B5.025 - Liver Disorders 1 Flashcards

1
Q

Identify the bile duct, hepatic artery and the portal vein

A

String of pearls - bile duct

hepatic artery - bottom right

Portal vein - big one in the middle

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2
Q

What is cholestasis

A

Impaired bile formation and bile flow

acccumulation of bile pigment in hepatic parenchyma

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3
Q

Causes of cholestasis

A

intrahepatic obstruction

extrahepatic obstruction

defects in bile secretion

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4
Q

Causes of obstruction or obliteration of the bile ducts

A

Cholelithiasis

cholangitis

PSC

post surgical strictures

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5
Q

Causes of compression of the bile ducts outside the liver

A

pancreatic cancer

pancreatitis

extrahepatic biliary tract cancers

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6
Q
A

Canalicular cholestasis

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7
Q

What is a lab study for obstructive liver disease

A

Alk phos, bilirubin, GGTP

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8
Q

Acute hepatitis pattern lab studies

A

AST, ALT

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9
Q

Cirrhosis pattern lab studies

A

decreased albumin, platelets, prolonged PT

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10
Q

What happens histologically in obstruction of bile ducts

A

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

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11
Q

What are canals of herring

A

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

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12
Q

What are stellate cells

A

Lead to fibrosis in cirrhotic livers

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13
Q

Describe labs for cholestasis

A

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

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14
Q

Clinical signs and symptoms of cholestasis

A

Jaundice/icterus

pruritis

skin xanthomas

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15
Q

Causes of chronic cholestasis of the liver

A

PBC

PSC

obstructive cholangitis

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16
Q

What is PBC

A

Chronic bile destructive disease

autoimmune in genetically predisposed people

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17
Q

Features of PBC

A

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

AMA is increased

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18
Q

Epidemiology of PBC

A

90% female

Peak incidence 40-50 yo

ealry labs - elevated GGT, alk phos,

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19
Q

Histo of PBC

A

destructive cholantitis

florid duct lesions

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

granulosus

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20
Q
A

Damaged bile ducts from PBC

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21
Q
A

Florid duct lesion

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22
Q
A

T cells infilatrating the bile duct

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

PBC

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23
Q

Treatment for PBC

A

Oral UDCA

slows progression

mech uncertain

24
Q

What is PSC

A

Involvement of extrahepatic and in most cases intrahepatic biliary tree

25
Q

Epidemiology of PSC

A

20-40

M>F

3rd-5th decade

Ulcerative colitis

p-ANCA

26
Q
A

P-ANCA

27
Q
A

C-ANCA

28
Q

Describe PSC linkage to UC

A

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

29
Q

Serum tests for PSC

A

chronic choestatic process - increased GGT, alk phos,

hallmark - cholangiogram - beaded areas

progressive concentric periductal fibrosis “onion skin”

30
Q

PSC histo

A

Fibrous cholangitis

adjecent mixed inflammatory infiltrate

lesions are focal, patchy

can just have ductopenia without ductal scars

31
Q
A

Periductal fibrosis in PSC

32
Q
A

Periductal fibrosis in PSC

33
Q

Clinical sequalae of PSC

A

Acute bouts of ascending cholangitis

persistent elevation of alk phos

cholelithiasis

annual risk of developing cholangiocarcoma .6-1/5%

34
Q

Large duct obstructions

A

gallstones

malignancy

stricture from previous injury

35
Q

Large duct obstructions in children

A

Biliary atresia

CF

Choledochal cyts

syndromes with insufficient intrahepatic bile ducts

36
Q
A

Ductular proliferation

37
Q
A

Ductular proliferation and acute periductal inflammation seen in large obstruction

38
Q
A

Ductular proliferation seen in obstruction

39
Q

Describe cholestasis of sepsis

A

direct effect of intrahepatic bacterial infection

ischemia related to hypotension caused by sepsis

circulating microbial products

40
Q
A

Cholangitis lenta seen in cholestasis of sepsis

41
Q

Drug induced liver injury drug types

A

Abx

psychotropic

anticonvulsants

OCPs

anti inflammatories

42
Q

Describe histo of acute injury from predominant cholestasis

A

Centrilobular hepatocytes, formation of canalicular plugs

Abx or contraceptive use

43
Q

Describe liver injury from predominantly cytolytic sources

A

spotty necrosis/apoptosis

submassive/massive necrosis

acetaminphen, psychotropic, herbal medicines, cocaine, carbon tetrachloride

44
Q
A

Canalicular cholestasis

45
Q
A

Cholestatic bile plugs, from acute injury

46
Q

Choledochal cysts

A

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

47
Q

What is fibropolycystic disease

A

Congenital malformations of biliary tree

von meyenburg complexes, small bile duct

polycystic liver disease often associated

caroli disease

congenital hepatic fibrosis

48
Q
A

Bile duct hemartoma - von mayenburg complex seen in fibropolycystic disease

note abnormally formed bile ducts

49
Q
A

Congenital hepatofibrosis

* note large regions of scarring and fibrosis

50
Q

Risk factors of gallstones - cholesterol and pigmented

A

Cholesterol - female, fat, forty

pigment - chronic hemolytic syndrome

biliary infection

51
Q

Organisms associated with pigment stones

A

E. Coli, ascaris, liver fluke

52
Q

Clinical features of gallstones

A

majority asymptomatic

colicky pain

smaller stones cause more pain

53
Q

Risk factors of cholangiocarcinoma

A

Primary ascending cholangitis

congenital fibropolycystic disease

previous exposure to thorotrast

opisthorchis sinesis (liver fluke)

54
Q
A

Adenocarcinoma

cholangiocarcinoma

55
Q
A

Cholangiocarcinoma note the glands

56
Q
A

Cholangiocarcinoma

associated with ulcerative colitis - think p ANCA