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130 Cards in this Set
- Front
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possible diseases presenting with: -initially asymptomatic (routine lab tests show elevated AP) -progresses to: pruritus & fatigue -URQ (biliary colic) & fever -later presentation: jaundice & melanosis -hepatocellular failure & portal HTN -dark urine, hepatosplenomegaly, light stools |
primary biliary cirrhosis or secondary biliary obstruction |
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partial or total mechanical blockage of extra hepatic bile ducts leading to cirrhosis |
secondary biliary cirrhosis (biliary obstruction) |
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obstruction in secondary biliary cirrhosis can be due to? (6) |
-stone formation -duct atresia or stricture -expanding ductal or extraductal tumors such as pancreatic head cancer -pancreatitis -traumatic obstruction of bile duct leads to stasis -cholangitis |
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autoimmune disease causing inflammation / fibrosis of hepatic ducts -occurs in men <40 y/o |
primary sclerosing cholangitis |
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what other disease is primary sclerosing cholangitis assoc. w/? |
ulcerative colitis |
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autoimmune destruction of intrahepatic bile ducts --> portal inflammation -cirrhosis, liver failure -seen in middle aged women (40-50) |
primary biliary cirrhosis |
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over years to decades, what can PBC eventually lead to? |
-portal inflammation -cirrhosis -liver failure |
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90% of ppl with ____ have anti-mitochondrial auto-antibodies (AMA) |
PBC |
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nonsuppurative destruction of small to medium sized intrahepatic bile ducts |
PBC |
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ursodeoxycholic acid is tx for ____ |
PBC |
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#2 cause of fatal liver disease -liver gets metastatic cells from breast, colon, lung |
metastatic liver cancer |
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3 most common locations liver gets metastatic cancer from |
-breast, lung, colon |
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-sx typically refer to the primary cancer -obstructive jaundice -elevated LFTs -hepatomegaly |
metastatic liver disease |
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malignant hepatocytes occurring in liver already affected by cirrhosis |
hepatocellular carcinoma |
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highest rates of hepatocellular carcinoma are seen in what parts of world? |
-Asia, Africa |
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pre-existing conditions/disorders/lifestyle behaviors that are assoc. w/ hepatocellular carcinoma (4) |
-chronic alcoholism -viral hepatitis -cirrhosis -dietary factors |
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-asymptomatic until later stages -abdominal pain -weight loss -malaise -anorexia -cachexia -jaundice, pale stools, dark urine -fever -check their alpha-fetoprotein levels |
hepatocellular carcinoma |
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check pt's alpha-fetoprotein levels is common to do for what liver disease? |
hepatocellular carcinoma |
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common condition in which fatty liver disease develops in ppl who don't drink alcohol |
non-alcoholic fatty liver disease |
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3 types of changes to liver that occur in non-alcoholic fatty liver disease |
-steatosis -steatohepatitis -cirrhosis |
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5 disorders/ conditions/ syndrome assoc. w/ NAFLD |
-metabolic syndrome -DM II -HTN -obesity |
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cryptogenic cirrhosis is seen in ______ |
NAFLD |
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hepatic fat accumulation & resultant hepatic oxidative stress |
NAFLD |
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tx/management for NAFLD |
-no proven tx but should aggressively manage associated conditions such as DMII, obesity, HTN |
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type of inheritance pattern for Wilson disease |
autosomal recessive |
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marked accumulation of toxic levels of copper in brain, eye, liver |
Wilson disease |
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how does one get Wilson disease? |
inherited: AR
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what happens pathophysiologically in Wilson disease? |
when you inherit the mutation in the ATP7B gene, the ATP dependent cation channel that transports copper within hepatocytes doesn't work so copper isn't excreted out of liver and into bile; it stays stuck within liver --> damages hepatocytes |
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failure of hepatic excretion of copper from liver into bile -what is this due to? which gene? |
Wilson disease due to mutation in ATP7B gene |
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Wilson disease: _____ accumulates within liver --> catalyzes formation of _________ which induces damage in hepatocytes |
copper; O2 radicals |
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Pigmentation in cornea: Kayser-Fleisher rings) -seen in what? |
Wilson disease |
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Wilson disease: failure of copper to enter circulation as ____________ |
ceruloplasmin |
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Copper is Hella BAD 5 C's 3 D's For what disease? |
Wilson disease -ceruloplasmin low -carcinoma -cirrhosis -corneal deposits (Kayser-Fleisher rings) -copper accumulation -Hemolytic anemia -Basal ganglia degeneration -Asterixis -Dementia, dyskinesia, Dysarthria |
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pt population affected by Wilson disease |
teenagers - adults |
