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LECOM S3 PBL > pathology chapter 18 (pg 852-880) liver and gallbladder > Flashcards

pathology chapter 18 (pg 852-880) liver and gallbladder Flashcards

1
Q

what is bilirubin the end production of

A

heme degradation

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2
Q

what coverts heme to biliverdin

A

intracellular heme oxygenase

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3
Q

what happens to bilirubin formed outside the liver

A

it is released and bound to serum albumin

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4
Q

how are bile salts formed

A

by the conjugation of bile acids with taurine or glycine

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5
Q

what is the major catabolic product of cholesterol

A

bile acids

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6
Q

describe unconjugated bilirubin in water

A

virtually insoluble and exists in tight complexes with serum albumin

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7
Q

what is cholestasis caused by

A

impaired bile formation and bile flow that gives rise to accumulation of bile pigment in the hepatic parenchyma

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8
Q

what is a characteristic lab finding with cholestasis

A

elevated serum alkaline phosphatase and gamma-glutamyl transpeptidase

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9
Q

what is morphologically common to both obstruction and non obstructive cholestasis

A

accumulation of bile pigment within the hepatic parenchyma

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10
Q

what is the most common cause of bile duct obstruction in adults

A

extra hepatic cholelithiasis (gallstones)

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11
Q

what is the most severe form of cholangitis

A

suppurative cholangitis

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12
Q

what is the histological hallmark of ascending cholangitis

A

influx of periductular neutrophils directly into the bile duct epithelium and lumen

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13
Q

where are bile plugs predominant with canalicular cholestasis

A

centrilobular canaliculi

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14
Q

what does hepatolithiasis lead to repeated bouts of

A

ascending cholangitis, progressive inflammatory destruction of hepatic parenchyma, and predisposes to biliary neoplasia

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15
Q

what kind of stones does hepatolithiasis have in distended intrahepatic bile ducts

A

pigmented calcium bilirubina stones

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16
Q

what are the morphological features of neonatal hepatitis

A

lobular disarray with focal liver cell apoptosis and necrosis

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17
Q

what disorder is defined as complete or partial obstruction of the lumen of the extra hepatic biliary tree within the first 3 months of life

A

biliary atresia

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18
Q

what are the salient features of biliary atresia

A

inflammation and fibrosing stricture of the hepatic or common bile ducts

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19
Q

what are the 2 main autoimmune disorders of the intrahepatic bile ducts

A

primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC)

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20
Q

what is primary biliary cirrhosis characterized by

A

nonsuppurative, inflammatory destruction of small and medium-sized intrahepatic bile ducts

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21
Q

who does PBC primarily effect

A

middle aged women with a female to male ratio of 9:1

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22
Q

what is the most characteristic finding for PBC

A

antimitochondrial antibodies

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23
Q

what do the antimitochondrial antibodies involved in PBC recognize

A

the E2 component of the pyruvate dehydrogenase complex

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24
Q

what lab values are elevated with PBC

A

asymptomatic with elevated serum alkaline phosphatase and gamma-glutamyltransferase

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25
Q

when is liver biopsy considered diagnostic for PBC

A

when a florid duct lesion is present

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26
Q

what is primary sclerosing cholangitis (PSC) characterized by

A

inflammation and obliterative fibrosis of intrahepatic and extra hepatic bile ducts with dilation of preserved segments

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27
Q

what is large duct inflammation seen in PSC similar to

A

that seen in ulcerative colitis

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28
Q

what is described as congenital dilations of the common bile duct

A

choledochal cysts

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29
Q

what are the primary abnormalities with fibropolycystic disease

A

congenital malformations of the biliary tree

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30
Q

what are von meyenburg complexes

A

small bile duct hamartomas

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31
Q

what does the thrombosis of intrahepatic portal vein radicals result in

A

sharply demarcated area of red-blue discoloration called infarct of zahn

32
Q

what is the most common cause of small portal vein branch obstruction

A

schistosomiasis

33
Q

what is the most common intrahepatic cause of blood flow obstruction

A

cirrhosis

34
Q

what condition is the obstruction of two or more major hepatic veins that produces liver enlargement, pain and ascites

A

budd-chiari syndrome

35
Q

describe the shape and coloration of the liver in budd-chiari syndrome

A

it is swollen and red-purple and has a tense capsule

36
Q

what does sinusoidal obstruction syndrome arise from

A

toxic injury to the sinusoidal endothelium

37
Q

what is sinusoidal obstruction syndrome characterized by

A

obliteration of the terminal hepatic venues by sub endothelial swelling and collagen deposition

