laracrystalo's version from 2015-06-13 15:45


Question Answer
portal triadportal vein, hepatic artery, common bile duct
liver "enzymes"AST, ALT, alk phos, bili
liver "function" testsalbumin, prothrombin time, platelets
unconjugated hyperbilirubinemiasgilbert and criggler najar, hemolytic and physiologic, hepatitis
UGT1A1 mutationGilbert's
what is the Gilbert's mutation?UGT1A1 it is a benign unconjugated hyperbilirubinemia (bili <6) more common in males and is aggravated by stress
how are gilbert's and crigler najjar different?Gilbert's has 10-33% of normal UGTransferase enzyme activity and C-N has no functional activity of the enzyme.
does crigler-najjar II have enzyme activity?yes, markedly reduced, but detectable. More than type I.
how is c-n II treated?phenobarbital to induce UGT enzyme activity
conjugated hyperbilirubinemiasdubin johnson, rotor, PBC, PSC, bile obstructions
MRP2/cMOAT mutationdubin-johnson
dubin-johson syndromemutation of MRP2/cMOAT gene encoding for canalicular transporter that leads to defect in organic anion transport so that conjugated bili cannot get into the bile ductule leading to intermittent jaundice and a BLACK LIVER, scleral icterus, moderately enlarged liver
grossly pigmented (black) liver?dubin-johnson syndrome
what hyperbilirubinemias are associated with intermittent jaundice?dubin-johnson, gilberts
can you rely on alk phos for intrahepatic cholestasis of pregnancy?No. Difficult to interpret bc placenta produces it as well
what has higher AST > ALTalcoholic hepatitis
why is gender a risk factor for ALD?gastric mucosal alcohol dehydrogenase activity is lower in women, making them more vulnerable to greater hepatic exposure to ingested alcohol than men
is alcoholic steatosis irreversible?no--generally considered benign and reversible by itself with the cessation of alcohol
where is alcoholic hepatitis most pronounced in the liver?zone 3. Combo of steatosis, hepatocellular necrosis, and acute inflammation
what are mallory denk bodies?damaged cytokeratin intermediate filaments in hepatocytes that appear like eosinophilic cytoplasmic inclusions. A hallmark of alcoholic hepatitis.
which zone is most affected by virus infections?zone 1
which zone most affected by ischemia?zone 3
highest p450 zone?zone 3
pale stool and dark urine?obstructive jaundice
jaundice in first 24 hours of life?ABO incompatibility
jaundice after first 24 hours of life - 1 wk?physiologic jaundice of newborn
treatment for criggler-najar?phenobarbital--> induces p450 and --> increases liver production of UDP
LFTs in rotor and dubin-johnson?normal
alk phos levels in hepatitis?normal! this is a biliary function enzyme.
councilman bodies?apoptotic hepatocytes in zone I seen in all viral hepatitis
anti-smooth muscle antibody?autoimmune hepatitis
alcoholic hyaline?mallory bodies
increased NADH?alcoholics
drug induced hepatitis causes?halothane, valproate, acetaminophen, isoniazid


Question Answer
splenomegaly?portal vein -- splenic vein
esophageal varices?L gastric -- esophageal vein
internal hemorrhoids?superior rectal vein -- middle and inferior rectal veins
caput medusae?paraumbilical vein -- superior and inferior epigastric veins
spontaneous bacterial peritonitis bug?E. coli
TIPS connection?splenic vein to L renal vein
pre-hepatic HTN causes?schistosomiasis, portal vein thrombosis (normal sized liver)
hepatic causes of portal HTN?cirrhosis (small liver)
post-hepatic portal HTN causes?nutmeg liver bud chiari --obstruction of hepatic vein or IVC OR R-sided CHF (large liver)
what causes the edema?hypoalbuminemia
Nitrogen transported in body asammonia
Nitrogen excreted from body asurea
treatment for hyper ammonia / cirrhotics?low protein diet, lactulose (provides H+ to bind to NH3), neomycin to kill ammonia producing bacteria in the gut


