Pathology: Tbl 8: Hepatobiliary Disease: Block Iii

Front 1

Bilirubin is conjugated to glucuronic acid by _______.

Back 1

Glucuronyl Transferase

Front 2

What diseases are associated with unconjugated hyperbilirubinemia?

Back 2

1. Gilbert's/Crigler-Najjar
2. Hemolysis
3. Hepatitis

Front 3

This is a marker of bile duct obstruction or canaliculus damage and increased levels indicate failure of bile flow:

Back 3

Alkaline Phosphatase

Front 4

Conjugated hyperbilirubinemia is associated with what?

Back 4

Bile obstruction

Front 5

If Alk phos is increased but GGT is not, what does this indicate?

Back 5

It indicates that liver dysfunction is not present.

Front 6

In _______, alk phos and GGT are increased with mild increases in other liver enzymes.

Back 6

Obstructive Jaundice

Front 7

This is the first marker to present in HBV infection (2-8 weeks) and indicates HB carrier state:

Back 7

HBsAg

Front 8

This marker for HBV is the last to leave with waning infection and when it is present for more than 6 months it indicates chronic HBV.

Back 8

HBsAg

Front 9

This marker for HBV is protective and arrives after the window period:

Back 9

Anti-HBs

Front 10

This marker for HBV indicates immunization after an infection or vaccination:

Back 10

Anti-HBs

Front 11

This is a marker of previous HBV infection but not a marker of immunization/vaccination:

Back 11

Anti-HBc

Front 12

This is a non-protective marker of recent HBV infection that is positive during acute infections:

Back 12

IgM Anti-HBc

Front 13

This marker for HBV persists during the window phase (others are absent) and converts to IgG by 6 months.

Back 13

IgM Anti-HBc

Front 14

This is a marker of both active infection and recovery for Hepatitis C:

Back 14

Anti-HCV

Front 15

This marker for HCV doesn't distinguish between acute, chronic, or recovered persons and is not protective:

Back 15

Anti-HCV

Front 16

What infection is a concern with portal HTN?

Back 16

Spontaneous Bacterial Peritonitis due to translocation of the gut bacteria, usually coliforms.

Front 17

Tumors found in the liver, usually associated with chronic liver disease (esp. EtOH cirrhosis) and Hep C.

Back 17

Hepatocelluar Carcinoma

Front 18

This condition has a marked elevation of alpha feto-protein.

Back 18

Hepatocellular Carcinoma

Front 19

This condition causes severe spasmotic pain radiating to the back or right scapula that can be intermittent at first and gets worse with fatty meals - patient has a positive Murphy's sign:

Back 19

Gallstones

Front 20

This condition is characterized by an inflammed gallbladder due to obstruction and usually associated with gallstones:

Back 20

Cholecystitis

Front 21

This condition gives a positive Murphy's Sign

Back 21

Cholecystitis

Front 22

What is the biggest concern involving Cholecystitis?

Back 22

That the gallbladder may become necrotic and perforate, giving rise to peritonitis

Front 23

This condition involves the presence of gallstones within the biliary tree (usually pigmented), is more common in Asians and is associated with biliary tract infections:

Back 23

Choledocholithiasis

Front 24

This is the bacterial infection of the bile ducts that presents with a classic triad of a septic looking patient, fever, RUQ pain, and Jaundice:

Back 24

Ascending (infectious) Cholangitis

Front 25

This condition would have positve blood cultures, increased conjugated bilirubin, and increased AST and ALT:

Back 25

Ascending (infectious) Cholangitis

Front 26

This is a disease of unknown pathogenesis characterized by segmental inflammation of the intra and extrahepatic bile ducts. There is onion skinning on histologic section and it is associated with IBS (ulcerative colitis).

Back 26

Sclerosing Cholangitis

Front 27

This is an autoimmune disease characterized by granulomas of intrahepatic bile ducts. Patients present with pruritus, jaundice, painful hepatosplenomegaly, and xanthomas. Has serologic antimitochondrial antibody marker (AMA)

Back 27

Primary Biliary Cirrhosis

Front 28

This condition presents with fever, nausea/vomiting, with severe, stabbing, mid-epigastric pain that radiates to the back. May have hypovolemic shock. Can also cause ARDS because circulating phospholipases inactivate surfactant:

Back 28

Gallstone Pancreatitis

Front 29

This condition causes increased amylase and lipase with hypocalcemia, hyperglycemia and neutrophilic leukocytosis

Back 29

Gallstone Pancreatitis

Front 30

This is an autosomal dominant, multiorgan disorder that causes direct hyperbilirubinemia in infants. What is the significance of urobilinogen in the urine?

Back 30

Congenital Absence of bile ducts (Alagille Syndrome) and if urobilinogen is in the urine, bile is getting into the gut and things are less urgent.

Front 31

Gilbert and Cragler-Najaar are syndroms involving what enzyme?

