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GI II Exam 1 > Liver Path VI > Flashcards

Liver Path VI Flashcards

1
Q

wilson disease

A

auto recessive - impaired excretion of cupper

accumulates in liver, brain, eyes

age 5-15yo

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2
Q

kayser fleischer rings

A

wilson disease - copper overload

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3
Q

diagnosis of wilson disease

A

low serum ceruloplasmin - screening
increased urine copper - specific

liver biopsy

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4
Q

tx of wilson disease

A

chelation or transplant for cirrhosis

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5
Q

ATP7B

A

wilson disease

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6
Q

ATP7A

A

mencke

brittle hair - death - no Cu absorption

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7
Q

menke

A

no Cu absorption

  • brittle hair
  • death
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8
Q

steatosis

A

can occur in wilson disease

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9
Q

rhondanine stain

A

copper stain

for wilson diseas

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10
Q

diagnosis of wilson disease

A

kayser-fleischer ring - of eye

copper deposition

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11
Q

basal ganglia lesions

A

wilson disease

globus pallidus

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12
Q

alpha1 antitrypsin deficiency

A

auto recessive

protein accumulate in hepatocyte inducing apoptosis

also - neutrophil elastase - emphysema of lung

worse with smoking

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13
Q

diagnosis of alpha-1 antitrypsin

A

serum protein electrophoresis

low alpha-1 band

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14
Q

PAS stain

A

alpha-1 antitrypsin deficiency

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15
Q

neonatal cholestasis

A

alpha-1 antitrypsin deficiency

biliary atresia

idiopathic

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16
Q

intrahepatic biliary tract disease

A

biliary cirrhosis
sclerosing cholangitis
anomalies of biliary tree

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17
Q

extrahepatic biliary tract disease

A

large duct obstruction - lead to second biliary cirrhosis if prolonged

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18
Q

primary biliary cirrhosis

A

progressive cholestatic liver disease
-destruction of small to medium bile ducts

AMA against PCD_E2

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19
Q

AMA against PCD-E2 in small bile duct of hering canal

A

with primary biliary cirrhosis

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20
Q

primary biliary cirrhosis clinical

A

middle aged women

pruritis, late jaundice

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21
Q

diagnosis of PBC

A

positive AMAs

liver biopsy - non-suppurative granulomatous destruction of medium sized bile ducts - florid duct lesion

22
Q

florid duct lesion

A

medium sized bile ducts show intense chronic inflammation and damage

in PBC

granulomatous reaction

23
Q

AMA test

A

positive in primary biliary cirrhosis

pt serum on normal test tissue

incubated - allowing AMA to bind PDC-E2

washed and immunofluorescent AMA-Ab dropped to tissue

if positive - PBC

24
Q

primary sclerosing cholangitis

A

progressive liver disease with cholestasis

obliterative fibrosis of intra and extrahepatic bile ducts

seen in UC and crohn - majority UC

male age 40yo

cholelithiasis formation - stones

25
Q

diagnosis of PSC

A

beading on imaging

NOT biopsy

  • rarely see the onion skinning fibrotic lesions
  • biopsy for staging
26
Q

beading of bile ducts

A

PSC

27
Q

von meyenburg complex

A

abnormal bile duct proliferation

-benign - but more problem with ciliopathy elsewhere

28
Q

ciliopathy

A

cilia often involved in biochemical signaling
-so defective - can cause problems

ex - polycystic kidney disease

29
Q

caroli syndrome

A

cysts in bile duct and hepatic fibrosis

ciliopathy

30
Q

ADPKD

A

auto dom polycystic kidney disease

hepatic cysts

rupture berry aneurysm - circle of willis

mitral valve prolapse

abdominal or flank pain

31
Q

meckel syndrome

A

auto recessive

perinatal death

dysplasion and malformation of multiple organs

ciliopathy

32
Q

mutated polycystin

A

Wnt an hedgehog malfuction

loss of cell polarity and cystic expansion occurs

33
Q

liver segmentation

A

8 divisions

can be removed individually during surgery

liver will regenerate

34
Q

as occlude portal vein

A

flow in hepatic artery increases

35
Q

space of mall

A

has defining membrane

chemicals - adenosine and NO can control blood flow to the portal tract

control flow in this shunt - to get more O2 to hepatocytes

36
Q

portal vein thrombosis

A

seen with ultrasound

37
Q

fenestrations of sinusoids

A

can get bigger/smaller

38
Q

hypovolemic shock

A

zone 3 has least O2 supply

hepatocytes here die first

first necrotic changes due to hypovolemia

39
Q

shock liver

A

zone 3 necrosis

like acetaminophen toxicity

pink areas - necrosis

40
Q

CHF

A

pressure damage and ischemic damage in zone 3

get necrosis around zone 3

acute passive congestion

41
Q

nutmeg liver

A

acute passive congestion

with centrilobular necrosis

42
Q

impaired intrahepatic flow

A

sickle cell
DIC
tumor

43
Q

hepatic vein outflow obstruction

A
  • budd-chiari - hepatic vein thrombosis

- sinusoid obstructive syndrome

44
Q

infarct of zahn

A

block of intrahepatic radical giving discrete red-blue area

45
Q

budd chiari syndrome

A

obstruction of at least TWO hepati veins or IVC

RUQ pain, ascites, hepatomeglay

imaging - doppler to see lack of flow

prognosis - life threatening if acute

tx - anticoagulant, thrombolytic, shunt

46
Q

tense capsule

A

with budd-chiari syndrome

47
Q

budd chiari

A

zone 3 hemorrhagic necrosis

48
Q

preeclampsia

A

HTN and proteinuria - pathologic edema

maternal endothelial dysfunction

49
Q

eclampsia

A

seizure in women with preeclampsia

50
Q

HELLP

A

hemolysis, elevated liver enzymes, low platelets
-unknown cause

periportal sinusoids accumulate fibrin deposits with hemorrhage to space of disse - necrosis of zone 1**

tx - delivery

51
Q

acute fatty liver of pregnancy

A

rare
microvesicular fatty change

3rd trimester - leading to liver failure

unknown cause

GI II Exam 1 (29 decks)

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