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Exocrine Pancreas, Gallbladder, and Liver Pathology
Pathoma and Goljan Path Shelf Review
32
Pathology
Graduate
04/04/2012

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Cards

Term
Annular Pancreas
Definition

Developmental malformation in which the pancreas forms a ring around the duodenum.

 

Risk of duodenal obstruction

Term
Acute Pancreatitis
Definition
  • Inflammation and hemorrhage ofthe pancreas
  • Due to autodigestion of pancreatic parenchyma by pancreatic enzymes
    • Premature activation of trypsin leads to activation of other pancreatic enzymes
  • Results in liquefactive hemorrhagic necorsis of the pancreas and fat necrosis of the peripancreatic fat
  • Most commonly due to alcohol and gallstones
  • Other causes include trauma, hypercalcemia, hyperlipidemia, drugs, scorpion stings, umps, and ruptue of a posterior duodenal ulcer
  • Clinical Features:
    • Epigastric abdominal pain that radiates to the back
    • Nausea and vomiting
    • Periumbilical and flank hemorrhage- necrosis spreads into the periumbilical soft tissue and retroperitoneum 
    • Elevated serum lipase and amylase: lipase is more specific for pancreatic damge
    • Hypocalcemia: calcium is consumed during fat necrosis
  • Complications:
    • Shock: Due to peripancreatic hemorrhage and fluid sequestration
    • Pancreatic Pseudocyst: 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
    • Pancreatic abscess: often due to E coli
      • Presents with abdominal pain
      • High Fever
      • Persistently elevated amylase
    • DIC and ARDS
Term
Chronic Pancreatitis
Definition
  • Fibrosis of pancreatic parenchyma most often secondary to actue pancreatitis
  • MC due to alcohol in adults and CF in children
  • Many cases are idiopathic
  • Clinical Features:
    • Epigastric abdominal pain that radiates to the back
    • Pancreatic insufficiency- results in malabsorption with steatorrhea and fat soluble deficiencies
    • Amylase and lipase are not useful serologic markers of chronic pancreatitis
    • Dystrophic calification of pancreatic parenchyma on imaging contrast studies reveal a "chain of lakes" pattern due to dilation of pancreatic ducts
    • Secondary to diabetes- late complication due to destruction of islets
    • Increased risk for pancreatic carcinoma  
Term
Pancreatic Carcinoma
Definition
  • Adenocarcinoma arising from the pancreatic ducts
  • Most commonly seen in the elderly-avg age is 70
  •  Major risk factors are smoking and chronic pancreatitis
  • Epigastric abdominal pain
  • Weight loss
  • Obstructive jaundice with pale stools andPalpable gallbladder are associated with tumors that arise in the head of the pancreas
    • MC location
  • Secondary diabetes mellitus is assocaited with tumors that arise in the body or tail
  • Pancreatitis
  • Migratory thrombophlebitis (Trousseau sign): Presents as swelling, erythema, and tenderness in the extremities (seen in 10% of patients)
  • Serum tumor marker is CA 19-9
  • Surgical resection
  • Very Poor prognosis: 1-year survival is < 10%
Term
Biliary Atresia
Definition
  • Failure to form or early destruction of extrahepatic biliary tree
  • Leads to biliary obstruction within the first 3 months of life
  •  Presents with jaundice and progresses to cirrhosis
Term

