocarcinoma?

A

intrahepatic

extrahepatic

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27
Q

what are rf for cholangiocarcinoma?

A

old age
smoking
obesity
chronic inflammation of the bile ducts

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28
Q

what are signs and symptoms of cholangiocarcinoma?

A 
virchows node
sister mary joesph nodule
FLAWS
RUQ pain
if extrahepatic: painless jaundice, pale stools, dark urine, pruritus
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29
Q

what is sister mary joseph nodule and when would you see it?

A

it is metastatic deposits at the umbilicus, you would see in advanced abdominal cancers eg cholangiocarcinomas

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30
Q

in what type of cholangiocarcinoma would you get symptoms of biliary obstruction?

A

extrahepatic

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31
Q

what ix are done for cholangiocarcinoma? what is gold standard?

A

GS- ERCP with biopsy
may do USS and MRCP
bloods- CEA and CA19-9, LFTs, clotting studies

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32
Q

how is cholangiocarcinoma managed?

A

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

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33
Q

what is hepatocellular carcinoma?

A

primary malignancy of the hepatocytes

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34
Q

what is the most common type of liver cancer?

A

hepatocellular carcinoma

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35
Q

what are rf for hepatocellular carcinoma?

A

cirrhosis- can be due to hepatitis, alcohol overuse, NAFLD, PBC, haemochromatosis, AI hep

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36
Q

what are signs and symptoms of hepatocellular carcinoma? when does it usually present?

A

presents late
signs of chronic liver failure: jaundice, pruritus, hepatosplenomegaly
often presents with acute decompensation eg acute liver failure or hepatic encephalopathy

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37
Q

what tumor marker is associated with hepatocellular carcinoma?

A

aFP

38
Q

what tumor marker is associated with cholangiocarcinoma?

A

CEA

Ca19-9

39
Q

what ix are done for hepatocellular carcinoma?

A

aFP tumor marker

USS imaging

40
Q

how is hepatocellular carcinoma mananged?

A

surgical if early stage: resection if non cirrhotic and liver transplant if cirrhotic
non operable: ethanol injections for peripheral lesions, sorafenib (multikinase inhibitor)

41
Q

how is non operable hepatocellular carcinoma managed?

A

ethanol injections for peripheral lesions

sorafenib- multikinase inhibitor

42
Q

what is primary biliary cirrhosis?

A

an autoimmune condition where there is granuloma deposition in the bile ducts leading to inflammation and destruction of the bile ducts

43
Q

what are rf for primary biliary cirrhosis?

A

middle aged females

pmhx of other AI conditions

44
Q

what are signs and symptoms of primary biliary cirrhosis?

A 
pruritus
cholestatic jaundice
RUQ pain
xanthelasma
clubbing
hepatosplenomegaly
45
Q

what ix are done for primary biliary cirrhosis and what is seen?

A

bloods- AMA M2 antibodies, SMA, raised serum IgM

46
Q

how is primary biliary cirrhosis managed?

A

liver transplant if bilirubin is over 100
cholestyramine for pruritus
vitamin A,D,E,K supplements
ursodeoxycholic acid

47
Q

what is primary sclerosing cholangitis?

A

biliary inflammation of unknown aetiology

inflammation of intra and extra hepatic ducts

48
Q

who is more likely to get primary sclerosing cholangitis?

A

male

49
Q

what are signs and symptoms of primary sclerosing cholangitis?

A

pruritus
cholestatic jaundice
RUQ pain
fatigue

50
Q

what ix are done for primary sclerosing cholangitis and what is seen?

A

bloods- pANCA positive
ERCP/MRCP- beads on a string appearance
biopsy- onion skin fibrosis and obliteration of ducts

51
Q

how is primary sclerosing cholangitis managed?

A

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

52
Q

patients with what condition are at higher risk of cholangiocarcinoma and have to be screened?

A

primary sclerosing cholangitis

53
Q

what is the screening programme for those with primary sclerosing cholangitis?

A

tumor markers via blood every 6 months

imaging every 12 months

54
Q

what is wilson’s disease?

A

an autosomal recessive disorder where there is a build up of copper in the body due to increased absorption and decreased excretion

55
Q

at what age does wilsons disease usually present?

A

10-25 yrs

56
Q

what gene is associated with wilsons disease and what chromosome is it on?

A

ATP7B

on chromosome 13

57
Q

what is the inheritance pattern of wilsons disease?

A

autosomal recessive

58
Q

what are signs and symptoms of wilson’s disease?