38
Q

what two things act synergistically to cause centrilobular hemorrhagic necrosis

A

combination of hypo perfusion and retrograde congestion

39
Q

what is preeclampsia characterized by

A

maternal hypertension, proteinuria, peripheral edema and coagulation abnormalities

40
Q

what do the periportal sinusoids contain with preeclampsia

A

fibrin deposits associated with hemorrhage into the space of disse, leading to periportal hepatocellular coagulative necrosis

41
Q

what does the diagnosis of acute fatty liver of pregnancy rest on

A

biopsy identification of the characteristic diffuse microvesicular steatosis of hepatocytes

42
Q

what is the primary treatment for acute fatty liver of pregnancy

A

termination of pregnancy

43
Q

how does focal nodular hyperplasia appear morphologically

A

as a well-demarcated but poorly encapsulated nodule

44
Q

describe the lesion color in focal nodular hyperplasia

A

generally lighter than the surrounding liver and is sometimes yellow indicating steatosis

45
Q

nodular regenerative hyperplasia can lead to the development of what

A

portal hypertension

46
Q

what are the most common benign liver tumors

A

cavernous hemangiomas

47
Q

where are cavernous hemangioma generally located

A

directly beneath the capsule

48
Q

describe the morphology of tumors resulting from HNF1-alpha mutations

A

often fatty and devoid of cellular or architectural atypia

49
Q

what immunostain change is diagnostic for beta-catenin mutated hepatocellular adenomas

A

beta catenin usually shows nuclear translocation indicative of its activated state

50
Q

what do most primary liver cancers arise from and what are they termed

A

arise from hepatocytes and termed hepatocellular carcinoma

51
Q

what is the most common liver tumor of early childhood

A

hematoblastoma

52
Q

what pathway is characteristically activated with hepatoblastomas

A

activation of WNT signaling pathway

53
Q

what is the most common setting for emergence of HCC

A

chronic liver diseases

54
Q

what are the most important underlying factors in hepatocarcinogenesis

A

viral infections and toxic injuries

55
Q

what are the most common early mutation events for the emergence of HCC

A

activation of beta-catenin and inactivation of p53

56
Q

describe the morphology of large cell change in HCC

A

show scattered hepatocytes, usually near portal tracts or septa, that are larger than normal hepatocytes and with large, often multiple, often moderately pleomorphic nuclei

57
Q

how does fibrolamellar carcinoma typically present

A

single large, hard scirrhous tumor with fibrous bands coursing through it

58
Q

define cholangiocarcinoma

A

malignancy of the biliary tree, arising from bile ducts within and outside of the liver

59
Q

what form of cholangiocarcinoma is klatskin tumors associated with

A

extra hepatic forms

60
Q

where are klatskin tumors located

A

at the junction of the right and left hepatic ducts

61
Q

how do most extra hepatic cholangiocarcinomas present

A

firm, gray nodules within the bile duct wall

62
Q

what type of tumors are cholangiocarcinomas usually

A

adenocarcinomas

63
Q

what are most biliary tract diseases attributable to

A

cholelithiasis

64
Q

what are pigmented gallstones complex mixtures of

A

insoluble calcio salts of unconjugated bilirubin along with inorganic calcium salts

65
Q

where do cholesterol stones exclusively arise

A

gallbladder

66
Q

describe pure cholesterol stones

A

pale yellow, round to ovoid, and have a finely granular, hard external surface

67
Q

what happens to the color of cholesterol stones when the proportion of calcium carbonate, phosphates and bilirubin increase

A

the stones take on a gray-white to black color and may be lamellated

68
Q

what is the primary complication of gallstones

A

acute calculous cholecystitis

69
Q

what does acute calculous cholecystitis result from

A

chemical irritation and inflammation of a gallbladder obstructed by stones

70
Q

what is acute calculus cholecystitis without stone involvement thought to result from

A

ischemia

71
Q

where is the obstructed stone usually present in calculous cholecystitis

A

ni the neck of the gallbladder or the cystic duct

72
Q

what is chronic cholecystitis usually associated with

A

cholelithiasis

73
Q

what is the most common malignancy of the extra hepatic biliary tract

A

carcinoma

74
Q

what is the most important risk factor for gallbladder cancer

A

gallstones

75
Q

what are the 2 patterns of growth that carcinomas of the gallbladder show

A

infiltrating and exophytic

76
Q

how does the exophytic pattern of gallbladder carcinomas grow

A

into the lumen as an irregular, cauliflower mass

77
Q

what type of carcinomas are most carcinomas of the gallbladder

A

adenocarcinomas

LECOM S3 PBL (10 decks)

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