B5-025 Liver Disorders I Flashcards

1
Q

the dual blood supply of the liver comes from

2

A
  • portal vein
  • hepatic vein
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2
Q

macrophages that live in the sinusoids of the liver

A

Kupffer cells

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3
Q

lined with simple cuboidal epithelium

“string of pearls”

A

bile duct

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4
Q
A
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5
Q

blood in zone one is […] in oxygen

A

rich

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6
Q

blood in zone 3 is […] in oxygen

A

low

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7
Q

there is a more dense distribution of cytochrome p450 enzymes in zone […]

A

3

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8
Q

large cells with centrally located nucleus and prominate nucleolus

A

hepatocytes

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9
Q

hepatocytes are connected to their neighbor hepatocytes through a

A

bile cannaliculus

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10
Q

space between hepatocytes and sinusoids

A

space of Disse

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11
Q

stellate cells live in the

A

space of Disse

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12
Q

what do stellate cells do in their quiescient state?

A

store fat-soluble vitamins

ADEK

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13
Q

in a normal adult state, the cell plates should be […] cells thick

how many cells

A

1
2 cells max (regenerative state)

3+ indicates neoplastic process

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14
Q

feathery degeneration indicates an accumulation of

A

bile

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15
Q

steatosis can be due to

3

A

obesity, alcohol, diabetes

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16
Q

accumulation of iron in a canalicular distribution

A

hemochromatosis

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17
Q

accumulation of copper

Cu not excreted in bile

A

Wilson’s disease

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18
Q

accumulation of misfolded protein in the RER

A

a1 antitrypsin deficiency

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19
Q

old age/”wear and tear” pigment seen in hepatocytes

A

lipofuscin

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20
Q
  • clumped cytokeratin filaments
  • ballooned degeration
A

Mallory hylanine

steatohepatitis, alcohol use

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21
Q
  • hyperpigmentation of the skin
  • jaundice
  • diabetes
A

genetic hemochromatosis

“bronze diabetes”

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22
Q

what lab values would you expect to be elevated in hemochromatosis?

4

A
  • AST/ALT
  • serum iron
  • transferrin
  • serum ferritin
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23
Q

