Term
| What is the functional reserve of the liver? |
|
Definition
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Term
| Name the liver function tests. |
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Definition
| total serum proteins, albumin, PT (prothrommbin time), PTT (partial thromboplastin time), AST, ALT, ALP, GGT (gamma-glutamyl transferase), and bilirubin |
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Term
| What's the normal weight of an adult liver? |
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Definition
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Term
| What is the normal span of the liver on palpation/percussion? |
|
Definition
midclavicular is 6-12 cm
midsternal is 4-8 |
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Term
| What are the two models of hepatic microarchitecture? |
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Definition
| lobule (anatomic) and acinus (functional) |
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Term
| What are the acinar zones? |
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Definition
| gradients of metabolic activity where zone 1 is closes to the hepatic triad and zone 3 is closest to the central vein |
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Term
| What's another name for central veins? |
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Definition
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Term
| What do you call the row of hepatocytes that surrounds the heptic triad? |
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Definition
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Term
| What stain often used for the liver stains the CT around the portal triad blue? |
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Definition
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Term
| How are the individual liver cells organized in relation to other liver cells? |
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Definition
| hepatic cords (sheets, and plates of cells), seen best with reticulin stain |
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Term
| Describe the endothelium of the sinusoids? |
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Definition
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Term
| Besdies hepatocytes what other cells are associated with liver sinusoids? |
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Definition
| perisinusoidal stellate cells, endothelial cells and kupffer cells |
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Term
| What stain is used to identified kupffer cells? |
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Definition
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Term
| What's another name for perisinusoidal stellate cells? What do they stain with? |
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Definition
| ito cells, osmium tetroxide |
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Term
| Name the prgression from small to large of bile conduits? |
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Definition
| canaliculi, canals of hering, ductules (or cholangiolse), bile ducts |
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Term
| What can be found in/around the hepatic triad? |
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Definition
| ductules, duct, hepatic artery, portal vein, lymhpatics |
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Term
| What do you call it when hepatocytes swell in response to injury? |
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Definition
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Term
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Definition
| cytokeratin tangles found in injured hepatocytes |
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Term
| What is foamy or feathery degeneration? |
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Definition
| when droplets of bile pigment accumulate within the hepatocytes which take on a fine, foamy appearance |
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Term
| What's a relatively easy way to tell between macrovesicular and microvesicular steatosis? |
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Definition
| microvesicular steotosis has centrally placed nuclei; macrovesicular steotosis has peripherally displaced nuclei |
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Term
| What type of necrosis occurs in the liver? |
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Definition
| focal lytic necrosis (drop out); also, piece meal necrosis (interface hepatitis); bridging necrosis (central-portal); submassive necrosis; massive necrosis |
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Term
| What's the difference between a councilman body and an apoptotic body? |
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Definition
| apoptotic body has a nuclei |
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Term
| What are the two types of fibrosis in the liver? |
|
Definition
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Term
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Definition
a diffuse process characterized by fibrosis (broad bridging fibrous septa), structurally abnormal and regenerative parenchymal nodules
i.e. scarring and regenration affecting the entire liver |
|
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Term
| What are the two types of cirrhosis? |
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Definition
| macronodular (more common) and micronodular |
|
|
Term
| What is the pathogenesis of cirrhosis? |
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Definition