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autosomal recessive disorder of AAT gene |
alpha-1 antitrypsin disease |
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abnormally low serum levels of AAT within hepatocytes |
alpha-1 antitrypsin disease |
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what is the function of alpha-1 antitrypsin? -what happens when there is a deficiency of this? |
to inhibit proteases that are released at sites of inflammation -there is unrestrained activity of the proteases leading to destructive proteases |
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pulmonary emphysema is seen as a result of a deficiency in _______ what disease is this? |
AAT --> AAT disease |
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homozygous for PiZZ alpha-1 antitrypsin: causes _____ to build up in ______ inducing ______ in ______ what disease? |
mutant AAT protein; hepatocytes; apoptosis in hepatocytes alpha-1 antitrypsin disease |
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1/3 most common lethal genetic disease found in Caucasians |
AAT disease |
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genetic disorder: adults: asymptomatic cirrhosis, pulmonary disease/emphysema, chronic hepatitis children: cholestasis, jaundice, acholic stool, hepatomegaly |
AAT disease |
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classic triad: 1. cirrhosis 2. diabetes 3. skin pigmentation seen in ? |
hemochromatosis |
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excessive deposition of iron within various body tissues, esp. liver |
hemochromatosis |
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2 forms of hemochromatosis |
1. herediatry (AR) - primary 2. excessive iron intake - secondary |
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age/ gender of ppl most commonly affected by hemochromatosis |
men age 40-60 |
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genetic pattern for inheriting hemochromatosis |
AR |
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gene & chromosome for hemochromatosis |
HFE gene on chromosome 6 |
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defect in regulation of intestinal absorption of iron |
hemochromatosis |
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what is the main pathogenetic reason why one would develop hemochromatosis? |
there is a defect in regulation of intestinal absorption of iron |
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enterocytes can't import iron into their cytoplasm, sense iron deficiency, and increase absorption of iron |
hemochromatosis |
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diffuse deposition of iron in hepatocytes leads to what pathology within liver? |
fibrosis and cirrhosis (hepatomegaly) |
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what other organs are affected by diffuse deposition of iron? how does this manifest? |
pancreas --> DM; endocrinopathy Heart --> CHF skin --> increased melanin production = pigmentation Joints --> arthropathy
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difference between Criggler-Najjar & Gilbert syndrome? |
G: mildly low UGT activity --> not as significant a buildup of UCB --> no jaundice, except during times of stress or infection CN: near absence of UGT --> massive buildup of UCB --> jaundice, kernicterus, fatal disease |
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2 types of Criggler-Najjar syndrome- explain them |
CN Type I: total dysfunction of UGT enzyme CN Type II: little function of UGT enzyme |
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hereditary hyperbilirubinemia -inherited defect in _____ leading to death by 18 months of age or kernicterus what disease? |
UGT enzyme --> Criggler-Najjar |
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which type of Criggler-Najjar leads to death by 18 mon of age? |
I |
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which type of Criggler-Najjar leads to kernicterus? |
II |
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kernicterus |
accumulation of UCB within basal ganglia --> neuro deficits such as athetosis, dyskinesia, intellectual disabilities, vision problems, poor feeding, seizures |
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hereditary hyperbilirubinemia -mild defect in UGT --> intermittent jaundice |
Gilbert syndrome |
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when does Gilbert syndrome present? in which ppl? -how do they present? |
at puberty --> mildly elevated bilirubin or jaundice |
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in Gilbert syndrome, elevated bilirubin or jaundice is accentuated during which conditions? (5) |
-stress -menstrual periods -infections (or other comorbid illnesses) -dehydration -fasting |
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children <15 y/o who have decreased mitochondrial enzyme function |
Reye syndrome |
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acute onset, profound hepatic mitochondrial dysfunction leading to hepatic failure |
Reye syndrome |
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Reye syndrome usually occurs after _____ |
acute viral infections such as influenza |
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Reye syndrome: 4 potential presenting findings? |
-vomiting -hypoglycemia -progressive CNS damage --> stupor -hepatic injury/hepatomegaly |
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-inhibition of oxidative phosphorylation & fatty acid beta-oxidation -cytoplasmic fatty vacuolization in hepatocytes -astrocyte edema & loss of neurons in brain secondary to hyperammonemia -edema & fatty degeneration of proximal lobules of kidneys |
Reye syndrome |
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cholestatic injury |
an injury that impairs bile from leaving liver --> blockage --> obstructive jaundice |
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which zone is most affected by drugs or toxins that injure the liver? |
3 |
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presentation of drug-induced or toxic hepatitis ranges from _____ to ______ |
