Exocrine Pancreas, Gallbladder, and Liver Flashcards Preview

Pathoma > Exocrine Pancreas, Gallbladder, and Liver > Flashcards

Flashcards in Exocrine Pancreas, Gallbladder, and Liver Deck (41):

Annular Pancreas

Developmental malformation where the pancreas forms a ring and obstructs the duodenum


Acute Pancreatitis
pathophysiology and causes

-Inflammation and hemmorhage of the pancrease due to premature activation of TRYPSIN which activates other enzymes still in the pancreas and results in autodigestion
-Get liquefactive and hemmorhagic necrosis of pancreas and fat necrosis of the peripancreatic fat
- Usually due to alcohol (stimulates contraction of sphincter of oddi) and gallstones (block ampulla)
-See in trauma (automobil accident in buckled child), hypercalcemia (activates enzymes), hyperlipidemia, drugs, scorpion stings, mumps, rupture of posterior duodenal ulcer


Acute Pancreatitis clinical features and complications

Epigastric pain that radiates to the BACK
Nausea and vommitting
Periumbilical and flank hemmorhage
elevated serum LIPASE and amylase
Hypocalcemia (Ca2+ consumed during saponification in fat necrosis)
Complications include
1) Shock
2) Pancreatic Pseudocyst = fibrous tissue arround liquefactive necrosis. presents as palpable abdominal mass persistently elevated amylase. Rupture can release enzymes into abdominal cavity and cause hemmorhage
3) Pancreatic Abscess = usually due to E Coli. Get ab pain, fever, and persistenly elevated serum amylase
4) DIC and ARDS (enzymes in blood and can reach the lung)


Chronic Pancreatitis

Fibrosis of pancreatic parenchyma usually due to reccurent bouts of pancreatitis in alcoholics and CF (thick secretions)
Clinical features
1) Epigastric pain that RADIATES to the BACK
2) Pancreatic Insufficiency = malabsorption, steatorrhea, and deficiency of fat soluable vitamins
3) Dystrophic Calcification of parenchyma presents as "chain of lakes" due to dilation of pancreatic ducts
4) Can cause secondary DM if there is destruction of islets
5) Increased risk for pancreatic carcinoma


Pancreatic Carcinoma

Adenocarcinoma arising from the DUCTS usually seen in elderly
Risk factors = smoking and chronic pancreatitis
Clinical features
1) Epigastric abdominal pain and weight loss
2) Obstructive jaundice with PALE STOOLS and PALPABALE GALLBLADDER if tumor is in HEAD of pancreas
3) Can get secondary DM if tumor is in body or tail
4) Migratory thrombophlebitis = trousseau sign = swelling, erythema, and tenderness in extremities
Serum tumor marker is CA 19-9
Need to do whipple procedure = en bloc removal of head and neck of pancreas, proximal duodenum, and gallbladder
1 year survival <10%


Biliary Atresia

Failure to form or early destruction of extrahepatic biliary tree
Leads to biliary obstruction in first 3 months of life
Presents with jaundice (conjugated bilirubin) that progresses to cirrhosis



Gallstones = solid round stones in the gallbladder
Due to precipitation of cholesterol or bilirubin in bile
arise when
1) supersaturation of of cholesterol or bilirubin
2) decreased phopsholipids or bile acids that normally increase the solubility of cholesterol and bilirubin
-colestyramine is a lipid lowering drug that binds bile acids
3) stasis
Usually asymptomatic but complications include biliary colic, acute/chronic cholecystitis, ascending cholangitis, gallstone illeus, gallbladder cancer


Cholesterol Stones

radiolucent usually
Risk factors
1) 40s to 50s
2) estrogen (female gender, obesity, multiple pregnancies, and oral contraceptives) = icreases HMGCoaA reductase activity leading to increased cholesterol synthesis. Also increases lipoprotein receptors on hepatocytes increasing cholesterol uptake
3) clofibrate = lipid lowering drug that increases HMGCoaA reductase activity leading to increased cholesterol synthesis. Also decreased conversion of cholesterol to bile acids
4) Native American ethnicity
5) Chrohn Disease = affects terminal illeum where bile salts and acids are normally taken up
6) Cirrhosis = decreased production of bile salts by hepatocytes


