Chapter 11 Exocrine pancreas, gall bladder, and liver

Question 1

What is an annular pancreas and what does it carry increased risk for?

Answer 1

Developmental malformation in which the pancreas forms a ring around the duodenum; risk of duodenal obstruction

Question 2

What is Acute pancreatitis and what is it due to? How is this set off?

Answer 2

Inflammation and hemorrhage of the pancreas. Due to autodigestion of pancreatic parenchyma by pancreatic enzymes. Premature activation of trypsin leads to activation of other pancreatic enzymes

Question 3

What does acute pancreatitis result in?

Answer 3

liquefactive hemorrhagic necrosis of the pancreas and fat necrosis of the peripancreatic fat.

Question 4

What is acute pancreatitis most commonly due to? (2) what are some other causes(7)

Answer 4

Most common: Alcohol and gallstones

Others: trauma, hypercalcemia, hyperlipidemia, drugs,, scorpion stings, mumps, and rupture of a posterior duodenal ulcer

Question 5

What are 5 clinical features of acute pancreatitis?

Answer 5

1 Epigastric abdominal pain that radiates to the back
2 Nausea and vomiting
3 Periumbilical and flank hemorrhage (necrosis spreads into the periumbilical soft tissue and retroperitoneum)
4 Elevated serum lipase and amylase; lipase is more specific for pancreatic damage.
5 Hypocalcemia (calcium is consumed during saponification in fat necrosis)

Question 6

What is a poor prognostic indicator in acute pancreatitis?

Answer 6

hypocalcemia

Question 7

What are 4 complications of acute pancreatitis (further questions on each)?

Answer 7

1 Shock
2 Pancreatic pseudocyst
3 pancreatic abscess
4 DIC and ARDS

Question 8

What causes shock in acute pancreatitis?

Answer 8

due to peripancreatic hemorrhage and fluid sequestration

Question 9

What forms pancreatic pseudocysts? how do they present? What is a complication?

Answer 9

formed by fibrous tissue surrounding liquefactive necrosis and pancreatic enzymes. Presents as an abdominal mass with persistently elevated serum amylase. rupture is associated with release of enzymes into the abdominal cavity and hemorrhage.

Question 10

What are pancreatic abscesses commonly due to in acute pancreatitis? how do they present?

Answer 10

often due to E. Coli; presents with abdominal pain, high fever, and persistently elevated amylase.

Question 11

What causes DIC and ARDS in Acute Pancreatitis?

Answer 11

DIC- enzymes activate coag cascade.

ARDS- enzymes chew up alveolar/capillary interface

Question 12

What is chronic pancreatitis and what is it most commonly due to?

Answer 12

Fibrosis of pancreatic parenchyma, most often secondary to recurrent acute pancreatitis. Most commonly due to alcohol (adults) and cystic fibrosis (children); however many cases are idiopathic.

Question 13

What are 5 clinical features of chronic pancreatitis?

Answer 13

1 Epigastric pain that radiates to the back
2 pancreatic insufficiency
3 Dystrophic calcification of pancreatic parenchyma on imaging.
4 secondary diabetes mellitus - complication due to destruction of islets
5 Risk for pancreatic carcinoma

Question 14

What does pancreatic insufficiency in chronic pancreatitis result in? Are amylase and lipase useful markers?

Answer 14

results in malabsorption with steatorrhea and fat-soluble vitamin deficiencies. Amylase and lipase are not useful serologic markers of chronic pancreatitis because vast majority of the pancreas has been destroyed and you are not making them.

Question 15

What does dystrophic calcification in chronic pancreatitis show on imaging?

Answer 15

Contrast studies reveal a “chain of lakes” pattern due to dilation of pancreatic ducts.

Question 16

What is pancreatic carcinoma, where does it arise and who is it most commonly seen in?

Answer 16

Adenocarcinoma arising from the pancreatic ducts. Most commonly seen in elderly (average age is 70 years)

Question 17

What are the 2 major risk factors for pancreatic carcinoma?

