Rubin's Liver (1) Flashcards
What is the basic unit of the liver?
Liver lobule (hexagon with portal triads at the angles)
“bile duct + hepatic artery + portal vein”
Portal triad
What is in the center of the liver acinus?
portal tract
What are the zones of the liver acinus?
Zone 1= most highly oxygenated zone arond the portal tracts
Zone 2= intermediate
Zone 3= surrounds central vein (poorly oxygenated)
Name the 3 cell types in the hepatic sinusoid?
Endothlail cells
Kupffer cells
Stellate cells
Function of stellate cells?
storage cells for fat and vitamins ADEK
Function of kupffer cells?
bone marrow derived phagocytes
What are the phases of hepatic regeneration?
Priming
Progression to mitosis
Nonparenchymal cells
Priming depends on…
TNF-alpha and IL-6
“reversible brain injury due to fre bilirubin toxicity in newborns”
Kernicterus
:inherited disease that results in the absence of UGT”
Crigler- Najjar Syndrome
What are the symptoms of crigler- najjar?
Increased UCB
Kerncterus
Usually fatal
Which type of crigler- najjar more severe (type I or type II)?
Type I (AR inheritance) but it is also more rare
“mildly low UGT activity leading to mild chronic unconjugated hyperbilirubinemia”
Gilbert Syndrome
Symptoms of Gilbert syndrome?
Increased UCB
Jaundice during stress
“deficiency of bilirubin canalicular transport protein (AR)”
Dubin’ Johnson Syndrome
Symptoms of Dubin- Johnson Syndrome?
“black” liver (melanin like migment from kupffer cells with Fe)
intermittent jaundice and dark urine
“AR inherited familial conjugated hyperbilirubinemia”
Rotor Syndrome
“AR disorder mutation i FIC1 transporter, bile salt transporters, MRPs”
Progressive familial intrahepatic cholestasis
“transiently low UGT activitiy treatable with phototherapy”
Neonatal (physiologic) Jaundice
Symptoms of neonatal jaundice?
deposition of UCB in basal ganglia (kernicterus)
neurologic deficits and death
“bilirubin overproduction due to maternal- fetal blood group mismatch and immune mediated hemolysis”
Erythroblastosis fetalis
Name 4 clinical manifesatations of impaired canalicular bile flow?
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
“bile plugs in dilated canaliculi, without inflammation”
pure cholestasis
Finding seen in rupture of dilated bile ducts?
Golden bike lakes
Name 7 major extrahepatic obstructions to bile flow?
Bile duct carcinoma
Enlarged lymph nodes
sclerosing cholangitis
congenital biliary atresia
Pancreatic carcnoma
Gallstones
Carcinoma of ampulla of Vater
“end stage liver damage characterized by disruption of the normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes”
Cirrhosis
What mediated fibrosis in cirrhosis?
TGF-beta from stellate cells
“small uniform nodules separated by thin fibrous septa”
micronudular type of cirrhosis
“large, coarse irregular nodules with bands of collagenous septa”
macronodular type
“clinical syndrome that occurs when the mass of liver cells or their function cannot sustain the vital activities”
hepatic failure
Symptoms of hepatic failure?
Jaundice
Hepatic encephalopathy
Defects of coagulation
Hypoalbuminemia
Cirrhosis
What causes hepatic encephalopathy?
a combination toxicity due to impaired hepatocyte metabolism and shunting
How can hepatic encephalopathy present?
cerebral edema (changes in Alzheumer type II astrocytes)
sleep disturbance–> lethargy and disorientation–> deep somnolence–> coma
What usually causes intrahepatic portal HTN?
cirrhosis
How does schistocomiasis lead to portal HTN?
granulomas in the portal venules–> prehepatic portal HTN symptoms
What usually causes presinusoidal portal HTN?
Portal Vein thrombosis, AV fistulas, splenomegaly
“congestive disease of the liver because occlusion of the hepatic veins and their tributaries, commonly due to Polycythemia vera”
Budd- Chiari Syndrome