| hepatocyte injury and regernation, inflammation, transformation of stellate cells into myofibroblast-like cells, deposition of collagen into the spaces of disse, and bridging fibrosis |
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Term
| Deposition of collagen in the spaces of disse in cirrhosis results in... |
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Definition
| loss of sinusoidal fenestrations, impaired exchange of nutrients, waste products, impaired secretion of proteins |
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|
Term
| Bridging fibrosis associated with cirrhosis results in... |
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Definition
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Term
|
Definition
| alcoholic, viral, primary biliary cirrhosis, autoimmune hepatitis, inherited disorders, non-alcoholic steatohepatitis, cyptogenic |
|
|
Term
| What are the leading causes of viral hepatitis? |
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Definition
| in the US mostly hepatitis C; in the world mostly hep B |
|
|
Term
| What inherited disorders cause cirrhosis? |
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Definition
| primary hemochromatosis, wilson's disease, alpha-1-antitrypsin deficiency |
|
|
Term
| What are possible outcomes of cirrhosis? |
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Definition
| remain asymptomatic, portal HTN, overt liver failure, hepatocellular carcinoma |
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Term
| What are the different types of hyperbilirubinemia? |
|
Definition
| unconjugated hyperbilirubinemia and conjugated hyperbilirubinemia (due to cholestasis) |
|
|
Term
| Where does bilirubin come from? |
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Definition
| derived from the prosthetic group heme which can be found in hemoglobin (old RBCs destroyed by spleen or new RBCs destroyed in bone marrow) or other hemoproteins (cytochromes mostly in the liver) |
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|
Term
| What causes newly formed RBCs to be destroyed in the bone marrow? |
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Definition
| ineffective erythropoiesis, thalassemia, sideroblastic anemia, and megaloblastic anemia |
|
|
Term
| What is the first step in heme breakdown? |
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Definition
| heme --> biliverdin by heme oxygenase with O2 and NADPH, creating Fe++, CO, H2O, and NADP |
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|
Term
| What is the second reaction of heme break down? |
|
Definition
| biliverdin --> bilirubin by biliverdin reductase using HADPH and H+ and creating NADP+ |
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|
Term
| What are the characteristics of unconjugated (indirect) bilirubin? |
|
Definition
| water-insoluble, tightly bound to albumin, not excreted in urine, potentially toxic |
|
|
Term
| How do you get rid of unconjugated or indirect bilirubin? |
|
Definition
| hepatocyte carrier-mediated uptake; conjugation with UDP-glucuronosyl transferase (UGT) |
|
|
Term
| How do you conjugate bilirubin? |
|
Definition
| two separte steps involving two UDP glucuronic acids turned into two UDPs. Makes bilirubin diglucuronide |
|
|
Term
| What are the characteristics of direct/conjugated bilirubin? |
|
Definition
| water-soluble, loosely bound to albumin, excreted in the urine, non-toxic |
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|
Term
| How do you further process or get rid of conjugated or direct bilirubin? |
|
Definition
| actively excrete into the bile canaliculi |
|
|
Term
| What heme break down product is excreted in the urine? |
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Definition
|
|
Term
| What bactieral enzyme converts bilirubin diglucoronide? |
|
Definition
|
|
Term
| What breakdown product of heme is found in the feces? |
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Definition
| 80% is stercobilinogen or stercobilin |
|
|
Term
| What heme breakdown product is found in the bile duct? |
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Definition
|
|
Term
| At what bilirubin level does a patient appear jaundiced? |
|
Definition
|
|
Term
| What causes unconjugated/indirect hyperbilirubinemia? |
|
Definition
| excessive bilirubin production (internal bleeding, hemolytic anemia), reduced uptake into hepatocytes (drugs/hepatocyte injury), impaired conjugation within the hepatocytes (hepatocyte injury, hereditary hyperbilirubinemia, physiologic jaundice of the newborn |
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|
Term
| What type of hyperbilirubinemia is present in erythroblastosis fetalis? |
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Definition
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Term
| Erythroblastosis fetalis can lead to kernicterus which is... |
|
Definition
| injury of basal ganglia, pons and cerebellum leading to cerebral palsy, MR, hearing loss, lethargy, and death |
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|
Term
| What is physiologic jaundice of the newborn aka neonatal jaundice? |
|
Definition
| transient, mild unconjugated hyperbilirubinemia and immature conjugation of bilirubin |
|
|
Term
| What is the time course/outcome of neonatal jaundice? |
|
Definition
visible from 2nd to 3rd day
disppears between 5th and 7th day |
|
|
Term
| What percent of infants have neonatal jaundice? |
|
Definition
| 60% of term infants and 80% of prematures |
|
|
Term
| What effect breast feeding have on neonatal jaundice? |
|
Definition