Answer 17

smoking and chronic pancreatitis

Question 18

What are 5 clinical features of pancreatic carcinoma and when do they occur?

Answer 18

Occur late
1 Epigastric abdominal pain with weight loss
2 Obstructive jaundice with pale stools and palpable gallbladder; associated with tumors that arise in the head of the pancreas (most common location)
3 Secondary diabetes mellitus; associated with tumors that arise in the body or tail
4 pancreatitis (tumor blocks ducts)
5 migratory thrombophlebitis (trousseau sign)

Question 19

What is the serum tumor marker for pancreatic carcinoma?

Answer 19

CA-19-9

Question 20

How does migratory thrombophlebitis present in pancreatic carcinoma?

Answer 20

Presents as swelling, erythema, and tenderness in the extremities. seen in 10% of patients

Question 21

What does treatment of pancreatic carcinoma involve?

Answer 21

Surgical resection en bloc removal of the head and neck of the pancres, proximal duodenum, and gall bladder (whipple procedure)

Question 22

What is the prognosis of pancreatic carcinoma?

Answer 22

very poor prognosis; 1 year survival is <10%

Question 23

What is biliary atresia, how does it arise and when, how does it present?

Answer 23

failure to form or early destruction of extrahepatic biliary tree. leads to biliary obstruction within the first 2 months of life. Presents with jaundice and progresses to cirrhosis

Question 24

What is cholelithiasis?

Answer 24

solid, round stones in the gallbladder

Question 25

What are gallstones due to and what are 3 situations that cause them?

Answer 25

due to precipitation of cholesterol or bilirubin in bile. Arises with (1) supersaturation of cholesterol or bilirubin. (2) decreased phospholipids (e.g. lecithin) or bile acids (normally increase solubility). (3) stasis (bacteria deconjugate bilirubin)

Question 26

What color are cholesterol stones? what percentage of stones? how do they appear on Xray?

Answer 26

Cholesterol stones are yellopw and the most common type of stone (90%), especially in the west. They are usually radiolucent (10% are radiopaque due to associated calcium)

Question 27

What are 6 risk factors for cholesterol stones?

Answer 27

1 Age (40s) 2 Estrogen (female gender, obesity, multigravida, OC) 3 Clofibrate (increases HMG-CoA red, and decreases conversion of cholesterol into bile acids.) 4 Native american ethnicity 5 Crohn Disease (damage to terminal ileum decreases reuptake of bile acids) 6 Cirrhosis (decreased production of bile acids)

Question 28

What color are bilirubin stones and how do they appear on xray?

Answer 28

pigmented stones that usually appear radiopaque.

Question 29

What are risk factors for bilirubin stones?

Answer 29

extravascular hemolysis (increases bilirubin in bile) and biliary tract infection (e.g., E coli, Ascaris lumbricoides, and Clonorchis Sinensis --> all deconjugate bilirubin)

Question 30

What is Ascaris Lubricoides?

Answer 30

common roundworm that infects 25% of the world's population, especially in areas with poor sanitation (fecal oral transmission); infects the biliary tract, increasing the risk for gallstones

Question 31

What is Clonorchis Sinensis?

Answer 31

endemic in China, Korea, and Vietnam (chinese liver fluke); infects the biliary tract, increasing the risk for gallstones, cholangitis, and cholangiocarcinoma

Question 32

Describe the symptoms of gallstones and 6 complications?

