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MedEd liver and biliary disease 2 Flashcards

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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
what is cholangiocainoma?
cancer arising from the bile ducts
26
what are the 2 types of cholangiocarcinoma?
intrahepatic | extrahepatic
27
what are rf for cholangiocarcinoma?
old age smoking obesity chronic inflammation of the bile ducts
28
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 ```
29
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
30
in what type of cholangiocarcinoma would you get symptoms of biliary obstruction?
extrahepatic
31
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
32
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
33
what is hepatocellular carcinoma?
primary malignancy of the hepatocytes
34
what is the most common type of liver cancer?
hepatocellular carcinoma
35
what are rf for hepatocellular carcinoma?
cirrhosis- can be due to hepatitis, alcohol overuse, NAFLD, PBC, haemochromatosis, AI hep
36
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
37
what tumor marker is associated with hepatocellular carcinoma?
aFP
38
what tumor marker is associated with cholangiocarcinoma?
CEA | Ca19-9
39
what ix are done for hepatocellular carcinoma?
aFP tumor marker | USS imaging
40
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)
41
how is non operable hepatocellular carcinoma managed?
ethanol injections for peripheral lesions | sorafenib- multikinase inhibitor
42
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
43
what are rf for primary biliary cirrhosis?
middle aged females | pmhx of other AI conditions
44
what are signs and symptoms of primary biliary cirrhosis?
``` pruritus cholestatic jaundice RUQ pain xanthelasma clubbing hepatosplenomegaly ```
45
what ix are done for primary biliary cirrhosis and what is seen?
bloods- AMA M2 antibodies, SMA, raised serum IgM
46
how is primary biliary cirrhosis managed?
liver transplant if bilirubin is over 100 cholestyramine for pruritus vitamin A,D,E,K supplements ursodeoxycholic acid
47
what is primary sclerosing cholangitis?
biliary inflammation of unknown aetiology | inflammation of intra and extra hepatic ducts
48
who is more likely to get primary sclerosing cholangitis?
male
49
what are signs and symptoms of primary sclerosing cholangitis?
pruritus cholestatic jaundice RUQ pain fatigue
50
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
51
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
52
patients with what condition are at higher risk of cholangiocarcinoma and have to be screened?
primary sclerosing cholangitis
53
what is the screening programme for those with primary sclerosing cholangitis?
tumor markers via blood every 6 months | imaging every 12 months
54
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
55
at what age does wilsons disease usually present?
10-25 yrs
56
what gene is associated with wilsons disease and what chromosome is it on?
ATP7B | on chromosome 13
57
what is the inheritance pattern of wilsons disease?
autosomal recessive
58
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
59
what do kaiser fleischer rings look like?
dark ring encircling the iris
60
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
61
how is wilsons disease managed?
copper chelation with penicillamine first line | other copper chelation methods inc trientine, hydrochloride, tetrathiomolybdate
62
what is there a build up of in the body in wilsons disease?
copper
63
what is haemochromatosis?
a disorder where there is abnormal iron deposition in organs causing oxidative damage
64
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 ```
65
in what gene is there a mutation in in hereditary haemochromatosis? wat chromosome is it on
C282Y mutation HFE gene chromosome 6
66
who is more likely to get haemochromotosis?
middle aged men
67
how does haemochromatosis present in women?
later
68
what are some causes of secondary haemochromatosis?
blood transfusions, iron supplementation, disease of erythropoiesis
69
what is the triad of symptoms for haemochromatosis?
cirrhosis bronze pigmentation diabetes
70
what are signs and symptoms for haemochromatosis?
``` cirrhosis diabetes bronze pigmentation lethargy impotence arthralgia ```
71
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
72
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
73
what LFTs are raised in wilsons?
AST and ALT
74
what is the difference between a cyst and an abscess?
``` cyst= fluid filled abscess= pus filled ```
75
what will a liver cyst/abscess contain?
dead cells, pathogens | it may contain blood
76
in what patients is liver cyst/abscess more common?
diabetics
77
what are the 4 types of liver cyst/abscess?
pyogenic cyst amoebic cyst hydatid cyst PCLD
78
what are signs and symptoms of a liver cyst/abscess?
``` fever malaise weight loss/anorexia RUQ pain jaundice hepatosplenomegaly ascites hx ```
79
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
80
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
81
how do you differentiate primary sclerosing cholagitis and primary biliary cirrhosis based off clinical features
``` PSC= progressive obstructive jaundice PBC= fatigue and pruritus ```
82
how is PBC vs PSC diagnosed?
``` PBC= AMA antibodies PSC= string of pearls on MRCP and onion skin fibrosis ```
83
what condition is primary sclerosing cholangitis associated with?
UC
84
out of PSC and PBC which increases risk of cholangiocarcinoma?
PSC
85
what is the difference between PBC and PSC in terms of aetiology?
``` PBC= granuloma formation PSC= fibrotic destruction of bile ducts ```
86
what organisms cause pyogenic liver abscess in kids vs adults?
``` kids= s aureus adults= e coli ```
87
what will US show if there is a pyogenic liver abscess?
fluid filled cavity with hyperechoic walls
88
what does fluid look like in an amoebic abscess?
odourless and pasty in consistency
89
what organism commonly causes amoebic liver abscess?
entamoeba histolytica
90
what symptoms might you get with amoebic liver abscess?
amoebic dysentery which gives you profuse bloody diarrhoea
91
what are hyatid liver cyst/abscess associated with?
tapeworm echinococcus granulosis sheep rearing is huge in size eosinophilia and abnormal LFTs

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