Cholelithiasis

Gallstones

Definition
  • Solid round stones in the gallbladder
  • Due to precipitation of cholesterol or bilirubin in bile
  • Arises with supersaturation of cholesterol or bilirubin
  • Arises with decreases phospholipids (lecithin) or bile acids
    • Bile Acids normally increase solubility
  • Arises with stasis
  • Cholesterol stones are yellow and the most common type (90%) 
    • Radiolucent (10% are radioopaque due to association with calcium)
    • Risk factorse include: age (40s), estrogen (female gender, obesity, multiple pregnancies, and oral contraceptives), Native American ethnicity, Crohns disease, and cirrhosis
  • Bilirubin Stones (pigmented) 
    • Usually radioopaque
    • Risk factors include extravascular hemolysis and biliary tract infection (E coli, Ascaris lumbrioides, Clonorchis sinensis)
    • Ascaris lumbricoides: common roundworm that infects 25% of the worlds population, especially in areas with poor sanitation.  Fecal oral transmission.  Infects the biliary tract increasing the risk for gallstones
    • Clonorchis sinensis: Endemic in China, Korea, and Vietnam. Called the Chinese liver fluke.  Infects the biliary tract increasing the risk for gallstones, cholangitis, and cholangiocarcinoma
  • Gallstones are usually asymptomatic
  • Complication include: biliary colic, acute and chronic cholecystitis, ascending cholangitis, gallstone ileus, and gallbladder cancer  
Term
Biliary Colic
Definition
  • Waxing and waning RUQ pain
  • Due to the 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  
Term
Acute Cholecystitis
Definition
  • Acute inflammation of the gallbladder wall
  • Impacted stone in the cystic duct results in dilation with pressure ischemia, bacterial overgrowth ( E coli) and inflammation
  • Presents with RUQ pain, often radiating to the right scapula
  • Fever
  • Increased WBC count
  • Nausea
  • Vomiting
  • Increased serum Alkaline phosphatse
  • Risk of rupture if left untreated  
Term
Chronic Cholecystitis
Definition
  • Chronic inflammation of the gallbladder
  • Due to chemical irritation from longstanding cholelithiasis with or without superimposed bouts of acute cholecystitis
  • Characterized by herniation of gallbladder mucosa into the muscular wall  called Rokitansky-Aschoff sinus
  • Presents with vague RUQ pain, especially after eating
  • Porcelain gallbladder is a late complication
    • Shrunken, hard gallbladder due to chronic inflammation, fibrosis, and dystrophic calcification
    • Increased risk for carcinoma
  • Treatment is cholecystectomy especially if porcelain gallbladder is present
Term
Ascending Cholangitis
Definition
  • Bacterial infection of the bile ducts
  • Usually due to ascending infection with enteric Gm (-) bacteria
  • Presents as sepsis (high fever and chills), jaundice and abdominal pain
  • Increased incidence with choledocholithiasis (stone in biliary duct)  
Term
Gallstone Ileus
Definition
  • Gallstone enters and obstructs the small bowel
  • Due to cholecystitis with fistula formation between the gallbladder and small bowel 
Term
Gallbladder Carcinoma
Definition
  • Adenocarcinoma arising from the glandular epithelium that lines the gallbladder wall
  • Gallstones area major risk factor, especially when complicated by porcelain gallbladder
  • Classically presents as cholecystitis in an elderly woman
  • Poor prognosis  
Term
Normal Bilirubin Metabolism
Definition
  • RBCs are consumed by macrophages of the reticuloendothelial system  
  • Protoporphyrin (from heme) is converted to unconjugated bilirubin
  • Albumin carries unconjugated bilirubin to the liver
  • Uridine glucuronyl transferase in hepatocytes conjugates bilirubin
  • Conjugated bilirubin is transferred to the bile canaliculi to form bile which is stored in the gallbladder
  • Bile is released into the small bowel to aid in digestion
  • Intestinal flora converts conjugated bilirubin to urobilinogen which makes the stool brown. 
  • Urobilinogen is also partially reabsorbed into the blood and filtered by the kidney, making urine yellow  
Term

Paravascular hemolysis

or

 Ineffective erythropoiesis

as a casue of Jaundice

Definition
  • High Levels of unconjugated Bilirubin (UCB) overwhelm the conjugating ability of the liver
  • Increased UCB on lab findings
  • Dark urine due to increased urine urobilinogen
  • UCB is not water soluble and this is absent from urine
  • Increased risk for pigmented bilirubin gallstones
Term
Physiologic Jaundice of the Newborn
Definition
  • Newborn liver has transiently low uridine glucuronyl transfease (UGT) activity
  • Increased UCB on lab findings
  • UCB is fat soluble and can deposit in the basal ganglia causing kernicterus leading to neurological deficits and death
  • Treatment is phototherapy which makes UCB water soluble
Term
Gilbert Syndrome
Definition
  • Midly low UGT activity
  • AR
  • Increased UCB
  • Jaundice during stress (severe infections) otherwise not clinically significant  
Term
Crigler-Najjar Syndrome
Definition
  • Absence of UGT
  • Increased UCB on lab findings
  • Kernicterus-UCB deposited in basal ganglia
  • Usually fatal

 

Term
Dubin Johnson Syndrome
Definition
  • Deficiency of bilirubin canalicular transport protein
  • AR
  • Increased conjugated bilirubin
  • Liver is dark
  • Not clinically significant
  • Rotor syndrome is similar to Dubin Johnson syndrome but lacks the liver discoloration  
Term