Answer 32

``` Gallstones are usually asymptomatic complications: 1 Biliary colic 2, acute cholecystitis 3 Chronic cholecystitis 4 ascending cholangitis 5 gallstone ileus 6 gallbladder cancer ```

Question 33

What is Biliary Colic? what is it due to? how are symptoms relieved? What are some complications?

Answer 33

Waxing and waning RUQ pain. Due to gallbladder contracting against a stone lodged in the cystic duct. Symptoms are relieved if the stone passes. Common bile duct obstruction may result in acute pancreatitis or obstructive jaundice.

Question 34

What is acute Cholecystitis? What causes it?

Answer 34

Acute inflammation of the gallbladder wall. Impacted stone in the cystic duct results in dilation with pressure ischemia, bacterial overgrowth (E. coli), and inflammation

Question 35

How does acute cholecystitis present and what is a risk if left untreated?

Answer 35

Presents with RUQ pain, often radiating to right scapula, fever with increased WBC count, nausea, vomiting, and increased serum alkaline phosphatase (from duct damage). risk of rupture if untreated.

Question 36

What is chronic cholecystitis and what is it due to??

Answer 36

Chronic inflammation of the gallbladder. Due to chemical irritation from longstanding cholelithiasis, with or without superimposed bouts of acute cholecystitis.

Question 37

What is Chronic cholecystitis characterized by? and how does it present?

Answer 37

Characterized by herniation of gallbladder mucosa into the muscular wall (rokitansky-aschoff sinus). Presents with vague RUQ pain, especially after eating.

Question 38

What is a late complication of chronic cholecystitis? What does it increase risk for?

Answer 38

Porcelain gallbladder. Shrunken, hard gallbladder due to chronic inflammation, fibrosis, and dystrophic calcification. Increased risk for carcinoma

Question 39

What is treatment of chronic cholecystitis?

Answer 39

Cholecystectomy, especially if porcelain gallbladder is present.

Question 40

What is ascending cholangitis and what is it usually due to?

Answer 40

Bacterial infection of the bile ducts. usually due to ascending infection with enteric gram negative bacteria from bowel.

Question 41

How does ascending cholangitis present and what other disease shows increased incidence of ascending cholangitits?

Answer 41

Presents as sepsis (high fever and chills), jaundice, and abdominal pain. Increased incidence with choledocholithiasis (stone in the biliary ducts)

Question 42

What is gallstone ileus and what is it due to?

Answer 42

Gallstone enters and obstructs the small bowel. Due to cholecystitis with fistula formation between the gallbladder and small bowel.

Question 43

What is gallblader carcinoma? what does it arise from, what is the major risk factor?

Answer 43

Adenocarcinoma arising from the glandular epithelium that lines the gallbladder. Gallstones are a major risk factor, especially when complicated by porcelain gallbladder.

Question 44

How does gallbladder carcinoma present and what is the prognosis?

Answer 44

Classically presents as cholecystitis in an ELDERLY woman. Poor prognosis

Question 45

What is jaudnice and what is it due to and when does it arise?

Answer 45

Yellow discoloration of the skin; earliest sign is scleral icterus. Due to increased serum bilirubin, usually >2.5 mg/dL. Arises with disturbances in bilirubin metabolism

Question 46

What are the steps of bilirubin metabolism (7)?

Answer 46

1 RBCs are consumed by macrophages of the RES 2 protoporphyrin (from heme) is converted into unconjugated bilirubin (UCB). 3 albumin carries UCB to liver 4 Uridine glucuronyl transferase (UGT) in hepatocytes conjugates bilirubin 5 Conjugated bilirubin (CB) is transferred to bile canaliculi to form bile, which is stored in the gallbladder 6 Bile is released into the small bowel to aid in digestion 7 intestinal flora convert CB to urobilinogen, which is oxidized to stercobilin (makes stool brown) and urobilin (partially reabsorved into blood and filtered by kidney, making urine yellow)

Question 47

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of extravascular hemolysis or ineffective erythropoiesis?

Answer 47

Etiology: High levels of UCB overwhelm the conjugating ability of the liver. Laboratory Findings: increased UCB Clinical Features: Dark urine due to increased urine urobilinogen (UCB is not water soluble, and this is absent from urine) Increased risk for pigmented bilirubin gallstones

