118b Liver-bilary disease Flashcards
extraheptatic duct flow to duodenum
L and R hepatic duct –> common hepatic duct (+ cystic duct) –> common bile duct (+ pancreatic duct) –> ampula of vater
where is bilirubin conjugated? how does it get there in the blood? what conjugates it?
liver
albumin
UDP
crigler-najjar syndrome
1) cause
2) age affected
3) findings
4) treatment
1) absent UDP-glucironyl tranferase in hepatocyte
2) early with early death
3) jaundice, kernicterus (bilirubin deposits in brain), high unconjugated bilirubin
4) plasmapheresis and phototherapy
Gilbert syndrome
1) cause
2) findings
3) consequences
1) TATA region = promotor region defect leads to low UDP-glucironyl tranferase in hepatocyte
2) asymptomatic; elevated UCB
3) no clinical consequences
cholestatis - how and what does it cause?
bile components leak out and lead to
1) bile acids –> pruritus
2) bilirubin and lipids –> jaundice and xanthomas
3) no bile acid into intestine –> malabsorption (steatorrhea, coagulopahty-K, night blindness -A) ADEK vitamins not absorped
4) bile salts + copper are toxic to liver –> liver damage –> cirrhosis + HCC
primary biliary cirrhosis (PBC)
1) sex and age
2) cause/pathology
3) symptoms
4) lab findings
5) genetics?
1) female; 40-50
2) AMA against PDC-E2 autoimmune destruction of intrahepatic biliary ductules (BEC destruction)
CD+4 t-cells involved
3) symptoms = pruritus, jaundice, xanthomas, dark urine, light stools
4) high alkaline phosphatase
high GGT
high Ig - all classes
AMA
5) genetics + xenobiotic modification of PDC-E2
Primary sclerosing cholangitis
1) sex; age
2) cause/pathology and appearance
3) what does it affect
4) symptoms
5) risk
6) lab findings
7) assoicated diseases
1) sex - males more common; onion-skin fibrosis around bile duct
3) extrahepatic and large intreahepatic bile ducts
4) symptoms - fatigue, pain, jaundice, death;
5) cholangiocarcinoma
6) lab findings from cholestasis
p-ANCA
high alkaline phosphatase
ERCP findings - diagnostic b/c visable
7) ulcerative colitis and Crohn’s
PSC association
crohn’s
ulcerative colotis
types of stones
cholestesterol stones - western diet (805)
pigment/non-chol - black or brown pigments (asia/africa)
mixed stones - chol + pigment
bile duct - bilary epithelial cell (BEC) name? intra or extraheptatic?
cholangiocytes - intraheptatic
fisk factors for cholelithiasis
female
fat
fertile (pregnant)
forty+
pigment stones -types
black - hemolytic disease
brown - infection and cholangitis (infection of CBD)
both made up of bilirubin pigment
what can chronic cholecystitis lead to?
adenocarcinoma of gallbladder
what can acute cholecystitis lead to?
infarction, perforation
fistula
mucocele - outpouching of gut wall
cholecystitis
gallbladder inflammation
from gallstones usually
