what mediates (what cytokine) fibrosis in cirhosis
TGF-B from stellate cells which lie beneath the endothelial cells that line the sinusoids
what are 4 things that portal HTN leads to?
- ascitis
- congestive splenomegaly/hypersplenism
- portosystemic shunts
- hepatorenal syndrome
what are the 3 classical portosystemic shunts
esophageal varices, hemorrhoids and caput medusae
what is hepatorenal syndrome
rapidly developing renal failure secondary to cirrhosis
what causes the eventual coma in cirrhosis
increased serum ammonia levels
what are some SE that can be seen because the loss of the liver’s ability to metabolize estrogen
gynecomastia
spider angiomata
palmar erythema
how do you follow the degree of coagulation factor deficiency caused by hepatic cirrhosis?
follow the PT
what is the most common cause of liver disease in the west?
alcohol related liver disease
what is the appearance of the liver in alcohol related liver disease
heavy, greasy liver (resolves with abstinence)
what metabolite of alcohol mediates the cell damage?
acetaldehyde
what are mallory bodies and when are they seen?
they are damaged cytokeratin filaments and they are seen in alcohol related liver disease
what liver enzyme is more elevated in alcoholic related liver disease?
both AST and ALT are elevated but AST it more markedly elevated
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
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)
what word..
“ excess body iron leading to deposition in tissues”
hemosiderosis
what word..
“ excess body iron leading to deposition in tissues and organ damage”
hemachromatosis
what is technically causing the damage in hemachromatosis
formation of free radicals
what causes primary hemachromatosis?
due to mutation in HFE gene (usually C282Y)
what causes secondary hemachromatosis?
chronic transfusions
what is the classic triad of symptoms seen in hemachromatosis
cirrhosis, secondary DM and bronze skin
what are some other signs/symptoms that can be see in hemachromatosis (besides the triad)
dilated cardiomyopathy
cardiac arrhythmias
gonadal dysfunction (due to testicular atrophy)
what do the labs show in hemachromatosis ferritin TIBC serum iron % saturation
ferritin- increase
TIBC- decrease
serum iron- increase
% saturation- increase
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
prussian blue stain distinguishes iron (blue) from lipofuscin
you have a recessive defect in ATP7B, what disease do you have? what does this gene encode for?
wilsons disease
gene encodes for an ATP mediated hepatocyte copper transport
how does wilsons disease present
presents in childhood with cirrhosis, neurologic manifestations and kayser fleisher rings in cornea
what do the labs show in wilsons disease
urinary copper
serum ceruloplasmin
copper on liver biopsy
urinary copper- increase
serum ceruloplasmin- decrease
copper on liver biopsy-increase
what is the treatment for wilsons (im just asking this bc its a pharm tie in and we gotta start thinking about that shiz)
D-penicillamine (chelates copper)
what disease
“autoimmune granulomatous destruction of intrahepatic bile ducts”
primary biliary cirhosis
what population does primary biliary cirrhosis present in?
woman, average age 40
how does primary biliary cirrhosis present?
obstructive jaundice
what disease
“inflammation and fibrosis of intrahepatic and extrahepatic bile ducts”
primary sclerosing cholangitis
how does primary sclerosing cholangitis appear?
periductal fibrosis with “onion skin” appearance
uninvolved regions are dilated–“beaded appearance”
what GI disease is primary sclerosing cholangitis associated with?
ulcerative colitis
YOU BETTER KNOW THIS. I BET $$ ITS GONNA BE A QUESTION
what marker is often + in primary sclerosing cholangitis
p-ANCA
what cancer are people with primary sclerosing cholangitis at an increased risk for?
cholangiocarcinoma
what disease
“fulminant liver failure and encephalpathy in children with viral illness who take aspirin”
reye syndrome
how does reye syndrome present
hypoglycemia, elevated liver enzymes, N/V
can progress to coma and death
hepatic adenoma is associated with..
oral contraceptives
hepatic adenomas are benign but carry some risks, what are these
rupture and intraperitoneal bleeding (esp during prego because the tumors grow in response to estrogen)
what are the 3 risk factors for hepatocellular carcinoma
chronic hepatitis (HBV, HCV)
cirrhosis
aflatoxins from aspergillus
what do aflatoxins from aspergillus do
induce p53 mutations
what is the serum tumor marker for hepatocellular carcinoma
alpha fetoprotein
how does budd chiari syndrome present (a complication of hepatocellular carcinoma)
painful hepatomegaly and ascitis
what is the most common cancer in the liver
metastases
most common locations are colon, pancreas, lung and breast