| increases it via two mechanisms: 1)maternal milk inhibits UGT and 2)betaglucouronidases in maternal milk deconjugates bilirubin in the intestine (increased enterohepatic circulation) |
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|
Term
| What are the unconjugated hereditary hyperbilirubinemias? |
|
Definition
| they all involve impaired conjugation (UGT defect): crigler-Najjar type I and type II, and Gilbert syndrome |
|
|
Term
| What is Crigler-Najjar type I? |
|
Definition
| UGT is completely absent leading to kernicterus and death |
|
|
Term
| Whta is Crigler-Najjar type II? |
|
Definition
| markedly reduced UGT activity; nonfatal but risk of kernicterus |
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|
Term
|
Definition
| reduced UGT activity (about 30% of normal levels), common (6% of the population), and mild hyperbilirubinemia with fasting or stress |
|
|
Term
| What causes conjugated (direct) hyperbilirubinemia? |
|
Definition
| decreased hepatocellular excretion (hepatocellular injury or hereditary hyperbilirubinemia), or cholestasis (arrest in the flow of bile) |
|
|
Term
| What are the conjugated hereditary hyperbilirubinemias? |
|
Definition
| impaired bilirubin excretion with jaundice that are clinically inconsequential. Examples: rotor syndrome and dubin-johnson syndrome |
|
|
Term
|
Definition
| conjugated hereditary hyperbilirubinemia characterized by a non-pigmented liver |
|
|
Term
| What is Dubin-Johnson syndrome? |
|
Definition
| a conjugated hyperbilirubinemia that is characterized by a darkly pigmented liver. It is caused by a defect in a canalicular multi-specific organic anion transport protein and polymers of epinephrine accumulate. |
|
|
Term
|
Definition
| arrest in the flow of bile that leads to retention of bilirubin, bile acids and other solutes |
|
|
Term
| What are the primary bile acids? |
|
Definition
| chenodeoxycholic acid and cholic acid |
|
|
Term
| What is the purpose of bile acids? |
|
Definition
| excrete lipids from the liver and absorption of lipids from the intestines |
|
|
Term
| Bile acids emulsify lipids by forming _______. |
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Definition
|
|
Term
| What percent of bile acids are actively reaborbed? where are they reabosrbed? |
|
Definition
| 95% in the terminal ileum |
|
|
Term
| What do you call the process by which bile acids go from liver to bile to intestine to blood to liver and over and over again? |
|
Definition
| enterohepatic circulation |
|
|
Term
| What are the manifestations of cholestasis? |
|
Definition
| conjugated hyperbilirubinemia, elevated bile acids, hypercholesterolemia, elevated ALP and GGT, malabsorption of fat soluble vitamines (ADEK, bleeding diathesis), steatorrhea and acholia |
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|
Term
|
Definition
|
|
Term
| What is bleeidng diathesis? |
|
Definition
| impaired synthesis of vitamin K dependent coagulation factors II, VII, IX, and X |
|
|
Term
| What do you call grossly visible accumulations of cholesterol around the eyelids? |
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Definition
|
|
Term
| Chronic cholestasis leads to.. |
|
Definition
|
|
Term
| What are the major causes of cholestasis excluding neonatal? |
|
Definition
| obstructive (cholelithiasis and cholangitis) and hepatocellular (drug related and hepatitis) |
|
|
Term
| What are the different histological manifestations of cholestasis? |
|
Definition
| intracellular cholestasis, canalicular cholestasis, bile lakes, foamy/feathery degeneration, ductular proliferaiton, biliary cirrhosis |
|
|
Term
| What are the causes of neonatla cholestasis? |
|
Definition
obstructive: extrahepatic biliary atresia, choledochal cyst
hepatocellular (neonatal hepatitis): congenital metabolic disorders, infections, drugs, idiopathic |
|
|
Term
| What percent of hepatocellular neonatal cholestasis is idiopathic? |
|
Definition
|
|
Term
| What congenital metabolic disorders cause hepatocellular neonatal cholestasis? |
|
Definition
| alpha 1 antitrypsin deficiency, tyrosinemia, niemann-pick, galactosemia, etc. |
|
|
Term
| What is the histological finidng of neonatal (giant cell) hepatitis? |
|
Definition
| panlobular giant cell transofrmation of hepatocytes |
|
|
Term
| What causes liver failure? |
|
Definition
| massive necrosis (acute, fulminant, failure), dysfunction without overt necrosis, decompensation of chronic liver disease (chronic hepatitis and cirrhosis) |
|
|
Term
| What causes liver failure via massive necrosis? |
|
Definition
| acetaminophen and other drugs/toxins, hepatitis (viral hep A and B, and AI hep) |
|
|
Term
| What causes liver failure due to dysfunction without overt necrosis? |
|
Definition
| acute fatty liver (microvesicular necrosis) due to reye syndrome or acute fatty liver of pregnancy |
|
|
Term
| What causes liver failure via decompensation of chronic liver disease/ |
|
Definition
| chronic hepatitis and cirrhosis |
|
|
Term
| What are the manifestations of liver failure? |
|
Definition
jaundice, hypoalbuminemia (peripheral edema and ascites), coagulopathy, and hyperestrogenemia (gynecomastia)
also, spider angiomas, palmar erythema and asterixis, |
|
|
Term
| What are manifestations of liver failure? |
|
Definition
| fetor hepaticus, hyperammonemia/hepatic encephalopathy, hepatorenal syndrome (renal failure), respiratory failure (hepatopulmonary syndrome) |
|
|
Term
| What causes fetor hepaticus? |
|
Definition
| shunting of mercaptans derived from methionine |
|
|
Term
| What are the symptoms of hepatic encephalopathy (hyperammonemia)? |
|
Definition
| abnormal neurotransmission, brain edema, confusion, stupor, coma, rigidity, hyperreflexia, and asterixis |
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