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Block 10 Week 4 > Usera > Liver 3 (or 2 cont'd) > Flashcards

Usera > Liver 3 (or 2 cont'd) Flashcards

1
Q

what are the 3 major intrahepatic biliary tract disorders?

A
  1. primary biliary cirrhosis
  2. secondary biliary cirrhosis
  3. primary sclerosing cholangitis
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2
Q

how do you get secondary biliary cirrhosis?

A

uncorrected obstruction of the EXTRAhepatic biliary tree

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3
Q

what are the clinical features of secondary biliary cirrhosis?

A 
pruritis
jaundice
malaise
dark urine
light stools
HSM
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4
Q

what are the 4 lab findings of secondary biliary cirrhosis?

A
  1. conjugated hyperbilirubinemia
  2. inc serum alk phos
  3. inc bile acids
  4. inc cholesterol
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5
Q

what can obstruct the extrahepatic biliary tree & cause secondary biliary cirrhosis (7 things)

A
  1. extrahepatic cholelithiasis
  2. malignancy of biliary tree or head of pancreas
  3. strictures d/t previous surgery
  4. biliary atresia
  5. CF
  6. choledochal cysts
  7. paucity of bile duct syndromes
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6
Q

what happens initially with secondary biliary cirrhosis?

A

cholestasis

it’s reversible if you fix the obstruction

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7
Q

what happens when you get secondary inflammation in secondary biliary cirrhosis?

A

initiates periportal fibrosis > hepatic scarring + nodule formation

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8
Q

what is primary biliary cirrhosis?

A

inflammatory autoimmune dz affecting the INTRAhepatic bile ducts

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9
Q

what is the primary feature of primary biliary cirrhosis?

A

nonsuppurative inflammatory destruction of MEDIUM & SMALL sized intrahepatic bile ducts

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10
Q

what is characteristic of primary biliary cirrhosis & essential for the dx?

A

ANTI-MITOCHONDRIAL ABS!

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11
Q

what are the 4 clinical features of primary biliary cirrhosis?

A
  1. middle aged woman
  2. insidious onset
  3. fatigue
  4. abd discomfort
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12
Q

what is primary sclerosing cholangitis?

A

chronic cholestatic disorder

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13
Q

what 3 things characterize primary sclerosing cholangitis?

A
  1. non-specific inflammation
  2. sclerosing fibrosis
  3. strictures of the LARGE INTRA & EXTRA HEPATIC bile ducts
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14
Q

what might cause primary sclerosing cholangitis, broadly speaking?

A

immunologically-mediated injury

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15
Q

what is the most important association of primary sclerosing cholangitis?

A

ulcerative colitis

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16
Q

what are the top 5 immunologically-mediated injuries that can cause primary sclerosing cholangitis?

A
  1. ULCERATIVE COLITIS
  2. anti-smooth muscle ab
  3. ANA
  4. rheumatoid factor
  5. atypical P-ANCA
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17
Q

what are the clinical features of primary sclerosing cholangitis?

A 
fatigue
pruritis
jaundice
INC alk phos
chronic pancreatitis
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18
Q

what can primary sclerosing cholangitis predispose you to?

A

cholangiocarcinoma

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19
Q

what happens to contrast media when you do radiography in a pt w/ primary sclerosing cholangitis?

A

it beads

in the intra & extra hepatic biliary tree

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20
Q

what can you see on histology of primary sclerosing cholangitis?

A

onion-skin sclerosing pattern surrounding large intra (or extra) hepatic bile ducts

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21
Q

what are the 4 main anomalies of the biliary tree?

A
  1. von meyenberg complexes
  2. polycystic liver dz
  3. congenital hepatic fibrosis/caroli dz
  4. alagille syndrome
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22
Q

what are von meyenberg complexes?

A

small clusters of modestly dilated bile ducts embedded in fibrous stroma

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23
Q

what is another name for von meyenberg complexes?

A

bile duct hamartomas

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24
Q

T/F: von meyenberg complexes are rare and dangerous

A

FALSE
common!
clinically insignificant!

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25
Q

what are von meyenberg complexes a/w?

A

PCKD

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26
Q

what is polycystic liver dz?

A

multiple diffuse cysts in the liver

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27
Q

what is polycystic liver dz a/w?

A

PCKD

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28
Q

why does congenital hepatic fibrosis a/w caroli’s dz arise?

A

d/t persistance of the embryonic form of the biliary tree

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29
Q

what happens to the portal tracts in congenital hepatic fibrosis a/w caroli’s dz?

A

fibrosis

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30
Q

do you get cirrhosis w/ congenital hepatic fibrosis a/w caroli’s dz?

A

NOPE

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31
Q

what does congenital hepatic fibrosis a/w caroli’s dz increase your risk for?

A

cholangiocarcinoma

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32
Q

what is congenital hepatic fibrosis a/w caroli’s dz a/w?

A

PCKD

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33
Q

what is caroli dz?

A

segmental dilation of the larger ducts of the intrahepatic biliary tree

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34
Q

what is caroli dz a/w?