asymptomatic elevated liver enzymes to fulminant liver failure |
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drug used to acetaminophen toxicity of liver |
N-acetyl cysteine |
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2 injuries that result during drug-induced or toxic hepatitis |
1. cholestatic injury 2. hepatocellular injury |
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in drug-induced or toxic hepatitis, certain drugs such as acetaminophen, predictable hepatic injury occurs based on _____? |
amount of exposure to the drug |
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most common toxic hepatic disease in Western countries |
alcoholic liver disease/cirrhosis |
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3 related entities that make up alcoholic liver disease |
1. hepatic steatosis 2. alcoholic hepatitis 3. alcoholic cirrhosis |
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3 factors that contribute to development of alcoholic liver disease |
-genetic predisposition -quantity -duration of alcoholic consumption |
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hepatic steatosis -how much alcohol consumption -liver changes |
-entity of alcoholic liver disease -macrovesicular fatty changes in liver -reversible -occurs w/ moderate alcohol intake |
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alcoholic hepatitis -how much alcohol consumption -describe hepatocytes -presence of ____ -AST & ALT findings |
-entity of alcoholic liver disease -requires long term, sustained alcoholic consumption -swollen/ necrotic hepatocytes w/ neutrophilic infiltration -mallory bodies present -AST > ALT |
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alcoholic cirrhosis -liver changes -appearance of liver? -what happens to central vein? -manifestations similar to what? |
-entity of alcoholic liver disease -final & irreversible form -micronodular irregularly shrunken liver "hobnail" appearance sclerosis around central vein -has manifestation of chronic liver disease: jaundice, hypoalbuminemia |
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diagnostics to check for alcoholic liver disease |
-PT -transaminase levels -serum bilirubin, albumin, ammona -CBC -liver biopsy |
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findings of alcoholic liver disease |
-asymptomatic elevation of LFTs -fever, anorexia, malaise, abdominal pain -icterus, tender hepatomegaly, spider angiomas -signs of portal HTN -cirrhosis |
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autoimmune attack against unknown hepatic antigen |
autoimmune hepatitis |
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chronic progressive immune attack is mounted on hepatocytes --> cirrhosis, loss of liver function & portal HTN |
autoimmune hepatitis |
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2 types of autoimmune hepatitis -most common one? -who typically presents w/ this type? |
type I & II type I: most common --> found in young girls & women |
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hepatitis in neonates results from which 2 processes? which is more common? |
-systemic disease (more common) -primary infections (unusual) |
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non-infectious causes of neonatal hepatitis (3) |
-anatomic: biliary atresia -metabolic: AAT disease -toxic causes |
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yellow discoloration of skin |
jaundice |
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how does jaundice develop |
increased serum bilirubin: >2.5 mg/dl |
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RBCs have life cycle of ____ days |
120 |
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which system consumes RBCs once their lifecycle is over? |
reticuloendothelium system |
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most prominent location of reticuloendothelium system |
spleen |
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bilirubin metabolism |
-RBCs & hemoglobin consumed by macrophages of RE system -Hb --> heme & globin -globin --> AA -heme --> Fe & protoporphyrin (become UCB) -UCB attaches to albumin in liver -liver takes up UCB --> UGT conjugates it -CB --> bile canaliculi --> bile ducts --> gallbladder for storage -excreted into duodenum during digestion of fats -intestinal flora convert CB --> urobilinogen --> makes stool brown -some urobilinogen is reabsorbed into blood and filtered thru kidneys --> makes urine yellow |
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increased resistance in right side of heart, hepatic vein, liver, or portal vein -splanchnic blood seeks other routes of exit from visceral circulation |
portal HTN |
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prehepatic causes of portal HTN |
-portal vein thrombosis -increased splenic flow |
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intrahepatic causes of portal HTN (4) |
-cirrhosis -primary biliary cirrhosis -toxins to liver -congenital hepatic fibrosis |
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posthepatic causes of portal HTN (4) |
-venal caval obstruction -Budd-Chiari syndrome (thrombosis of one or major hepatic veins) -tricuspid regurg -sarcoidosis
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effects of portal HTN (6) |
-esophageal varicies (hematemesis) -splenomegaly (ascites) -caput medusae -hemorrhoids -peptic ulcer --> gastropathy -spontaneous bacterial peritonitis |
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end point of progressive damage to liver -most severe clinical consequence of liver disease |
hepatic failure |
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how much liver function must be lost before hepatic failure occurs? |
80-90% |
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temporal profile of chronic liver failure |
> 6 mon |
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temporal profile of subacute liver failure |
3-6 mon |
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temporal profile of acute liver failure |
<3 mon |
