B5.025 - Liver Disorders 1

Question 1

Identify the bile duct, hepatic artery and the portal vein

Answer 1

String of pearls - bile duct

hepatic artery - bottom right

Portal vein - big one in the middle

Question 2

What is cholestasis

Answer 2

Impaired bile formation and bile flow

acccumulation of bile pigment in hepatic parenchyma

Question 3

Causes of cholestasis

Answer 3

intrahepatic obstruction

extrahepatic obstruction

defects in bile secretion

Question 4

Causes of obstruction or obliteration of the bile ducts

Answer 4

Cholelithiasis

cholangitis

PSC

post surgical strictures

Question 5

Causes of compression of the bile ducts outside the liver

Answer 5

pancreatic cancer

pancreatitis

extrahepatic biliary tract cancers

Question 6

Answer 6

Canalicular cholestasis

Question 7

What is a lab study for obstructive liver disease

Answer 7

Alk phos, bilirubin, GGTP

Question 8

Acute hepatitis pattern lab studies

Answer 8

AST, ALT

Question 9

Cirrhosis pattern lab studies

Answer 9

decreased albumin, platelets, prolonged PT

Question 10

What happens histologically in obstruction of bile ducts

Answer 10

Other bile ducts form around the obstructed one from the canals of herring

Question 11

What are canals of herring

Answer 11

they have pluripotent cells that can become other types of liver cells they are found in the ductular cells

Question 12

What are stellate cells

Answer 12

Lead to fibrosis in cirrhotic livers

Question 13

Describe labs for cholestasis

Answer 13

Alkaline phosphatase

present in bile duct epithelium and canalicular membrane of hepatocytes

released due to detergent action of retained bile salts on hepatocyte membranes

GGT

Question 14

Clinical signs and symptoms of cholestasis

Answer 14

Jaundice/icterus

pruritis

skin xanthomas

Question 15

Causes of chronic cholestasis of the liver

Answer 15

PBC

PSC

obstructive cholangitis

Question 16

What is PBC

Answer 16

Chronic bile destructive disease

autoimmune in genetically predisposed people

Question 17

Features of PBC

Answer 17

Aberrant expression of MHC class 2 molecules on bile duct epithelial cells and autoreactive T cells around ducts

AMA is increased

Question 18

Epidemiology of PBC

Answer 18

90% female

Peak incidence 40-50 yo

ealry labs - elevated GGT, alk phos,

Question 19

Histo of PBC

Answer 19

destructive cholantitis

florid duct lesions

disruption of BM, intraepithelial lympsh and plasma cells, cytoplasmic vacuolizationl regenerative hyperplasia, occasional mitosis

granulosus

Question 20

Answer 20

Damaged bile ducts from PBC

Question 21

Answer 21

Florid duct lesion

Question 22

Answer 22

T cells infilatrating the bile duct

note the longitudinal x section of the hepatic artery to the right

PBC

Question 23

Treatment for PBC

Answer 23

Oral UDCA

slows progression

mech uncertain

Question 24

What is PSC

Answer 24

Involvement of extrahepatic and in most cases intrahepatic biliary tree

Question 25

Epidemiology of PSC

Answer 25

20-40 M\>F 3rd-5th decade Ulcerative colitis p-ANCA

Question 26

Answer 26

P-ANCA

Question 27

Answer 27

C-ANCA

Question 28

Describe PSC linkage to UC

Answer 28

T cells activated in damaged mucosa of patients with UC migrate to the liver and recognize a cross reacting bile duct antigen

Question 29

Serum tests for PSC

Answer 29

chronic choestatic process - increased GGT, alk phos, hallmark - cholangiogram - beaded areas progressive concentric periductal fibrosis “onion skin”

Question 30

PSC histo

Answer 30

Fibrous cholangitis adjecent mixed inflammatory infiltrate lesions are focal, patchy can just have ductopenia without ductal scars

Question 31

Answer 31

Periductal fibrosis in PSC

Question 32

Answer 32

Periductal fibrosis in PSC

Question 33

Clinical sequalae of PSC

Answer 33

Acute bouts of ascending cholangitis persistent elevation of alk phos cholelithiasis annual risk of developing cholangiocarcoma .6-1/5%

Question 34

Large duct obstructions

Answer 34

gallstones malignancy stricture from previous injury

Question 35

Large duct obstructions in children

Answer 35

Biliary atresia CF Choledochal cyts syndromes with insufficient intrahepatic bile ducts

Question 36

Answer 36

Ductular proliferation

Question 37

Answer 37

Ductular proliferation and acute periductal inflammation seen in large obstruction

Question 38

Answer 38

Ductular proliferation seen in obstruction

Question 39

Describe cholestasis of sepsis

Answer 39

direct effect of intrahepatic bacterial infection ischemia related to hypotension caused by sepsis circulating microbial products

Question 40

Answer 40

Cholangitis lenta seen in cholestasis of sepsis

Question 41

Drug induced liver injury drug types

Answer 41

Abx psychotropic anticonvulsants OCPs anti inflammatories

Question 42

Describe histo of acute injury from predominant cholestasis

Answer 42

Centrilobular hepatocytes, formation of canalicular plugs Abx or contraceptive use

Question 43

Describe liver injury from predominantly cytolytic sources

Answer 43

spotty necrosis/apoptosis submassive/massive necrosis acetaminphen, psychotropic, herbal medicines, cocaine, carbon tetrachloride

Question 44

Answer 44

Canalicular cholestasis

Question 45

Answer 45

Cholestatic bile plugs, from acute injury

Question 46

Choledochal cysts

Answer 46

Congenital dilation of common bile duct most present in kids \< 10 yo jaundice and abdominal pain increased risk of stones, stricture, stenosis, obstructive biliary complications, bile duct carcinoma

Question 47

What is fibropolycystic disease

Answer 47

Congenital malformations of biliary tree von meyenburg complexes, small bile duct polycystic liver disease often associated caroli disease congenital hepatic fibrosis

Question 48

Answer 48

Bile duct hemartoma - von mayenburg complex seen in fibropolycystic disease note abnormally formed bile ducts

Question 49

Answer 49

Congenital hepatofibrosis \* note large regions of scarring and fibrosis

Question 50

Risk factors of gallstones - cholesterol and pigmented

Answer 50

Cholesterol - female, fat, forty pigment - chronic hemolytic syndrome biliary infection

Question 51

Organisms associated with pigment stones

Answer 51

E. Coli, ascaris, liver fluke

Question 52

Clinical features of gallstones

Answer 52

majority asymptomatic colicky pain smaller stones cause more pain

Question 53

Risk factors of cholangiocarcinoma

Answer 53

Primary ascending cholangitis congenital fibropolycystic disease previous exposure to thorotrast opisthorchis sinesis (liver fluke)

Question 54

Answer 54

Adenocarcinoma cholangiocarcinoma

Question 55

Answer 55

Cholangiocarcinoma note the glands

Question 56

Answer 56

Cholangiocarcinoma associated with ulcerative colitis - think p ANCA