Liver Path VI

Question 1

wilson disease

Answer 1

auto recessive - impaired excretion of cupper

accumulates in liver, brain, eyes

age 5-15yo

Question 2

kayser fleischer rings

Answer 2

wilson disease - copper overload

Question 3

diagnosis of wilson disease

Answer 3

low serum ceruloplasmin - screening
increased urine copper - specific

liver biopsy

Question 4

tx of wilson disease

Answer 4

chelation or transplant for cirrhosis

Question 5

ATP7B

Answer 5

wilson disease

Question 6

ATP7A

Answer 6

mencke

brittle hair - death - no Cu absorption

Question 7

menke

Answer 7

no Cu absorption

• brittle hair
• death

Question 8

steatosis

Answer 8

can occur in wilson disease

Question 9

rhondanine stain

Answer 9

copper stain

for wilson diseas

Question 10

diagnosis of wilson disease

Answer 10

kayser-fleischer ring - of eye

copper deposition

Question 11

basal ganglia lesions

Answer 11

wilson disease

globus pallidus

Question 12

alpha1 antitrypsin deficiency

Answer 12

auto recessive

protein accumulate in hepatocyte inducing apoptosis

also - neutrophil elastase - emphysema of lung

worse with smoking

Question 13

diagnosis of alpha-1 antitrypsin

Answer 13

serum protein electrophoresis

low alpha-1 band

Question 14

PAS stain

Answer 14

alpha-1 antitrypsin deficiency

Question 15

neonatal cholestasis

Answer 15

alpha-1 antitrypsin deficiency

biliary atresia

idiopathic

Question 16

intrahepatic biliary tract disease

Answer 16

biliary cirrhosis
sclerosing cholangitis
anomalies of biliary tree

Question 17

extrahepatic biliary tract disease

Answer 17

large duct obstruction - lead to second biliary cirrhosis if prolonged

Question 18

primary biliary cirrhosis

Answer 18

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

AMA against PCD_E2

Question 19

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

Answer 19

with primary biliary cirrhosis

Question 20

primary biliary cirrhosis clinical

Answer 20

middle aged women

pruritis, late jaundice

Question 21

diagnosis of PBC

Answer 21

positive AMAs

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

Question 22

florid duct lesion

Answer 22

medium sized bile ducts show intense chronic inflammation and damage

in PBC

granulomatous reaction

Question 23

AMA test

Answer 23

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

Question 24

primary sclerosing cholangitis

Answer 24

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

Question 25

diagnosis of PSC

Answer 25

beading on imaging NOT biopsy - rarely see the onion skinning fibrotic lesions - biopsy for staging

Question 26

beading of bile ducts

Answer 26

PSC

Question 27

von meyenburg complex

Answer 27

abnormal bile duct proliferation | -benign - but more problem with ciliopathy elsewhere

Question 28

ciliopathy

Answer 28

cilia often involved in biochemical signaling -so defective - can cause problems ex - polycystic kidney disease

Question 29

caroli syndrome

Answer 29

cysts in bile duct and hepatic fibrosis ciliopathy

Question 30

ADPKD

Answer 30

auto dom polycystic kidney disease hepatic cysts rupture berry aneurysm - circle of willis mitral valve prolapse abdominal or flank pain

Question 31

meckel syndrome

Answer 31

auto recessive perinatal death dysplasion and malformation of multiple organs ciliopathy

Question 32

mutated polycystin

Answer 32

Wnt an hedgehog malfuction loss of cell polarity and cystic expansion occurs

Question 33

liver segmentation

Answer 33

8 divisions can be removed individually during surgery liver will regenerate

Question 34

as occlude portal vein

Answer 34

flow in hepatic artery increases

Question 35

space of mall

Answer 35

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

Question 36

portal vein thrombosis

Answer 36

seen with ultrasound

Question 37

fenestrations of sinusoids

Answer 37

can get bigger/smaller

Question 38

hypovolemic shock

Answer 38

zone 3 has least O2 supply hepatocytes here die first first necrotic changes due to hypovolemia

Question 39

shock liver

Answer 39

zone 3 necrosis like acetaminophen toxicity pink areas - necrosis

Question 40

 CHF

Answer 40

pressure damage and ischemic damage in zone 3 get necrosis around zone 3 acute passive congestion

Question 41

nutmeg liver

Answer 41

acute passive congestion with centrilobular necrosis

Question 42

impaired intrahepatic flow

Answer 42

sickle cell DIC tumor

Question 43

hepatic vein outflow obstruction

Answer 43

- budd-chiari - hepatic vein thrombosis | - sinusoid obstructive syndrome

Question 44

infarct of zahn

Answer 44

block of intrahepatic radical giving discrete red-blue area

Question 45

budd chiari syndrome

Answer 45

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

Question 46

tense capsule

Answer 46

with budd-chiari syndrome

Question 47

budd chiari

Answer 47

zone 3 hemorrhagic necrosis

Question 48

preeclampsia

Answer 48

HTN and proteinuria - pathologic edema maternal endothelial dysfunction

Question 49

eclampsia

Answer 49

seizure in women with preeclampsia

Question 50

HELLP

Answer 50

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

Question 51

acute fatty liver of pregnancy

Answer 51

rare microvesicular fatty change 3rd trimester - leading to liver failure unknown cause