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Block 10 Week 4 > Usera > Liver 2 > Flashcards

Usera > Liver 2 Flashcards

1
Q

what is autoimmune hepatitis?

A

idiopathic chronic progressive hepatitis

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2
Q

what sex does autoimmune hepatitis have a preponderance for?

A

females

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3
Q

what is defective in autoimmune hepatitis?

A

T-cell regulation

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4
Q

what can trigger autoimmune hepatitis?

A

infxn
acute illness
drugs

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5
Q

what is autoimmune hepatitis a/w?

A

other autoimmune dz (how SHOCKING)

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6
Q

what is the infiltrate like in autoimmune hepatitis?

A

portal plasma cell infiltrate

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7
Q

what 2 things elevate in autoimmune hepatitis?

A

serum IGG & gamma globulin levels

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8
Q

T/F: there are 2 types of autoimmune hepatitis

A

TRUE

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9
Q

what are the 4 types of antibodies that you have in type 1 autoimmune hepatitis?

A
  1. ANA (anti-nuclear ab)
  2. SMA (anti-smooth muscle ab)
  3. AAA (anti-actin ab)
  4. SLA/LP (anti-soluble liver ag/liver-pancreas ag ab)
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10
Q

what is type 1 autoimmune hepatitis a/w?

A

HLA-DR3 serotype

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11
Q

what are the 2 types of antibodies you have in type 2 autoimmune hepatitis?

A
  1. ALKM-1 (anti-liver kidney microsome 1 ab)

2. ACL-1 (anti-liver cytosol-1 ab)

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12
Q

what are anti-liver kidney microsome 1 abs directed against?

A

CYP2D6

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13
Q

10% of adverse drug rxns end in what?

A

toxicity

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14
Q

what is the most common cause of fulminant hepatitis?

A

drug toxicity

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15
Q

what are the 3 mechanisms that can lead to drug toxicity?

A
  1. direct injury
  2. injury d/t toxic metabolites
  3. immunogenic
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16
Q

what is the classic example of a drug that causes toxicity via injury d/t toxic metabolites?

A

acetaminophen
NAPQI is a reactive intermediate
Glutathione neutralizes it, but some remains unbound & just goes to the liver & kidney & causes toxicity

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17
Q

what are the 2 types of drug rxns?

A
  1. predictable (intrinsic)

2. unpredictable (idiosyncratic)

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18
Q

how do you get a predictable/intrinsic drug rxn?

A

these can occur in anyone who receives a sufficient dose of an agent

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19
Q

how do you get an unpredictable/idiosyncratic drug rxn?

A

depends on host idiosyncrasies, like metabolic rate or intensity of immune response

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20
Q

how long does it take for a drug rxn to occur?

A

can be immediate, or it may take weeks or months to dvlp

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21
Q

look at slide 7 cause idk if we need to know all that???

A

yikes

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22
Q

what can acetaminophen toxicity cause on histo?

A

perivenular necrosis

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23
Q

what is Reye syndrome?

A

rare
potentially fatal
syndrome of mitochondrial dysfxn in liver & brain

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24
Q

what characterizes Reye syndrome?

A

extensive microvesicular steatosis

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25
Q

what is Reye syndrome a/w?

A

administration of ASPIRIN

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26
Q

what age group should you avoid giving aspirin to for fear of Reye syndrome?

A

KIDS

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27
Q

when would you see alcoholic steatohepatitis

A

appears acutely after heavy drinking episode

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28
Q

what are the lab findings like in alcoholic steatohepatitis?

A

may range from minimal to fulminant hepatitis

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29
Q

what are the 4 sx of alcoholic steatohepatitis?

A
  1. anorexia
  2. weight loss
  3. upper abd discomfort
  4. tender hepatomegaly
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30
Q

what is the final & possibly irreversible form of alcoholic liver dz?

A

alcoholic cirrhosis

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31
Q

what % of alcoholics develop cirrhosis?

A

only 10-15%

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32
Q

what can contribute to the development of alcoholic cirrhosis?

A

gender
ethnicity
genetics
comorbidities

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33
Q

what is hepatic steatosis?