Question Answer
florid duct lesionprimary biliary cirrhosis
AMA +primary biliary cirrhosis
elevated serum IgMprimary biliary cirrhosis
PBC symptoms?abnormal liver biochemistries, pruritis, fatigue
more common in middle aged women?PBC
affects only intrahepatic ductulesPBC
affects both intra and extrahepatic ductulesPSC
more common in early to middle aged menPSC
pANCA associatedPSC
associated with UCPSC
onion skin fibrosisPSC
+ SMAautoimmune hepatitis
string of pearls appearance (beaded)PSC
high risk for cholangiocarcinomaPSC
beading stricturingPSC
what has a 10 times more risk of colon cancer than the normal population?PSC
diagnostic criteria for AiHserum gamma globulin, IgG elevated, ANA +, SMA +, LKM1 +
elevated SMA and ANA?PSC and AiH
which is worse, AiH I or II?AiH II has an 80% likelihood of progressing to cirrhosis in 3 years
ATP7B gene mutationwilson's disease (chromosome 13) encodes for transmembrane transport of copper within hepatocytes
low serum ceruloplasminwilson's disease
what is wilson's disease characterized byATP7B mutation, low serum ceruloplasmin, kayser fleischer rings, elevated urinary copper
kayser fleischer ringswilson's disease
child with parkinsonismswilson's disease
wilson's treatmentchelating agents like D-penicillamine, zinc, low copper diet (avoid mushrooms, nuts, chocolate, dried fruit, liver, shellfish, transplant)
where can copper deposit in wilson's disease?brain, kidneys, cornea
what stain shows the copper deposition?rhodanine stain
frank psychosis as side effectwilson's
hemolytic anemia as side effectwilson's
HFE gene mutationhemochromatosis (on chromosome 6)
C282Y mutationhemochromatosis
mutation in hemochromatosis?C282Y HFE gene mutation causing impairment of iron sensing and absorption leading to excess iron absorbed from GI tract so that iron deposits in liver, heart, pituitary, thyroid, pancreas, joints, gonads
triad of hemochromatosis?cirrhosis, bronze skin, diabetes
bronze diabeteshemochromatosis
women have later onset of this diseasehemochromatosis due to menstruation
how does secondary hemochromatosis occur?multiple transfusions, excessive iron supplementation, S. African beer brewing vats
treatment for hemochromatosisfamily screening, phlebotomize 1-2 times per week to deplete iron stores
what stain is best for hemochromatosis?prussian blue stain
how are hepcidin levels affected in hemochromatosis?low hepcidin production, so more iron is absorbed and stored in macrophages
total copper level in wilsons?low
free copper level in wilsons?high
labs in biliary cirrhosis?high alk phos, high LDL, high HDL, high bile salts
primary symptom of itching?PBC
high alphafetoprotein cancers?yolk sac, endodermal sinus tumor, teratomas, HCC
most common met to liver?colon cancer
where does liver met like to go?lung (hematogenously)


Question Answer
primary liver tumorshepatocellular carcinoma, cholangiocarcinoma, angiosarcoma
200fold increase with Hep BHCC
can be due to anabolic steroid use for many yearsHCC
histo of HCC?pink cyto, large nuclei, no portal tracts
what neoplasm can produce bile?bile-producing HCC! looks green/yellow
adenocarcinoma with desmoplastic reactioncholangiocarcinoma (bile duct carcinoma)
strong association with PSC?cholangiocarcinoma
what is a klatskin tumor?hilar tumor arises at the convergence of the R and L hepatic ducts causing obstruction and jaundice.
angiosarcomamalignant vascular tumor with multiple hemorrhagic nodules. RBC inside and around tumor.


Question Answer
complication of annular pancreas?small bowel obstruction
what is pancreatic head derived from?ventral bud
what is uncinate process derived from?ventral bud
what is main pancreatic duct derived from?ventral bud
what is body derived from?dorsal bud
what is tail derived from?dorsal bud
causes of acute pancreatitis?alcoholic binge, gallstones (lodged in ampulla of vater), hypertriglyceridemia, mumps
labs of pancreatitis?high amylase, high lipase, hypocalcemia
complication of pancreatitis?pseudocyst (surrounded by granulation tissue! rather than epithelium)
painless jaundicepancreatic adenocarcinoma
courvoisier signnontender palpable gallbladder --> pancreatic adenocarcinoma
trousseau signmigratory thrombophlebitis (DVTs in unusual sites)
markers for pancreatic cancerCA 19-9 and CEA
DVT in the arm?think about pancreatic cancer bc that's a strange location