treatment for hereditary hemochromatosis

A

weekly phlebotomy and deferoxamine

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24
Q

inheritance pattern of hereditary hemochromatosis

bonus points if you name the gene

A

autosomal recessive inheritance of HFE gene

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25
HFE regulates
hepcidin | controls how much iron is absorbed in intestine
26
* caused by inappropriately high intestinal iron absorption * results in excess body iron stores
hereditary hemochromatosis
27
why do most cases of hereditary hemochromatosis present after age 40?
* takes a long time to accumulate * cirrhosis occurs when the body iron exceeds 30-40 g around age 40
28
risk factors for hereditary hemochromatosis
* male gender * hepatitis C * alcohol
29
PAS-diatase resistant globules
a1 antitrypsin deficiency
30
Piz allele
alpha 1 antitrypsin deficiency
31
present with cirrhosis of the liver and early onset empysema, chronic bronchitis, asthma, etc
alpha 1 antitrypsin deficiency
32
all patients with PiZZ genotype accumulate [...] in hepatocytes
alpha 1 antitrypsin deficiency
33
* resting tremor * brownish ring around cornea
wilson's disease
34
what labs would you expect to be abnormal in Wilson's disease?
* decreased ceruloplasmin * elevated urine copper
35
mutation in ATPase leading to defective conjugation of copper in ceruloplasmin
Wilson's disease
36
increased copper absorption in gut but decreased excretion in bile
wilson's disease
37
treatment for Wilson's
penicillamine | copper chelation
38
what kind of stain is used to visualize copper?
rhodadine
39
apoptosis is commonly seen in | "dead reds"
viral hepatitis
40
zonal toxic necrosis is due to
ischemia or toxins
41
causes centrolobular necrosis
acetaminophen overdose
42
midzonal necrosis is caused by | 2
adenovirus acute hepatitis
43
submassive or massive necrosis is due to
toxins viral hepatitis
44
laboratory tests to evaluate biliary function | 4
* total bilirubin * direct bilirubin * alk phos * GGT
45
laboratory tests to determine hepatocyte function | 3
* serum albumin * PTT * serum ammonia
46
what labs would you expect to be elevated in an obstructive liver disease pattern?
alk phos bilirubin GGTP | all elevated
47
what labs would you expect to be abnormal in an acute hepatitis pattern?
elevated AST/ALT bilirubin +/-
48
what labs would you expect to be abnormal in a cirrhosis pattern?
decreased albumin decreased platelets prolonged PTT
49
multinucleation, margination, molding
herpesvirus hepatitis
50
* seen in immunocompromised after liver/renal transplant * microabscesses, "owl's eyes"
CMV hepatitis
51
sinusoidal lymphocytosis ("string of pearls") with portal inflammation
EBV hepatitis
52
one pill once a day for 12 weeks treatment for HCV
harvoni | really expensive
53
this histology with plasma cells is characteristic of
autoimmune hepatitis
54
* ANA, ASMA + * AST, ALT > alk phos * hypergammaglobulinemia * absence of viral hepatitis
autoimmune hepatitis
55
features of metabolic resistance | 5
* DM/insulin resistance * hypertension * dyslipidemia * central obesity * microalbuminemia
56
just fat globules with very minimal risk to progression of cirrhosis
steatosis
57
* fat globules plus inflammation and ballooned hepatocytes * risk of progression to cirrhosis
steatohepatitis
58
cytokines released from Kupffer cells to activate stellate cells | 2
TNF-a TGF-b
59
what kind of cells deposit collagen in the liver?
stellate cells
60
store fat soluble vitamins
stellate cells
61
mallory hyaline =
alcoholic steatohepatitis | for testing purposes
62
cirrhosis
63
elevated AFP indicates
HCC
64
HCC
65
left: HCC right: normal
66
mutations associated with the development of HCC | 3
* b-catenin (activating) * TERT * TP53 (inactivating)
67
round globular inclusions of misfolded proteins in hepatocytes that stain bright pink with PAS-diastase
a1-antitrypsin deficiency
68
PAS stain removes [...] with diastase
glycogen
69
interface and lobular inflammatory activity
viral hepatitis
70
broad fibrous bands and complete parenchymal nodules
cirrhosis
71
cirrhosis causes
portal hypertension
72
portosystemic venous shunts cause | 3
* esophageal varices * periumbilical caput medusa * hemorrhoids
73
the livers inability to degrade estrogen in liver failure causes | 4
* skin spider angioma * palmar erythema * testicular atrophy * gyencomastia
74
increased ammonia levels in liver failure cause | 2
asterixis encephalopathy
75
decreased synthetic function in liver failure results in
low albumin
76
what causes the prolongation of PT in liver failure?
factor IIV degradation
77
how does alcohol cause hepatic steatosis?
excess NADH changes normal hepatic metabolism from catabolism of fat to anabolism of fats results in decreased mitochondrial oxidation of fatty acids and increased triglyceride production
78
lobular disarray with apoptotic hepatocytes and lobular inflammation
acute hepatitis
79
AMA is associated with
primary biliary cholangitis
80
extremely elevated transaminanses are associated with
acute hepatitis
81
ANA is associated with
autoimmune hepatitis
82
p-ANCA is associated with
primary sclerosing cholangitis
83
interface activity and prominent lymphoplasmacytic infiltrate
autoimmune hepatitis
84
more common in females may be asymptomatic or present with fatigue, nausea, pruritis
autoimmune hepatitis
85
associated with circulating anti-nuclear antibodies and high levels of serum immunoglobulins
autoimmune hepatitis
86
autosomal recessive genetic disorder characterized by excessive iron absorption and toxic accumulation of iron
hereditary hemochromatosis
87
diabetes, skin pigmentation, and cardiac failure
hereditary hemochromatosis
88
prussian blue stain binds
iron | hereditary hemochromotosis
89
heriditary hemochromatosis leads to
cirrhosis/HCC