Question 48

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of Physiologic jaundice of the newborn?

Answer 48

Etiology: newborn liver has transiently low UGT activity. Laboratory findings: Increased UCB Clinical Features: UCB is fat soluble and can deposit in the basal ganglia (kernicterus) leading to neurological deficits and death. Treatment is phototherapy (makes UCB more water soluble)

Question 49

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of Gilbert syndrome?

Answer 49

etiology: mildly low UGT activity; AR Laboratory findings: Increased UCB Clinical features: Jaundice during stress (e.g. severe infection); otherwise, not clinically significant.

Question 50

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of Crigler-najjar syndrome

Answer 50

etiology: absence of UGT laboratory findings: increased UCB Clinical Features: Kernicterus; usually fatal

Question 51

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of Dubin-Johnson syndrome?

Answer 51

Etiology: deficiency of bilirubin canalicular transport protein; AR Laboratory findings: increased CB Clinical features: Liver is dark; otherwise, not clinically significant.

Question 52

What is Rotor syndrome?

Answer 52

Similar to Dubin-Johnson syndrome, but lacks liver discoloration

Question 53

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of biliary tract obstruction (obstructive jaundice)?

Answer 53

Etiology: associated with gallstones, pancreatic carcinoma. cholangiocarcinoma, parasites, and liver fluke (clonorchis sinensis). Laboratory Findings: Increased CB (water soluble), decreased urine urobilinogen, and increased alkaline phosphatase Clinical Features: Dark urine (due to bilirubinuria) and pale stool. pruritus due to increased plasma bile acid deposits in skin. Hypercholesterolemia with xanthomas. steatorrhea with malabsorption of fat soluble vitamins.

Question 54

Wrt to jaundice, what is the etiology, laboratory findings and clinical features of Viral hepatitis?

Answer 54

Etiology : Inflammation disrupts hepatocytes and small bile ductules. Laboratory Findings: Increase in both CB and UCB Clinical Features: Dark urine due to increased urine bilirubin; urine urobilinogen is normal or decreased.

Question 55

REVIEW TABLE 11.2 on page 119

Answer 55

REVIEW TABLE 11.2 on page 119

Question 56

REVIEW TABLE 11.3 on page 120

Answer 56

REVIEW TABLE 11.3 on page 120

Question 57

What is viral hepatitis and what two types of hepatitis may result? What causes it?

Answer 57

Inflammation of liver parenchyma, usually due to hepatitis virus; other causes include EBV and CMV. Hepatitis virus causes acute hepatitis, which may progress to chronic hepatitis.

Question 58

How does the hepatitis virus cause damage?

Answer 58

Viral antigen is presented on hepatocytes by MHC class 1 and then recognized by CD8 T cells.

Question 59

How does acute hepatitis present?

Answer 59

Jaundice (mixed UCB and CB) with dark urine (due to CB), fever, malaise, nausea, and elevated liver enzymes (ALT>AST)

Question 60

Describe the inflammation in acute hepatitis? and describe how long symptoms last?

Answer 60

Inflammation involves lobules of the liver and portal tracts and is characterized by apoptosis of hepatocytes. Some cases may be asymptomatic with elevated liver enzymes. Symptoms last less than 6 months.

Question 61

What is chronic hepatitis characterized by and what does the inflammation involve and what is it at risk of progressing to?

Answer 61

Characterized by symptoms that last longer than 6 months. Inflammation predominantly involves portal tract and has the risk of progression to cirrhosis

Question 62

What is Cirrhosis?

Answer 62

End stage liver damage characterized by disruption of the normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes.

Question 63

What is fibrosis mediated by in cirrhosis?

Answer 63

mediated by TGF-Beta from stellate cells which lie beneath the endothelial cells that line sinusoids.

Question 64

What are 3 clinical features of Cirrhosis?

Answer 64

Portal hypertension Decreased detoxification Decreased protein synthesis

Question 65

What doe portal hypertension lead to in cirrhosis? (4)

Answer 65

1 Ascites (fluid in the peritoneal cavity) 2 Congestive splenomegaly/hypersplenism 3 portosystemic shunts (esophagela varices, hemorrhoids, and caput medusae) 4 Hepatorenal syndrome (rapidly developing renal failure secondary to cirrhosis)

