Liver- Cirrhosis & Neoplasms

Question 1

what mediates (what cytokine) fibrosis in cirhosis

Answer 1

TGF-B from stellate cells which lie beneath the endothelial cells that line the sinusoids

Question 2

what are 4 things that portal HTN leads to?

Answer 2

1. ascitis
2. congestive splenomegaly/hypersplenism
3. portosystemic shunts
4. hepatorenal syndrome

Question 3

what are the 3 classical portosystemic shunts

Answer 3

esophageal varices, hemorrhoids and caput medusae

Question 4

what is hepatorenal syndrome

Answer 4

rapidly developing renal failure secondary to cirrhosis

Question 5

what causes the eventual coma in cirrhosis

Answer 5

increased serum ammonia levels

Question 6

what are some SE that can be seen because the loss of the liver’s ability to metabolize estrogen

Answer 6

gynecomastia
spider angiomata
palmar erythema

Question 7

how do you follow the degree of coagulation factor deficiency caused by hepatic cirrhosis?

Answer 7

follow the PT

Question 8

what is the most common cause of liver disease in the west?

Answer 8

alcohol related liver disease

Question 9

what is the appearance of the liver in alcohol related liver disease

Answer 9

heavy, greasy liver (resolves with abstinence)

Question 10

what metabolite of alcohol mediates the cell damage?

Answer 10

acetaldehyde

Question 11

what are mallory bodies and when are they seen?

Answer 11

they are damaged cytokeratin filaments and they are seen in alcohol related liver disease

Question 12

what liver enzyme is more elevated in alcoholic related liver disease?

Answer 12

both AST and ALT are elevated but AST it more markedly elevated

Question 13

so some one has fatty liver disease and they sayyyy they dont drink but you know that sometimes people hide that shitttt so how do you tell if his fatty liver is due to alcohol or not

Answer 13

NONalcoholic fatty liver disease is a diagnosis of exclusion.. so you gotta have some faith in da patient

but also their ALT will be “more elevated” than their AST (and its opposite in alcoholic related fatty liver)

Question 14

what word..

“ excess body iron leading to deposition in tissues”

Answer 14

hemosiderosis

Question 15

what word..

“ excess body iron leading to deposition in tissues and organ damage”

Answer 15

hemachromatosis

Question 16

what is technically causing the damage in hemachromatosis

Answer 16

formation of free radicals

Question 17

what causes primary hemachromatosis?

Answer 17

due to mutation in HFE gene (usually C282Y)

Question 18

what causes secondary hemachromatosis?

Answer 18

chronic transfusions

Question 19

what is the classic triad of symptoms seen in hemachromatosis

Answer 19

cirrhosis, secondary DM and bronze skin

Question 20

what are some other signs/symptoms that can be see in hemachromatosis (besides the triad)

Answer 20

dilated cardiomyopathy
cardiac arrhythmias
gonadal dysfunction (due to testicular atrophy)

Question 21

what do the labs show in hemachromatosis
ferritin
TIBC
serum iron
% saturation

Answer 21

ferritin- increase
TIBC- decrease
serum iron- increase
% saturation- increase

Question 22

soooo normal aging of the liver will show brown spots (lipofuscin), but you’re telling me that in hemachromatosis there is also brown spots in the liver, so when i get a tricky question how am i suppose to know what the cause is

Answer 22

prussian blue stain distinguishes iron (blue) from lipofuscin

Question 23

you have a recessive defect in ATP7B, what disease do you have? what does this gene encode for?

Answer 23

wilsons disease

gene encodes for an ATP mediated hepatocyte copper transport

Question 24

how does wilsons disease present

Answer 24

presents in childhood with cirrhosis, neurologic manifestations and kayser fleisher rings in cornea

Question 25

what do the labs show in wilsons disease urinary copper serum ceruloplasmin copper on liver biopsy

Answer 25

urinary copper- increase serum ceruloplasmin- decrease copper on liver biopsy-increase

Question 26

what is the treatment for wilsons (im just asking this bc its a pharm tie in and we gotta start thinking about that shiz)

Answer 26

D-penicillamine (chelates copper)

Question 27

what disease | "autoimmune granulomatous destruction of intrahepatic bile ducts"

Answer 27

primary biliary cirhosis

Question 28

what population does primary biliary cirrhosis present in?

Answer 28

woman, average age 40

Question 29

how does primary biliary cirrhosis present?

Answer 29

obstructive jaundice

Question 30

what disease | "inflammation and fibrosis of intrahepatic and extrahepatic bile ducts"

Answer 30

primary sclerosing cholangitis

Question 31

how does primary sclerosing cholangitis appear?

Answer 31

periductal fibrosis with "onion skin" appearance | uninvolved regions are dilated--"beaded appearance"

Question 32

what GI disease is primary sclerosing cholangitis associated with?

Answer 32

ulcerative colitis | YOU BETTER KNOW THIS. I BET $$ ITS GONNA BE A QUESTION

Question 33

what marker is often + in primary sclerosing cholangitis

Answer 33

p-ANCA

Question 34

what cancer are people with primary sclerosing cholangitis at an increased risk for?

Answer 34

cholangiocarcinoma

Question 35

what disease | "fulminant liver failure and encephalpathy in children with viral illness who take aspirin"

Answer 35

reye syndrome

Question 36

how does reye syndrome present

Answer 36

hypoglycemia, elevated liver enzymes, N/V | can progress to coma and death

Question 37

hepatic adenoma is associated with..

Answer 37

oral contraceptives

Question 38

hepatic adenomas are benign but carry some risks, what are these

Answer 38

rupture and intraperitoneal bleeding (esp during prego because the tumors grow in response to estrogen)

Question 39

what are the 3 risk factors for hepatocellular carcinoma

Answer 39

chronic hepatitis (HBV, HCV) cirrhosis aflatoxins from aspergillus

Question 40

what do aflatoxins from aspergillus do

Answer 40

induce p53 mutations

Question 41

what is the serum tumor marker for hepatocellular carcinoma

Answer 41

alpha fetoprotein

Question 42

how does budd chiari syndrome present (a complication of hepatocellular carcinoma)

Answer 42

painful hepatomegaly and ascitis

Question 43

what is the most common cancer in the liver

Answer 43

metastases | most common locations are colon, pancreas, lung and breast