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MSII: Pathology Unit 6 > Rubin's Liver (1) > Flashcards

Rubin's Liver (1) Flashcards

1
Q

What is the basic unit of the liver?

A

Liver lobule (hexagon with portal triads at the angles)

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2
Q

“bile duct + hepatic artery + portal vein”

A

Portal triad

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3
Q

What is in the center of the liver acinus?

A

portal tract

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4
Q

What are the zones of the liver acinus?

A

Zone 1= most highly oxygenated zone arond the portal tracts

Zone 2= intermediate

Zone 3= surrounds central vein (poorly oxygenated)

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5
Q

Name the 3 cell types in the hepatic sinusoid?

A

Endothlail cells

Kupffer cells

Stellate cells

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6
Q

Function of stellate cells?

A

storage cells for fat and vitamins ADEK

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7
Q

Function of kupffer cells?

A

bone marrow derived phagocytes

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8
Q

What are the phases of hepatic regeneration?

A

Priming

Progression to mitosis

Nonparenchymal cells

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9
Q

Priming depends on…

A

TNF-alpha and IL-6

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10
Q

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

A

Kernicterus

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11
Q

:inherited disease that results in the absence of UGT”

A

Crigler- Najjar Syndrome

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12
Q

What are the symptoms of crigler- najjar?

A

Increased UCB

Kerncterus

Usually fatal

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13
Q

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

A

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

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14
Q

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

A

Gilbert Syndrome

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15
Q

Symptoms of Gilbert syndrome?

A

Increased UCB

Jaundice during stress

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16
Q

“deficiency of bilirubin canalicular transport protein (AR)”

A

Dubin’ Johnson Syndrome

17
Q

Symptoms of Dubin- Johnson Syndrome?

A

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

intermittent jaundice and dark urine

18
Q

“AR inherited familial conjugated hyperbilirubinemia”

A

Rotor Syndrome

19
Q

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

A

Progressive familial intrahepatic cholestasis

20
Q

“transiently low UGT activitiy treatable with phototherapy”

A

Neonatal (physiologic) Jaundice

21
Q

Symptoms of neonatal jaundice?

A

deposition of UCB in basal ganglia (kernicterus)

neurologic deficits and death

22
Q

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

A

Erythroblastosis fetalis

23
Q

Name 4 clinical manifesatations of impaired canalicular bile flow?

A

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

24
Q

“bile plugs in dilated canaliculi, without inflammation”

A

pure cholestasis

25
Q

Finding seen in rupture of dilated bile ducts?

A

Golden bike lakes

26
Q

Name 7 major extrahepatic obstructions to bile flow?

A

Bile duct carcinoma

Enlarged lymph nodes

sclerosing cholangitis

congenital biliary atresia

Pancreatic carcnoma

Gallstones

Carcinoma of ampulla of Vater

27
Q

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

A

Cirrhosis

28
Q

What mediated fibrosis in cirrhosis?

A

TGF-beta from stellate cells

29
Q

“small uniform nodules separated by thin fibrous septa”

A

micronudular type of cirrhosis

30
Q

“large, coarse irregular nodules with bands of collagenous septa”

A

macronodular type

31
Q

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

A

hepatic failure

32
Q

Symptoms of hepatic failure?

A

Jaundice

Hepatic encephalopathy

Defects of coagulation

Hypoalbuminemia

Cirrhosis

33
Q

What causes hepatic encephalopathy?

A

a combination toxicity due to impaired hepatocyte metabolism and shunting

34
Q

How can hepatic encephalopathy present?

A

cerebral edema (changes in Alzheumer type II astrocytes)

sleep disturbance–> lethargy and disorientation–> deep somnolence–> coma

35
Q

What usually causes intrahepatic portal HTN?

A

cirrhosis

36
Q

How does schistocomiasis lead to portal HTN?

A

granulomas in the portal venules–> prehepatic portal HTN symptoms

37
Q

What usually causes presinusoidal portal HTN?

A

Portal Vein thrombosis, AV fistulas, splenomegaly

38
Q

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

A

Budd- Chiari Syndrome

MSII: Pathology Unit 6 (12 decks)

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