A

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

59
Q

what do kaiser fleischer rings look like?

A

dark ring encircling the iris

60
Q

what ix are done for wilson’s disease? what is seen

A

bloods: raised serum copper, reduced serum caeruloplasmin
urinalysis: increased 24 hr urinary copper excretion
slit lamp examination of the eye
genetic testing
liver biopsy

61
Q

how is wilsons disease managed?

A

copper chelation with penicillamine first line

other copper chelation methods inc trientine, hydrochloride, tetrathiomolybdate

62
Q

what is there a build up of in the body in wilsons disease?

A

copper

63
Q

what is haemochromatosis?

A

a disorder where there is abnormal iron deposition in organs causing oxidative damage

64
Q

what are the 2 types of haemochromotosis and how do they differ?

A 
primary= inherited disorder due to mutation in HEF gene on chromosome 6
secondary= due to blood transfusion, iron supplementation, disease of erythropoiesis
65
Q

in what gene is there a mutation in in hereditary haemochromatosis? wat chromosome is it on

A

C282Y mutation
HFE gene
chromosome 6

66
Q

who is more likely to get haemochromotosis?

A

middle aged men

67
Q

how does haemochromatosis present in women?

A

later

68
Q

what are some causes of secondary haemochromatosis?

A

blood transfusions, iron supplementation, disease of erythropoiesis

69
Q

what is the triad of symptoms for haemochromatosis?

A

cirrhosis
bronze pigmentation
diabetes

70
Q

what are signs and symptoms for haemochromatosis?

A 
cirrhosis
diabetes
bronze pigmentation
lethargy
impotence 
arthralgia
71
Q

what ix are done for haemochromatosis? what is seen

A

bloods- raised ferritin, raised transferrin saturation, LFTs raised AST and ALT, FBC normal
genetic testing- mutation in HFE gene
liver biopsy

72
Q

how is haemochromatosis managed?

A

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

73
Q

what LFTs are raised in wilsons?

A

AST and ALT

74
Q

what is the difference between a cyst and an abscess?

A 
cyst= fluid filled
abscess= pus filled
75
Q

what will a liver cyst/abscess contain?

A

dead cells, pathogens

it may contain blood

76
Q

in what patients is liver cyst/abscess more common?

A

diabetics

77
Q

what are the 4 types of liver cyst/abscess?

A

pyogenic cyst
amoebic cyst
hydatid cyst
PCLD

78
Q

what are signs and symptoms of a liver cyst/abscess?

A 
fever
malaise
weight loss/anorexia
RUQ pain
jaundice
hepatosplenomegaly 
ascites 
hx
79
Q

what ix are done for liver abscess?

A

bloods- FBC, CRP, LFTs
stool cultures
aspiration and culture of lesion
imaging eg liver USS, CT or MRI

80
Q

how is liver abscess/cyst managed?

A

bacterial- drain and long course abx eg amoxicillin, cipro, metronidazole
amoebic- abx targeting anerobe eg metronidazole
hyatid- sterilise with anti fungals then surgical resection

81
Q

how do you differentiate primary sclerosing cholagitis and primary biliary cirrhosis based off clinical features

A 
PSC= progressive obstructive jaundice
PBC= fatigue and pruritus
82
Q

how is PBC vs PSC diagnosed?

A 
PBC= AMA antibodies
PSC= string of pearls on MRCP and onion skin fibrosis
83
Q

what condition is primary sclerosing cholangitis associated with?

A

UC

84
Q

out of PSC and PBC which increases risk of cholangiocarcinoma?

A

PSC

85
Q

what is the difference between PBC and PSC in terms of aetiology?

A 
PBC= granuloma formation 
PSC= fibrotic destruction of bile ducts
86
Q

what organisms cause pyogenic liver abscess in kids vs adults?

A 
kids= s aureus
adults= e coli
87
Q

what will US show if there is a pyogenic liver abscess?

A

fluid filled cavity with hyperechoic walls

88
Q

what does fluid look like in an amoebic abscess?

A

odourless and pasty in consistency

89
Q

what organism commonly causes amoebic liver abscess?

A

entamoeba histolytica

90
Q

what symptoms might you get with amoebic liver abscess?

A

amoebic dysentery which gives you profuse bloody diarrhoea

91
Q

what are hyatid liver cyst/abscess associated with?

A

tapeworm echinococcus granulosis
sheep rearing
is huge in size
eosinophilia and abnormal LFTs

Year 3 (104 decks)

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