A

congenital hepatic fibrosis
&
PCKD

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35
Q

what does caroli dz inc your risk for?

A

cholangiocarcinoma

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36
Q

what is alagille syndrome?

A

congenital absence/dearth of bile ducts

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37
Q

how do you inherit alagille syndrome?

A

autosomal dominant

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38
Q

what are the 5 major clinical features of alagille syndrome?

A
  1. chronic cholestasis
  2. peripheral stenosis of pulmonary artery
  3. butterfly-like vertebral arch defects
  4. eye defects
  5. peculiar hypertelic facies
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39
Q

T/F: focal nodular hyperplasia is a neoplasm

A

FALSE

“not a true neoplasm”

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40
Q

what are the 2 major types of hepatic “neoplasms”?

A
  1. focal nodular hyperplasia

2. nodular regenerative hyperplasia

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41
Q

what is focal nodular hyperplasia?

A

pseudo-mass lesion in an otherwise normal liver

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42
Q

what does focal nodular hyperplasia look like grossly?

A

central stellate scar w/ radiations of fibrous septae

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43
Q

what does focal nodular hyperplasia result from?

A

congenital vascular malformation

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44
Q

what is the most common benign liver tumor?

A

cavernous hemangioma

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45
Q

what is a cavernous hemangioma?

A

tumor of vascular channels in a bed of fibrous connective tissue

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46
Q

what can you confuse w/ a cavernous hemangioma?

A

metastatic tumors

but don’t confuse them!

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47
Q

what should you NOT perform on a cavernous hemangioma?

A

blind percutaneous biopsy!

48
Q

what is a hepatic adenoma?

A

a benign tumor arising from hepatocytes

49
Q

what is another name for hepatic adenoma?

A

liver cell adenoma

50
Q

what is hepatic adenoma strongly a/w?

A

ORAL CONTRACEPTIVES
&
ANABOLIC STEROIDS

51
Q

what bad thing can happen w/ hepatic adenoma?

A

subcapsular adenomas can RUPTURE > severe intra-abd hemorrhage

52
Q

what is a hepatoblastoma?

A

MALIGNANT tumor of young children

53
Q

is hepatoblastoma fatal?

A

YES, usu w/i a few years

54
Q

what are the 2 types of hepatoblastoma?

A
  1. epithelial type

2. mixed epithelial & mesenchymal type

55
Q

what does an epithelial type hepatoblastoma recapitulate?

A

the developing liver

56
Q

what does a mixed type hepatoblastoma show?

A

areas of primitive mesenchyme

57
Q

what is hepatoblastoma a/w?

A

familial adenomatous polyposis syndrome
OR
beckwith-wiedmann syndrome

58
Q

what hepatic neoplasm accounts for >90% of primary liver cancers?

A

hepatocellular carcinoma

59
Q

what are the 4 important etiologic factors for hepatocellular carcinoma?

A
  1. chronic viral infxn
  2. chronic alcoholism
  3. non-alcoholic steatohepatitis
  4. food contaminants (aflatoxins)
60
Q

what are the 3 morphological types of hepatocellular carcinoma?

A
  1. uninodular
  2. multinodular
  3. diffusely infiltrative
61
Q

what does hepatocellular carcinoma have a strong propensity for?

A

invasion of vascular structures

62
Q

what vascular structures does hepatocellular carcinoma like to invade?

A

portal vein & IVC

63
Q

what are satellite nodules?

A

intrahepatic metastases of hepatocellular carcinoma

64
Q

what type of variant are 5% of hepatocellular carcinomas?

A

fibrolamellar variant

65
Q

who gets fibrolamellar variant HCC?

A

young M & F w/o underlying chronic liver dz or cirrhosis

66
Q

what lab value increases in fibrolamellar variant HCC?

A

serum AFP

67
Q

what is cholangiocarcinoma?

A

malignancy of the biliary tree arising from bile ducts w/i & outside the liver

68
Q

what are the 4 risk factors for cholangiocarcinoma?

A
  1. primary sclerosing cholangitis
  2. congenital fibropolycystic dz
  3. HCV infxn
  4. thorotrast
69
Q

what is the distribution of cholangiocarcinomas?

A

60% perihilar
20-30% distal
10% intrahepatic

70
Q

what are Klatskin tumors?

A

extrahepatic perihilar tumors near the formation of the hepatic duct
(cholangiocarcinoma)

71
Q

what is notable about cholangiocarcinoma on histo?

A

“brisk desmoplastic response”

72
Q

what is the most common sarcoma arising in the liver?

A

angiosarcoma

73
Q

what is the behavior of angiosarcoma?

A

VERY AGGRESSIVE
malignant
widespread mets

74
Q

what 3 things can cause angiosarcoma?

A
  1. thorotrast
  2. vinyl chloride
  3. arsenic exposure
75
Q

which is more common: metastatic tumors or primary hepatic neoplasia?

A

metastatic tumors

76
Q

what are the 4 most common sites for metastasis of liver cancer?

A
  1. colon
  2. breast
  3. lung
  4. pancreas
77
Q

what are the 3 major groupings of circulatory disorders involving the liver?