Back 31

UGT1A1 Protein - UDP Glucuronyl Transferase

Front 32

Causes unconjugated hyperbilirubinemia in infants without evidence of hepatocellular damage:

Back 32

Criglar-Najaar and Gilbert's Syndromes

Front 33

This is a very common cause of mild unconjugated forms of jaundice with physiologic stress

Back 33

Gilbert's Syndrome

Front 34

What drug can cause problems with Crigler Najjar and Gilberts?

Back 34

Aspirin- acdic drugs can displace the albumin bound unconjugated bilirubin which can enter the brain

Front 35

This is a benign AR disorder caused by a defective cannalicular transport protein (MRP2) that causes conjugated hyperbilirubinemia. Has no consequences except for a black liver

Back 35

Dubin-Johnson Syndrome

Front 36

Is very similar to Dubin Johnson and may be related to defects in hepatic uptake of bilirubin and storage:

Back 36

Rotor Syndrome

Front 37

This condition is seen commonly in women and is associated with antinuclear antibodies (ANA) and anti smooth muscle antibodies (SMA). It can also be associated with the HLA-DR3 serotype. Both indirect and direct bilirubin are elevated and AST and ALT are in the thousands.

Back 37

Autoimmune Hepatitis

Front 38

This idiopathic giant cell hepatitis may be associated with congenital infections (CMV, syphilis, herpes) or Inborn Errors of Metabolism (A1-AT, Storage Diseases, CF).

Back 38

Neonatal Hepatitis

Front 39

Infant with hepatocellular damage, increased AST/ALT and both types of elevated bilirubin

Back 39

Neonatal Hepatitis

Front 40

Hepatitis transmitted by fecal-oral route, can get from shellfish, has an incubation period of 2-7 weeks and common in travelers:

Back 40

Hepatitis A

Front 41

Hepatitis with acute sickness, no carrier state and a vaccine:

Back 41

Hepatitis A

Front 42

This Hep A antibody indicates active infection

Back 42

Anti-HAV IgM

Front 43

What condition typically presents with ALT>AST? Which condition presents the opposite?

Back 43

Hepatitis commonly presents with ALT>AST whereas alcoholic hepatitis usually presents with AST>ALT

Front 44

This hepatitis has the typical acute presentation, about 90% of people recover and clear the infection without progression. If chronic, it is usually associated with PAN.

Back 44

Hepatitis B

Front 45

This Hep B antigen indicates that a patient it very infectious

Back 45

HBeAg

Front 46

This is the protective antibody against Hep B

Back 46

Anti-HBs IgG

Front 47

The acute form of this hepatitis is much less severe but a large percentage of infected patients become chronic and those who have chronic infections progress to cirrhosis.

Back 47

Hepatitis C

Front 48

If a hepatitis C RIBA and PCR are positive, what does this indicate?

Back 48

Active infection

Front 49

If a hepatitis C PCR is negative but RIBA is positive, what does this indicate?

Back 49

Prior Infection and Recovery

Front 50

Acute liver failure within 8 weeks of hepatic dysfunction that occurs when >80% of the parenchyma is lost. May result from viral/autoimmune hepatitis, drugs, or Reye syndrome. Presents with hepatic encephalopathy, asterixis, severe jaundice, and fetor hepaticus.

Back 50

Fulminant Hepatic Failure

Front 51

Steatosis of the liver with hepatocyte injury which may progress to cirrhosis. Associated with obesity, diabetes, and metabolic syndrome.

Back 51

Non-alcoholic Steatohepatitis (NASH)

Front 52

Presents clinically as asymptomatic increase in AST/ALT with fatigue, RUQ discomfort (hepatomegaly), and histologic slides show steatosis with inflammation, mallory bodies, and hepatocyte death.

Back 52

NASH

Front 53

This autosomal recessive disease is due to a mutation in the HFR gene that leads to unrestricted absorption of iron in the small intestine

Back 53

Hereditary Hemochomatosis

Front 54

This condition causes bronze diabetes due to increased serum iron

Back 54

Hereditary Hemochromatosis

Front 55

This condition shows liver or heart biposy with prussian blue stain

Back 55

Hereditary Hemochomatosis

Front 56

Autosomal dominant condition that is the most common cause of liver cirrhosis in children

Back 56

A-1 Antitrypsin Deficiency

Front 57

In this condition, the α-antitrypsin enzyme is not secreted properly and thus accumulates in hepatocytes and causes liver damage.

Back 57

α-1 antitrypsin deficiency

Front 58

This condition presents as neonatal hepatitis with intrahepatic cholestasis or as premature emphysema or bronchiectasis. Histologically shows PAS positive cytoplasmic granules

Back 58

α-1 Antitrypsin Deficiency

Front 59

This autosomal recessive disease is seen more commonly in males, ages 4-40, and is due to defective hepatocyte transport of copper

Back 59

Wilson Disease (Hepatolenticular Degeneration)

Front 60

This disease is associated with Kayser Fleischer rings, CNS symptoms producing movement disorders, hepatosplenomegaly, hemolytic anemia, and decreased serum ceruloplasmin and increased urine copper:

Back 60

Wilson Disease