A

micro & macrovesicular infiltration of liver things w/ fat

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34
Q

how can you reverse fatty change in hepatic steatosis?

A

stop DRINKING BOOZE

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35
Q

what are the clinical features of hepatic steatosis?

A

mildly elevated serum bilirubin & alk phos

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36
Q

T/F: a common consequence of hepatic steatosis is severe hepatic dysfxn

A

FALSE

severe hepatic dysfxn is unusual!

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37
Q

what are the histo findings of alcoholic steatohepatitis?

A
  1. fat
  2. hepatocyte swelling (ballooning degeneration)
  3. Mallory bodies
  4. lymphocytic & neutrophilic inflammation
  5. Perisinusoidal fibrosis
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38
Q

how do you get metabolic liver dz?

A

inherit it or acquire it

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39
Q

what is the acquired form of metabolic liver dz?

A

non-alcoholic fatty liver dz

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40
Q

what are the 3 forms of inherited metabolic liver dz?

A
  1. hemochromatosis
  2. wilson dz
  3. alpha 1 anti-trypsin deficiency
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41
Q

what is the most common cause of liver dz in the US?

A

nonalcoholic fatty liver dz

42
Q

70% of obese pts have at least some form of what?

A

nonalcoholic fatty liver dz

43
Q

what are the conditions that make up nonalcoholic fatty liver dz?

A

in pts who do NOT consume much alcohol:

  1. hepatic steatosis
  2. steatosis w/ minor inflammation
  3. non-alcoholic steatohepatitis (NASH)
44
Q

what are the possible clinical findings of nonalcoholic fatty liver dz?

A 
can be asymptomatic
elevated AST/ALT
fatigue
malaise
R side abd pain
45
Q

which condition w/i nonalcoholic fatty liver dz is stable w/o clinical probs?

A

hepatic steatosis, w/ or w/o inflammation

46
Q

what does NASH do clinically?

A

hepatocyte injury

progression to cirrhosis

47
Q

what % of cases of NASH progress to cirrhosis?

A

10-20%

48
Q

what is NASH strongly a/w?

A

obesity & metabolic syndrome

49
Q

what is the most common cause of cryptogenic cirrhosis?

A

NASH

50
Q

what is hemochromatosis?

A

primary & secondary disorder of XS body iron accumulation

51
Q

how do you get primary hemochromatosis?

A

homozygous recessive

52
Q

what is hemosiderosis?

A

secondary iron accumulation d/t acquired causes

53
Q

where can you get mutations that can cause hereditary hemochromatosis (4 places)?

A
  1. HFE
  2. transferrin receptor 2
  3. hepcidin genes
  4. HJV gene
54
Q

what 6 things can cause hemosiderosis (this is SO LONG I’M SORRY)

A
  1. parenteral iron overload
  2. poor erythropoiesis w/ inc erythroid activity
  3. inc oral iron
  4. congenital atransferrinemia
  5. chronic liver dz
  6. neonatal hemochromatosis
55
Q

what is the normal range of total body iron?

A

2-6 g

56
Q

how is the intestinal absorption of iron affected in hemochromatosis?

A

regulation is abnormal

57
Q

how much iron accumulates per year in hemochromatosis?

A

0.5-1.0g/yr

58
Q

when does hemochromatosis manifest?

A

after 20 g of iron has accumulated

59
Q

if you have 50 g of iron in your body (which is WAY TOO MUCH), how much of that gets put in your liver?

A

> 30%

60
Q

what are the 3 ways that iron is toxic to tissues?

A
  1. iron-catalyzed free radical pdtion > lipid peroxidation
  2. activates hepatic stellate cells > stimulates collagen formation
  3. iron interacts w/ ROS > affects DNA > lethal cell injury
61
Q

what type of cells can you rescue from iron effects?

A

cells that are not fatally injured

62
Q

what is hepcidin?

A

main regulator of iron absorption

63
Q

how does hepcidin work?

A

lowers plasma iron levels via an efflux channel ferrportin, preventing release of iron from intestinal cells & macrophages

64
Q

what happens if you have a hepcidin deficiency?

A

iron overload

65
Q

what is HJV?