Biliary Tract Obstruction

Obstructive Jaundice

Definition
  • Associated with gallstones, pancreatic carcinoma, cholangiocarcinoma, parasites, and liver fluke
  • Increased Conjugated bilirubin, increased urine urobilinogen, and increased alkaline phosphatase
  • Dark Urine-due to increased uring urobilinogen
  • Pale stool
  • Pruritis due to increased plasma bile acid
  • Hyprcholesterolemia with xanthomas
  • Steatorrhea with malabsorption of fat souble vitamins 
Term
Viral Hepatitis
Definition
  • Inflammation disrupts hepatocytes and small bile ductules
  • Increase in both conjugated and unconjugated bilirubin
  • Dark urine due to increased urine bilirubin
  • Urine urobilinogen is normal or decreased
  • HAV: 
    • Fecal oral transmission
    • Acute hepatitis
    • No Chronic state
    • Anti-IgM marks current infection
    • Anti-IgG is protective and indicated prior infection or immunization
  • HEV:
    • Acquired from contaminated water ot undercooked seafood
    • Pregnant women
    • Associated with fulfminant hepatits (liver failure with massive liver necrosis)
  • HBV:
    • Parenteral transmission (childbirth, unprotected intercourse, and IV drug use)
    • Results in acute hepatitis 80% of cases
  • HCV:
    • Parenteral transmission
    • Risk from transmission is almost nonexistant due to screening of the blood supply
    • Chronic Disease in most cases
    • HCV-RNA test confirms infection
    • Decreased RNA levels indicate recovery
    • Persistence indicates chronic disease
  •  HDV:
    • Dependeent on HBV for infection
    • Superinfection upon existing HBV is more severe than coinfection with HBV
Term
Viral Hepatitis
Definition
  • Inflammation of the liver parenchyma
  • Causes acute hepatitis which may progress to chronic hepatitis
  • Acute hepatitis presents as jaundice (mixed CB and UCB) with dark urine (due to CB), fever, malaise, nausea, and elevated liver enzymes (ALT>AST)
    •  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 < 6 months
  • Chronic hepatitis is characterized by symptoms that last > 6 months
    • Inflammation predominatly involves portal tract
    • Risk of progression to cirrhosis
Term
Cirrhosis
Definition
  • End-stage liver damage characterized by disruption of the normal hepatic parenchyma by bands of fibrosis and regenerative nodules of hepatocytes
  • Fibrosis is mediated by TGF-β from stellate cells which lie beneath the endothelial cells the line the sinusoid
  •  Portal hypertension leads to:
    • Ascites
    • Congestive splenomegaly
    • Portosystemic shunts- Esophageal varices, hemorrhoids, caput medusae (engorged paraumbilical veins)
    • Hepatorenal syndrome-rapidly developing renal failure
  • Decreased detoxification results in:
    • Mental status change, asterixis, and eventual coma due to increased ammonia
    • Gynecomastia
    • Spider Angioma
    • Palmar erythema
    • Jaundice
  • Decreased protein synthesis leads to:
    • Hypoalbuminemia with edema
    • Coagulopathy due to decreased synthesis of clotting factors
Term
Alcohol Related Liver Disease
Definition
  • Damage to hepatic parenchyma due to consumption of alcohol
  • MCC of liver disease in West
  • Fatty Liver is the accumulation of fat n hepatocytes
    • Results in a heavy, greasy liver, resolves with abstinence
  • Alcoholic hepatitis results from chemical injury to hepatocytes
    • Seen with binge drinkning
    • Acelaldehyde mediates damage
    • Characterized by swelling of hepatocytes with formation of Mallory bodies (damaged cytokeratin filaments)
    • Necrosis
    • Acute inflammation
    • Presents with painful hepatomegaly and elevated liver enzymes (AST>ALT)
    • May result in death
  • Cirrhosis is a complication of long term, chronic alcohol0induced liver damage
    • Occurs in 10-20% of alcoholics  
Term
Nonalcoholic Fatty Liver Disease
Definition
  • Fatty change, hepatitis, and/or cirrhosis that develop without exposure to alcohol
  • Associated with obesity
  • Diagnosis of exclusion
  • (ALT>AST) 
Term
Hemochromatosis
Definition
  • Excess body iron leading to deposition in tissues (hemosiderosis) and organ damage
  • M>W
  • If present in women not diagnoised until postmenopausal
  • Tissue damage is mediated by generation of free radicals