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temporal profile of fulminant liver failure |
<6 weeks |
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sudden & massive destruction of liver parenchyma -massive necrosis of liver -progresses to hepatic encephalopathy within 2-3 weeks |
acute hepatic failure |
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3 criteria for acute hepatic failure |
1. rapid development of hepatocellular dysfunction 2. encephalopathy 3. absence of prior liver disease hx |
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2 most common causes of acute hepatic failure |
drugs viral hepatitis |
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disruption of normal hepatocytes by fibrosis & nodular regenerative changes |
cirrhosis |
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cirrhosis leads to increased risk of developing ? |
HCC |
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effects of cirrhosis? |
1. ascites --> organs that drain vascular supply can't get the fluild thru liver --> back up into peritoneal cavity 2. congestive splenomegaly 3. portosystemic shunts --> blood can't get thru liver so finds other ways of getting back into circulation (EV, ARH, CM) 4. hepatorenal syndrome: due to splanchnic vasoconstriction 5. decreased hepatic fxn |
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physical exam findings of cirrhosis (11) |
1. hepatic encephalopathy 2. jaundice 3. bleeding from decreased clotting factors 4. anemia 5. edema 6. asterixis 7. scleral icterus 8. spider nevi 9. gynecomastia 10. testicular atrophy 11. fetor hepaticus (musty breath) |
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effusion & accumulation of serous fluid in peritoneal cavity |
ascites |
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2 types of ascites |
transudative & exudative |
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-increased R to fluid movement thru liver -P & V increase in splanchnic circulation -NO release --> splanchnic vasodilation --> decreased systemic circulation --> under-perfused kidneys -leads to ADH release Na retention --> increases fluid volume & CO -increased hydrostatic P, hypoalbuminemia, reduce plasma oncotic P |
ascites |
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-benign self-limited viral hepatitis -transmitted via fecal-oral route or ingestion of contaminated food/ water -does not cause chronic hepatitis or carrier state |
Hep A |
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viral hepatitis that has multiple clinical syndromes: 1. acute hep w/ full recovery 2. fulminant hepatitis w/ massive liver necrosis 3. nonprogressive chronic hepatitis 4. progressive chronic disease sometimes ending w/ cirrhosis or HCC 5. asymptomatic carrier state |
Hep B |
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routes of transmission for Viral Hep B |
blood or body fluids or vertical transmission in endemic regions |
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HBcAg is found in what situation vs HBsAg? -what is HBcAg vs HBsAg? |
Hep B core Ag: found in ppl who have been infected with the actual virus Hep B surface Ag: found in ppl who have been vaccinated against the virus |
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ground glass cell seen on histo -morphological feature of ____??? -what causes this appearance? |
Viral Hep B -accumulation of HBsAg in cytoplasm |
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how is Hep B largely prevented? |
vaccination & screening of blood donor organs/ tissues |
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infection of this viral hepatitis has greatest rate of progression to chronic disease and eventual cirrhosis and higher rate to progress to HCC |
Hep C |
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major route of transmission for Hep C |
-blood inoculation --> blood transfusions/ organ donation -IV drug users: sharing needles |
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hallmark of Hep C infection |
persistent infection |
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viral hep D only causes infection if ???? |
-if the person has been infected w/ Hep B and has HBsAg |
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2 clinical scenarios in which u can get infected w/ Hep D |
1. acute co-infection after exposure to serum containing HBV & HDV 2. superinfection --> already have Hep B infection and get newly inoculated w/ Hep D |
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viral hepatitis that is enterically transmitted -- waterborne infection -not assoc. w/ chronic liver disease or persistent viremia -usually is self-limited but has high mortality rate among pregnant women |
Hep E |
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-asymptomatic -has serologic anti-viral antibodies -mildly elevated serum transaminases |
asymptomatic carrier state for viral hepatitis |
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-fever, malaise, weight loss, vomiting, diarrhea, fatigue, HA, muscle/ joint aches, loss of appetite -elevated serum aminotransferases -mildly enlarged/ tender liver -jaundice/ dark colored urine -pale colored stool -pruritus -has serologic anti-viral antibodies |
acute hepatitis |
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acute hepatitis occurs in which types of hepatitis? -which one is most common? |
A-E B is most common |
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massive hepatic necrosis w acute liver failure |
fulminant hepatitis |
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-may or may not have progression to cirrhosis -defined by presence of symptomatic, biochemical, or serologic evidence of continuing or relapsing hepatic disease for >6 mon -persistent elevations of serum aminotransferases -fatigue, loss of appetite, jaundice, spider angiomas, palmar erythema, mild hepatomegaly, hepatic tenderness |
chronic hepatitis |
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asymptomatic but can harbor the virus and transmit to others -some can have liver damage evident on biopsy but are free of sx |
chronic carrier state |