A

hemojuvelin

66
Q

what does HJV do?

A

regulates hepcidin levels

67
Q

what happens if you have a mutation in HJV?

A

severe juvenile hemochromatosis

68
Q

what 3 things regulate hepcidin levels?

A
  1. HJV
  2. transferrin receptor 2
  3. HFE
69
Q

what happens if you have a mutation in transferrin receptor 2?

A

classic adult hemochromatosis

70
Q

what is HFE?

A

hemochromatosis gene

71
Q

what happens if you have a mutation in HFE?

A

classic adult hemochromatosis

72
Q

what organs/organ systems are affected by hemochromatosis?

A

(if you have to guess, guess all of them)

  1. liver
  2. pancreas
  3. myocardium
  4. pituitary
  5. adrenal
  6. thyroid
  7. parathyroid
  8. joints
  9. skin
73
Q

what sex is more often affected by hemochromatosis?

A

males

74
Q

when do you get sx of hemochromatosis (what age)?

A

50s-60s

75
Q

what are the sx of hemochromatosis?

A 
hepatomegaly (micronodular cirrhosis)
abd pain
skin pigmentation
DM (pancreatic fibrosis)
cardiac dysfxn
arthritis
hypogonadism
76
Q

how does neonatal hemochromatosis manifest?

A

severe liver dz
+
extrahepatic hemosiderin deposition

77
Q

is neonatal hemochromatosis inherited?

A

NOPE

78
Q

why do you get liver injury in utero in neonatal hemochromatosis?

A

possibly d/t maternal alloimmune injury to fetal liver

79
Q

what is wilson dz?

A

autosomal recessive disorder d/t mutated ATP7B gene

80
Q

what 2 things does wilson dz have?

A
  1. impaired copper excretion into bile

2. failure to incorporate copper into ceruloplasmin

81
Q

hemochromatosis is to iron as wilson dz is to THIS

A

copper

82
Q

where does copper accumulate to toxic levels in wilson dz?

A
  1. liver
  2. brain
  3. eye
83
Q

what are the liver sx of wilson dz?

A

steatosis
hepatitis
cirrhosis

84
Q

what are the brain sx of wilson dz?

A

atrophy of the basal ganglia & putamen

85
Q

what is the eye sx of wilson dz?

A

kayser-fleischer rings

86
Q

do all 300 of the identified mutations in ATP7B cause wilson dz?

A

nope

87
Q

how do most pts get wilson dz (what do their genes look like)?

A

compound heterozygotes w/ diff mutations on each gene

88
Q

what does ATP7B deficiency cause?

A
  1. DEC copper transport into bile
  2. DEC copper incorporation into ceruloplasmin
  3. DEC ceruloplasmin secretion into blood
89
Q

what are the 2 clinical features of wilson dz?

A
  1. chronic liver dz

2. neuropsych manifestations

90
Q

what 3 things can you use to dx wilson dz?

A
  1. serum ceruloplasmin
  2. hepatic copper content (INC)
  3. urinary copper excretion (INC)

DO NOT USE SERUM COPPER LEVELS

91
Q

can you use serum copper levels to dx wilson dz?

A

NOPe

don’t do it

92
Q

what is alpha 1 antitrypsin deficiency?

A

autosomal recessive disorder characterized by decreased inhibition of proteases

93
Q

how does alpha 1 antitrypsin deficiency manifest?

A
  1. pulmonary emphysema

2. liver dz

94
Q

where is alpha 1 antitrypsin synthesized?

A

the liver

95
Q

what is alpha 1 antitrypsin?

A

a serine protease inhibitor

96
Q

how many forms of alpha 1 antitrypsin are there?

A

at least 75

it’s polymorphic

97
Q

what are the 3 common genotypes of alpha 1 antitrypsin?

A

PIMM
PIZZ
PIMZ

98
Q

90% of people have which alpha 1 antitrypsin genotype?

A

PIMM

99
Q

which genotype of alpha 1 antitrypsin is the clinically significant mutation?

A

PIZZ

100
Q

how does alpha 1 antitrypsin manifest on histo?

A

PASD+ intracellular accumulations

Block 10 Week 4 (9 decks)

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