  • Due to AR defect in iron absorption (primary) or chronic transfusions (secondary)
  • Primary hemochromatosis is due to mutations in the HFE gene, usually C282Y on chromosome 6
    • Cysteine is replaced by tyrosine at amno acid 282
  • Presents in late adulthood
  • Classic triad:
    •  Cirrhosis
    • Secondary Diabetes mellitus
    • Bronze skin
  • Other findings are:
    • Cardiac arrhythmias
    • Gonadal dysfunction (due to testicular atrophy)
  • Increased serum iron-used to follow therapy
  • Increased % sat
  • Increased ferritin
  • Transferrin saturation is the best screening test: Values >45% indicate further testing is necessary
  • Decreased TIBC
  • Liver biopsy reveals accumulation of brown pigment in hepatocytes
  • Prussan blue stain distinquishes iron (blue) from lipofuscian (brown pigment that is a by-product from the turn over of peroxidized lipids.  Commonly present in hepatocytes)
  • Increased risk of cellular carcinoma
  • Treatment is phlebotomy
Term
Wilson's Disease
Definition
  • AR
  • ATP7B gene
  • Defective hepatocyte transport of copper into bile for excretion
  • Defective incorporation of copper into ceruloplasmin (binding protein for copper in blood)
  • Copper builds up in hepatocytes, leaks into serum, and deposits into tissues
  • Copper mediated production of hydroxy free radicals which leads to tissue damage
  • Onset of symptoms is 3-40 yrs old
  • Cirrhosis
  • Neurologic manifestation: behavioral changes, dementia, chorea, and Parkinsonian symptoms due to deposition of copper in the putamen
  • Copper deposits in the subthalamic nucleus produces hemiballismus
  • Copper is toxic to the neurons in the cerebral cortex and produces dementia
  • Kayser-Fleisher rings in the cornea
  • Decreased total serum copper-due to decreased ceruloplasmin
  • Decreased serum ceruloplasmin
  • Increasd serum and urine free copper
  • Increased risk for hepatocellular carcinoma
  • Treatment is D-penicillamine (chelates copper)
Term
Primary Biliary Cirrhosis
Definition
  • Autoimmune granulomatous destruction of intrahepatic bile ducts
  • Classically arises in women of avg age 40
  • Associated with other autoimmune disease
  • Etiology is unknown
  • Anti-mitochondrial antibody is present
  • Presents with features of obstructive jaundice
  • Cirrhosis is a late complication  
Term
Primary Sclerosing Cholangitis
Definition
  • Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts
  • Periductal fibrosis with an onion skin appearance
  • Ininvolved regions are dilated resulting in a beaded apearance on contrast imaging
  • Etiology is unknown but associated withulcerative colitis
  • p-ANCA is often present
  •  Presents with obstructive jaundice, cirrhosis is a late complication
  • Increased risk for cholangiocarcinoma
Term
Reyes Syndrome
Definition
  • Fulminant liver faiure and encephalopathy in children with viral illness who take asprin
  • Likely related to mitochondrial damage of hepatocyte
  • Presents with hypoglycemia, elevated liver enzymes, and nausea with vomiting
  • May progress to coma and death  
Term
Hepatic Adenoma
Definition
  • Benign tumor of hepatocytes
  • Associated wtih oral contraceptives: regresses with cessation of drug
  • Risk of rupture and intraperitoneal bleeding, especially during pregnancy
  • Tumors are subcapsular and grow with exposure to estrogen  
Term
Hepatocellular Carcinoma
Definition
  • Malignant tumor of hepatocytes
  • Risk Factors include:
    • Chronic hepatitis
    • Cirrhosis
    • Fatty liver disease
    • Hemochromatosis
    • Wilsons disease
    • AIAT dificiency
    • Anatoxins derived from Aspergillus (p53 mutations)
  • Increased risk for Budd-Chiari syndrome
    • Liver infarction secondary to hepatic vein obstruction
    • Presents with painful hepatomegaly and ascites
  • Tumors are often detected late because symptoms are masked by cirrhosis: poor prognosis
  • Serum tumor marker is alpha-fetoprotein  
Term
Metastasis to Liver
Definition
  • More common than primary liver tumors
  • MC sources include colon, pancreas, lung, and breast carcinoma
  • Results in multiple nodules in the liver
  • Clinically may be detected as hepatomegaly wiht a nodular free edge of the liver  
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