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Block 10 Week 4 > Usera > Liver 2 > Flashcards

Usera > Liver 2 Flashcards

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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
what is Reye syndrome a/w?
administration of ASPIRIN
26
what age group should you avoid giving aspirin to for fear of Reye syndrome?
KIDS
27
when would you see alcoholic steatohepatitis
appears acutely after heavy drinking episode
28
what are the lab findings like in alcoholic steatohepatitis?
may range from minimal to fulminant hepatitis
29
what are the 4 sx of alcoholic steatohepatitis?
1. anorexia 2. weight loss 3. upper abd discomfort 4. tender hepatomegaly
30
what is the final & possibly irreversible form of alcoholic liver dz?
alcoholic cirrhosis
31
what % of alcoholics develop cirrhosis?
only 10-15%
32
what can contribute to the development of alcoholic cirrhosis?
gender ethnicity genetics comorbidities
33
what is hepatic steatosis?
micro & macrovesicular infiltration of liver things w/ fat
34
how can you reverse fatty change in hepatic steatosis?
stop DRINKING BOOZE
35
what are the clinical features of hepatic steatosis?
mildly elevated serum bilirubin & alk phos
36
T/F: a common consequence of hepatic steatosis is severe hepatic dysfxn
FALSE | severe hepatic dysfxn is unusual!
37
what are the histo findings of alcoholic steatohepatitis?
1. fat 2. hepatocyte swelling (ballooning degeneration) 3. Mallory bodies 4. lymphocytic & neutrophilic inflammation 5. Perisinusoidal fibrosis
38
how do you get metabolic liver dz?
inherit it or acquire it
39
what is the acquired form of metabolic liver dz?
non-alcoholic fatty liver dz
40
what are the 3 forms of inherited metabolic liver dz?
1. hemochromatosis 2. wilson dz 3. alpha 1 anti-trypsin deficiency
41
what is the most common cause of liver dz in the US?
nonalcoholic fatty liver dz
42
70% of obese pts have at least some form of what?
nonalcoholic fatty liver dz
43
what are the conditions that make up nonalcoholic fatty liver dz?
in pts who do NOT consume much alcohol: 1. hepatic steatosis 2. steatosis w/ minor inflammation 3. non-alcoholic steatohepatitis (NASH)
44
what are the possible clinical findings of nonalcoholic fatty liver dz?
``` can be asymptomatic elevated AST/ALT fatigue malaise R side abd pain ```
45
which condition w/i nonalcoholic fatty liver dz is stable w/o clinical probs?
hepatic steatosis, w/ or w/o inflammation
46
what does NASH do clinically?
hepatocyte injury | progression to cirrhosis
47
what % of cases of NASH progress to cirrhosis?
10-20%
48
what is NASH strongly a/w?
obesity & metabolic syndrome
49
what is the most common cause of cryptogenic cirrhosis?
NASH
50
what is hemochromatosis?
primary & secondary disorder of XS body iron accumulation
51
how do you get primary hemochromatosis?
homozygous recessive
52
what is hemosiderosis?
secondary iron accumulation d/t acquired causes
53
where can you get mutations that can cause hereditary hemochromatosis (4 places)?
1. HFE 2. transferrin receptor 2 3. hepcidin genes 4. HJV gene
54
what 6 things can cause hemosiderosis (this is SO LONG I'M SORRY)
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
what is the normal range of total body iron?
2-6 g
56
how is the intestinal absorption of iron affected in hemochromatosis?
regulation is abnormal
57
how much iron accumulates per year in hemochromatosis?
0.5-1.0g/yr
58
when does hemochromatosis manifest?
after 20 g of iron has accumulated
59
if you have 50 g of iron in your body (which is WAY TOO MUCH), how much of that gets put in your liver?
>30%
60
what are the 3 ways that iron is toxic to tissues?
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
what type of cells can you rescue from iron effects?
cells that are not fatally injured
62
what is hepcidin?
main regulator of iron absorption
63
how does hepcidin work?
lowers plasma iron levels via an efflux channel ferrportin, preventing release of iron from intestinal cells & macrophages
64
what happens if you have a hepcidin deficiency?
iron overload
65
what is HJV?
hemojuvelin
66
what does HJV do?
regulates hepcidin levels
67
what happens if you have a mutation in HJV?
severe juvenile hemochromatosis
68
what 3 things regulate hepcidin levels?
1. HJV 2. transferrin receptor 2 3. HFE
69
what happens if you have a mutation in transferrin receptor 2?
classic adult hemochromatosis
70
what is HFE?
hemochromatosis gene
71
what happens if you have a mutation in HFE?
classic adult hemochromatosis
72
what organs/organ systems are affected by hemochromatosis?
(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
what sex is more often affected by hemochromatosis?
males
74
when do you get sx of hemochromatosis (what age)?
50s-60s
75
what are the sx of hemochromatosis?
``` hepatomegaly (micronodular cirrhosis) abd pain skin pigmentation DM (pancreatic fibrosis) cardiac dysfxn arthritis hypogonadism ```
76
how does neonatal hemochromatosis manifest?
severe liver dz + extrahepatic hemosiderin deposition
77
is neonatal hemochromatosis inherited?
NOPE
78
why do you get liver injury in utero in neonatal hemochromatosis?
possibly d/t maternal alloimmune injury to fetal liver
79
what is wilson dz?
autosomal recessive disorder d/t mutated ATP7B gene
80
what 2 things does wilson dz have?
1. impaired copper excretion into bile | 2. failure to incorporate copper into ceruloplasmin
81
hemochromatosis is to iron as wilson dz is to THIS
copper
82
where does copper accumulate to toxic levels in wilson dz?
1. liver 2. brain 3. eye
83
what are the liver sx of wilson dz?
steatosis hepatitis cirrhosis
84
what are the brain sx of wilson dz?
atrophy of the basal ganglia & putamen
85
what is the eye sx of wilson dz?
kayser-fleischer rings
86
do all 300 of the identified mutations in ATP7B cause wilson dz?
nope
87
how do most pts get wilson dz (what do their genes look like)?
compound heterozygotes w/ diff mutations on each gene
88
what does ATP7B deficiency cause?
1. DEC copper transport into bile 2. DEC copper incorporation into ceruloplasmin 3. DEC ceruloplasmin secretion into blood
89
what are the 2 clinical features of wilson dz?
1. chronic liver dz | 2. neuropsych manifestations
90
what 3 things can you use to dx wilson dz?
1. serum ceruloplasmin 2. hepatic copper content (INC) 3. urinary copper excretion (INC) DO NOT USE SERUM COPPER LEVELS
91
can you use serum copper levels to dx wilson dz?
NOPe | don't do it
92
what is alpha 1 antitrypsin deficiency?
autosomal recessive disorder characterized by decreased inhibition of proteases
93
how does alpha 1 antitrypsin deficiency manifest?
1. pulmonary emphysema | 2. liver dz
94
where is alpha 1 antitrypsin synthesized?
the liver
95
what is alpha 1 antitrypsin?
a serine protease inhibitor
96
how many forms of alpha 1 antitrypsin are there?
at least 75 | it's polymorphic
97
what are the 3 common genotypes of alpha 1 antitrypsin?
PIMM PIZZ PIMZ
98
90% of people have which alpha 1 antitrypsin genotype?
PIMM
99
which genotype of alpha 1 antitrypsin is the clinically significant mutation?
PIZZ
100
how does alpha 1 antitrypsin manifest on histo?
PASD+ intracellular accumulations

Block 10 Week 4 (9 decks)

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