Rubin's Liver (1)

Question 1

What is the basic unit of the liver?

Answer 1

Liver lobule (hexagon with portal triads at the angles)

Question 2

“bile duct + hepatic artery + portal vein”

Answer 2

Portal triad

Question 3

What is in the center of the liver acinus?

Answer 3

portal tract

Question 4

What are the zones of the liver acinus?

Answer 4

Zone 1= most highly oxygenated zone arond the portal tracts

Zone 2= intermediate

Zone 3= surrounds central vein (poorly oxygenated)

Question 5

Name the 3 cell types in the hepatic sinusoid?

Answer 5

Endothlail cells

Kupffer cells

Stellate cells

Question 6

Function of stellate cells?

Answer 6

storage cells for fat and vitamins ADEK

Question 7

Function of kupffer cells?

Answer 7

bone marrow derived phagocytes

Question 8

What are the phases of hepatic regeneration?

Answer 8

Priming

Progression to mitosis

Nonparenchymal cells

Question 9

Priming depends on…

Answer 9

TNF-alpha and IL-6

Question 10

“reversible brain injury due to fre bilirubin toxicity in newborns”

Answer 10

Kernicterus

Question 11

:inherited disease that results in the absence of UGT”

Answer 11

Crigler- Najjar Syndrome

Question 12

What are the symptoms of crigler- najjar?

Answer 12

Increased UCB

Kerncterus

Usually fatal

Question 13

Which type of crigler- najjar more severe (type I or type II)?

Answer 13

Type I (AR inheritance) but it is also more rare

Question 14

“mildly low UGT activity leading to mild chronic unconjugated hyperbilirubinemia”

Answer 14

Gilbert Syndrome

Question 15

Symptoms of Gilbert syndrome?

Answer 15

Increased UCB

Jaundice during stress

Question 16

“deficiency of bilirubin canalicular transport protein (AR)”

Answer 16

Dubin’ Johnson Syndrome

Question 17

Symptoms of Dubin- Johnson Syndrome?

Answer 17

“black” liver (melanin like migment from kupffer cells with Fe)

intermittent jaundice and dark urine

Question 18

“AR inherited familial conjugated hyperbilirubinemia”

Answer 18

Rotor Syndrome

Question 19

“AR disorder mutation i FIC1 transporter, bile salt transporters, MRPs”

Answer 19

Progressive familial intrahepatic cholestasis

Question 20

“transiently low UGT activitiy treatable with phototherapy”

Answer 20

Neonatal (physiologic) Jaundice

Question 21

Symptoms of neonatal jaundice?

Answer 21

deposition of UCB in basal ganglia (kernicterus)

neurologic deficits and death

Question 22

“bilirubin overproduction due to maternal- fetal blood group mismatch and immune mediated hemolysis”

Answer 22

Erythroblastosis fetalis

Question 23

Name 4 clinical manifesatations of impaired canalicular bile flow?

Answer 23

Pruritis–> bile acids in the skin

Xanthomas–> cholesterol accumulation in the skin

cirrhosis–> detergent and apoptotic actions of hepatocytes

Malabsorption–> chronic lack of bile in gut

Question 24

“bile plugs in dilated canaliculi, without inflammation”

Answer 24

pure cholestasis

Question 25

Finding seen in rupture of dilated bile ducts?

Answer 25

Golden bike lakes

Question 26

Name 7 major extrahepatic obstructions to bile flow?

Answer 26

Bile duct carcinoma Enlarged lymph nodes sclerosing cholangitis congenital biliary atresia Pancreatic carcnoma Gallstones Carcinoma of ampulla of Vater

Question 27

"end stage liver damage characterized by disruption of the normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes"

Answer 27

Cirrhosis

Question 28

What mediated fibrosis in cirrhosis?

Answer 28

TGF-beta from stellate cells

Question 29

"small uniform nodules separated by thin fibrous septa"

Answer 29

micronudular type of cirrhosis

Question 30

"large, coarse irregular nodules with bands of collagenous septa"

Answer 30

macronodular type

Question 31

"clinical syndrome that occurs when the mass of liver cells or their function cannot sustain the vital activities"

Answer 31

hepatic failure

Question 32

Symptoms of hepatic failure?

Answer 32

Jaundice Hepatic encephalopathy Defects of coagulation Hypoalbuminemia Cirrhosis

Question 33

What causes hepatic encephalopathy?

Answer 33

a combination toxicity due to impaired hepatocyte metabolism and shunting

Question 34

How can hepatic encephalopathy present?

Answer 34

cerebral edema (changes in Alzheumer type II astrocytes) sleep disturbance--> lethargy and disorientation--> deep somnolence--> coma

Question 35

What usually causes intrahepatic portal HTN?

Answer 35

cirrhosis

Question 36

How does schistocomiasis lead to portal HTN?

Answer 36

granulomas in the portal venules--> prehepatic portal HTN symptoms

Question 37

What usually causes presinusoidal portal HTN?

Answer 37

Portal Vein thrombosis, AV fistulas, splenomegaly

Question 38

"congestive disease of the liver because occlusion of the hepatic veins and their tributaries, commonly due to Polycythemia vera"

Answer 38

Budd- Chiari Syndrome