A
  1. impaired blood into the liver
  2. impaired blood flow thru the liver
  3. hepatic venous outflow obstruction
    (in, thru, out)
78
Q

what 2 things can cause impaired blood INFLOW to the liver?

A
  1. hepatic artery compromise

2. portal vein obstruction & thrombosis

79
Q

what causes hepatic artery compromise?

A

embolism
neoplasia
polyarteritis nodosa
sepsis

80
Q

what can hepatic artery compromise result in?

A

liver infarct

not often though, bc the liver has a dual blood supply

81
Q

what are the sx of portal vein obstruction & thrombosis?

A

abd pain
portal HTN
ascites

82
Q

what 7 things can cause EXTRAhepatic obstruction?

A
  1. subclinical occlusion from neonatal sepsis or umbilical vein cath
  2. intrabd sepsis
  3. hypercoagulable disorders
  4. trauma
  5. pancreatitis or pancreatic cancer
  6. invasion by HCC
  7. cirrhosis
83
Q

what can cause INTRAhepatic obstruction?

A

acute thrombosis

84
Q

what 7 things can cause impaired blood flow THROUGH the liver?

A
  1. R & L sided HEART FAILURE
  2. cirrhosis
  3. DIC
  4. mets
  5. eclampsia
  6. peliosis hepatis
  7. sickle cell
85
Q

what is budd-chiari syndrome?

A

thrombosis of 2 or more hepatic veins

86
Q

what does budd-chiari syndrome produce?

A

liver enlargement
pain
ascites

87
Q

what are predisposing factors for budd-chiari syndrome that 3/4 of pts have?

A
  1. hypercoagulable state
  2. polycythemia vera
  3. factor V leiden mutation
  4. contraceptive use
  5. pregnancy
88
Q

what is the cause of acute fatty liver of pregnancy?

A

mitochondrial dysfxn

89
Q

what is the primary treatment for acute fatty liver of pregnancy?

A

terminate the pregnancy

90
Q

what does the range of sx for acute fatty liver of pregnancy include?

A

hepatic dysfxn, failure, coma, death

91
Q

what is intrahepatic cholestasis of pregnancy?

A

altered hormonal state of pregnancy w/ biliary defects in secretion&raquo_space;> cholestasis

92
Q

T/F: acute fatty liver of pregnancy & intrahepatic cholestasis of pregnancy are both malignant

A

false

intrahepatic cholestasis is benign

93
Q

what % of the population is affected by cholelithiasis?

A

10-20%

94
Q

what is responsible for >95% of biliary tract dz?

A

cholelithiasis

95
Q

what are the 2 types of cholelithiasis?

A
  1. cholesterol stones

2. pigment stones

96
Q

what are cholesterol stones composed of?

A

cholesterol

97
Q

what are pigment stones composed of?

A

bilirubin calcium salts

98
Q

what is cholecystitis?

A

inflammation of the gallbladder

can be acute, chronic, or acute superimposed on chronic

99
Q

what are the 2 types of acute cholecystitis?

A

calculous or acalculous

100
Q

what is acute calculous cholecystitis d/t?

A

chemical irritation & inflammation from obstruction of the neck or cystic duct

101
Q

what is acute acalculous cholecystitis d/t?

A

ischemia

102
Q

what is weird about chronic cholecystitis?

A

evolution is obscure

can have calculous or acalculous forms

103
Q

what are the clinical features of acute cholecystitis?

A 
RUQ or epigastric pain
fever
anorexia
tachycardia
N/V
104
Q

what are the clinical features of chronic cholecystitis?

A

recurrent attacks of steady or colicky RUQ or epigastric pain
N/V
fatty food intolerance

105
Q

what are the 6 complications of cholecystitis (ugh sorry)?

A
  1. bacterial superinfection, cholangitis, sepsis
  2. gallbladder perforation + abscess
  3. gallbladder rupture + peritonitis
  4. biliary enteric fistula
  5. porcelain gallbladder
  6. aggravation of preexisting medical illness
106
Q

what is biliary atresia?

A

complete or partial obstruction of the lumen of the extrahepatic biliary tree w/i the first 3 mos of life

107
Q

what characterizes biliary atresia?

A

progressive inflammation & fibrosis of intra & extra hepatic ducts

108
Q

what are the 2 forms of biliary atresia we need to know?

A
  1. fetal

2. perinatal

109
Q

what is fetal biliary atresia?

A

aberrant intrauterine dvlpmnt of the extra hepatic biliary tree

110
Q

what is perinatal biliary atresia?

A

normally-formed biliary tree is destroyed following birth

111
Q

how do infants w/ biliary atresia present?

A

persistent neonatal cholestasis

112
Q

what type are most cases of biliary atresia?

A

type 4

113
Q

what are choledochal cysts?

A

congenital dilations of the common bile duct

114
Q

how do pts w/ choledochal cysts present?

A

jaundice or biliary colic

115
Q

what are choledochal cyst pts predisposed to?

A 
stone formation
stenosis
stricture
pancreatitis
obstructive biliary complications

Block 10 Week 4 (9 decks)

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