Question 66

What does decreased detoxification in cirrhosis result in? (3)

Answer 66

1 Mental status changes, asterixis, adn eventual coma (due to increased serum ammonia); metabolic, hence reversible 2 gynecomastia, spider angiomata, and palmar erythema due to high estrogen levels 3 Jaundice

Question 67

What does decreased protein synthesis in cirrhosis lead to? (2)

Answer 67

1 hypoalbuminemia with edema | 2 coagulopathy due to decreased synthesis of clotting factors; degree of deficiency is followed by PT

Question 68

What is Alcohol related liver disease? what does it result in?

Answer 68

Damage to hepatic parenchyma due to consumption of alcohol. Most common cause of liver disease in the west. Fatty liver is the accumulation of fat in hepatocytes. results in a heavy, greasy liver; resolves with abstinence.

Question 69

What does Alcohol related liver disease result from? what is it characterized by. and how does it present?

Answer 69

Chemical injury to hepatocytes. 1 Acetaldehyde (metabolite of alcohol) mediates damage 2 Characterized by swelling of hepatocytes with formation of mallory bodies (damaged cytokeratin filaments), necrosis and acute inflammation 3 Presents with painful hepatomegaly and elevated liver enzymes (AST>ALT) because AST in mitochondria and EtOH is a mitochondrial poison. may result in death

Question 70

What is a complication of long-term. chronic alcohol induced liver damage?

Answer 70

Cirrhosis occurs in 10-20% of alcoholics

Question 71

What is nonalcoholic fatty liver disease? what is it associated with? and how is a diagnosis made?

Answer 71

Fatty change, hepatitis, and/or cirrhosis that develop without exposure to alcohol (or other known insult). Associated with obesity. Diagnosis of exclusion ALT>AST

Question 72

What is hemochromatosis and what mediates tissue damage in it?

Answer 72

Excess body iron leading to deposition in tissues (hemosiderosis) and organ damage (hemochromatosis). Tissue damage is mediated by generation of free radicals.

Question 73

What is hemochromatosis due to? Primary? Secondary?

Answer 73

AR defect in the iron absorption (primary) or chronic transfusions (secondary)

Question 74

What is primary hemochromatosis due to and what role do enterocytes play in iron regulation?

Answer 74

Primary hemochromatosis is due to mutations in the HFE gene, usually C282Y (cysteine is replaced by tyrosine at amino acid 282) Enterocytes take up almost all iron in our diet but only release it into blood if it is needed. Dysregulation in this process results in too much iron released into the blood.

Question 75

When does hemochromatosis present?

Answer 75

Late adulthood

Question 76

What is the classic triad of hemochromatosis and what are some other symptoms?

Answer 76

triad: cirrhosis, secondary diabetes mellitus, and bronze skin. Other findings: dilated cardiomyopathy, cardiac arrythmias and gonadal dysfunction (due to testicular atrophy)

Question 77

What doe the following labs show in hemochromatosis? | ferritin, TIBC, serum iron, adn % saturation?

Answer 77

Ferritin is high TIBC is low Serum iron is high % saturation is high

Question 78

What does liver biopsy show in hemochromatosis?

Answer 78

Reveals accumulation of brown pigment in hepatocytes. Prussian blue distinguishes between iron (blue) and liopfuscin.

Question 79

What is lipfuscin?

Answer 79

Brown pigment that is a by-product from the turnover ('wear and tear') of peroxidized lipids; it is commonly present in hepatocytes.

Question 80

What does hemochromatosis carry and increased risk for and what is the treatment?

Answer 80

increased risk for Hepatocellular carcinoma and treatment is phlebotomy.

Question 81

What is defective in Wilson disease, what is the inheritance pattern, what does this result in and how does this cause damage?

Answer 81

AR defect (ATP7B gene) in ATP mediated hepatocyte copper transport. Results in a lack of copper transport into bile and lack of copper incorporation into ceruloplasmin. Copper builds up in hepatocytes, leaks into the serum, and deposits in tissues. Copper mediated production of hydroxyl free radicals leads to tissue damage.

Question 82

how does wilson disease present and when?

Answer 82

Presents in childhood with: 1 Cirrhosis 2 Neurologic manifestations (behavioral changes, dementia, chorea, and parkinsonian symptoms due to deposition of copper in basal ganglia 3 Kayser Fleisher rings in the cornea

Question 83

What do the following lab values show in Wilson disease? | urinary copper, serum ceruloplasmin, and copper on liver biopsy?

Answer 83

urinary copper increased serum ceruloplasmin decreased copper on liver biopsy increased

Question 84

What does Wilson disease carry an increased risk for? what is the treatment?

Answer 84

Increased risk of hepatocellular carcinoma. Treatment is D-Penicillamine (chelates copper)

Question 85

What is primary biliary cirrhosis, who does it arise in and when, and what is it associated with?

Answer 85

Autoimmune granulomatous destruction of intrahepatic bile ducts. Classically arises in women (average age is 40 years). Associated with other autoimmune diseases.

Question 86

What is the etiology of primary biliary cirrhosis? what antibody is present?

Answer 86

Etiology is unknown; antimitochondrial antibody is present and helps mark the disease

Question 87

How does primary biliary cirrhosis present and what is a late complication?

Answer 87

Presents with features of obstructive jaundice. Cirrhosis is a late complication

Question 88

What is primary sclerosing cholangitis? What is seen on contrast imaging and on histologic examination?

Answer 88

Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. Periductal fibrosis with an 'onion-skin' appearance on histology. Univolved regions are dilated resulting in a 'beaded' appearance on contrast imaging

Question 89

What is the etiology of Primary sclerosing cholangitis and what is it associated with and what is a histologic marker?

Answer 89

Etiology is unknown, but associated with ulcerative colitis; p-ANCA is often positive.

Question 90

How does primary sclerosing cholangitis present, what is a late complication, and what does it pose and increased risk for?

Answer 90

Presents with obstructive jaundice; cirrhosis is a late complications. Increased risk for cholangiocarcinoma.

Question 91

What is Reye syndrome and what is it likely related to?

Answer 91

Fulminant liver failure and encephalopathy in children with viral illness who take aspirin. Likely related to mitochondrial damage of hepatocytes.

Question 92

How does Reye syndrome present?

Answer 92

Presents with hypoglycemia, elevated liver enzymes, and nausea with vomiting; may progress to coma and death.

Question 93

What is a hepatic adenoma and what is it associated with?

Answer 93

Benign tumor of hepatocytes. Associated with oral contraceptive use; regresses upon cessation of drug.

Question 94

What risks do hepatic adenomas carry and describe where they grow and what they respond to?

Answer 94

Risk of rupture and intraperitoneal bleeding, especially during pregnancy. Tumors are subcapsular and grow with exposure to estrogen.

Question 95

What is hepatocellular carcinoma and what are 3 risk factors?

Answer 95

Malignant tumor of hepatocytes. Risk factors: 1 Chronic hepatitis (e.g. HBV and HCV) 2 Cirrhosis (e.g. alcohol, nonalcoholic fatty liver disease, hemochromatosis, wilson disease, and A1AT deficiency) 3 Aflatoxins derived from aspergillus (induce p53 mutations)

Question 96

What complication does hepatocellular carcinoma carry a risk for? explain it.

Answer 96

Budd Chiari syndrome. liver infarction secondary to hepatic vein obstruction. Presents with painful hepatomegaly and acites.

Question 97

When are hepatocellular carcinomas detected and what is the prognosis?

Answer 97

Tumors are often detected late because symptoms are masked by cirrhosis; poor prognosis.

Question 98

What is the serum tumor marker for hepatocellular carcinoma?

Answer 98

AFP

Question 99

How common are metastases to the liver and what are 4 common sources?

Answer 99

More common than primary liver tumors; most common sources include colon, pancreas, lung, and breast carcinomas.

Question 100

What do metastases in the liver result in and how may they be detected clinically?

Answer 100

Results in multiple nodules in the liver. May be detected as hepatomegaly with a nodular free edge of the liver.

Question 101

How does hypercalcemia cause acute pancreatitis?

Answer 101

Calcium is an activator of enzymes

Question 102

How does alcohol cause acute pancreatitis?

Answer 102

Alcohol causes contraction of the sphincter of oddi which leads to impeded drainage of enzymes