Bilirubin Stones

Risk Factors include
1) Extravascular hemolysis = increased unconjugated bilirubin in bile
2) Biliary tract infection by E. coli
3) Biliary tract infection by Ascaris lumbricoides = roundowrm that infects biliary tree
4) Biliary tract infection by Clonorchis sinensis = liver fluke in CHina, Korea, and Vietnam that infects biliary tree (also increases risk of gallstones, cholangitis, and cholangiosarcoma)


biliary colic

Waxing and waning right upper quadrant pain as the gallbladder contracts against a stone lodged in the cystic duct (relieved if stone passes)
If stone is lodged in the common bile duct then can get acute pancreatitis or obstructive jaundice


acute cholecystitis

Acute inflammation of the gallbladder wall
impacted stone in cystic duct results in dilation with pressure ischemia and bacterial overgrowth and inflammation
Present with right upper quadrant pain that RADIATES TO R SCAPULA, fever, nausea, vomiting, increased WBC and serum alkaline phosphatase
Risk of rupture if untreated


chronic cholecystitis

Chronic inflammation of the gallbladder due to chemical irritation of longstanding cholelithiasis
see rokitansky-aschoff sinus = herniation of gallbladder mucoa into muscular wall
presents with vague upper right quadrant pain, especially after eating
Porcelain gall bladder is late complication = shrunken, hard, fibrosis, dystrophic calcification
treatment = cholecystectomy


ascending cholangitis

bacterial infection of the bile ducts with enteric gram negative bacteria
Presents as jaundice , ab pain, and spsis (fever and hcills)


gallstone illeus

Cholecystitis causes fistula between gallbladder and small bowel allowing stones to pass directly into small bowel and obstruct them


gallbladder carcinoma

Adenocarcinoma arising from GLANDULAR EPITHELIUM that lines the gallbladder wall
Gallstones are major risk factor (especially if get porcelain gallbladder)
presents as cholecystitis in ELDERLY women (this is unusual cus cholecystitis typically presents in 40s-50s)



Yellow discoloration of the skin
earliest sign is scleral icterus (yellowing sclera)
Due to increased serum bilirubin usually >2.5mg/dL when have messed up bilirubin metabolism


Normal Bilirubin metabolism

1) RBS are consumed by macrophages of reticuloendothelial system
2) Protoporphyrin is converted into UnConjugated Bilirubin (UCB)
3) Albumin carries UCB to liver
4) Uridine Glucuonyl Transferase (UGT) in hepatocytes conjugates the bilirubin (CB)
5) CB is transferred to bile canniculi by a protein to form protein
6) Bile is stored in the gallbladder
7) Bile is released into duodenum to aid in digestion
8) Intestinal flora convert CB to urobilinogen
9) Urobilinogen is oxidized to stercobilin (makes poop brown) and urobilin (partially reabsorbed into blood and filtered by kidney to make urine yellow)
-Note UCB is not water soluable and therefore not present in urine


Extravascular Hemolysis or Inneffective Erythropoiesis

Extremely high levels of UCB overwhelm the conjugating ability of the liver
get increased serum UCB giving rise to jaundice
Clinically get dark urine due to increased urine urobilinogen
Increased risk of pigmented bilirubin gallstones due to excess bilirubin being made


Physiologic jaundice of the newborn

Newborn liver has transiently low Uridine Glucuonyl Transferase activity and so can't conjugate the normal amount of UCB that is presented to it.
UCB builds up so see increase in teh serum and get jaundice
UCB is FAT SOLUABLE and can deposit in the BASAL GANGLIA (kerinicterus) leading to neuro deficitis or death
treat with phototherapy that somehow makes UCB water soluable and able to excrete in urine


Gilbert Syndrome

Auto recessive Mildly low Uridine Glucuonyl Transferase activity. During times of stress/infection get increased UCB and liver cant keep up so person becomes jaundiced whenever they are physiologically challneged by something but otherwise are healthy


Crigler-Najjar Syndrome

Absence of Uridine Glucuonyl Transferase leads to elevated UCB in serum leading to jaundice. Get deposition in fat of basal ganglia resulting in kernicterus and typically fatal early in life


Dubin-Johnson Syndrome
Rotor syndrome

Auto recessive Deficiency of bilirubin canicular TRANSPORT PROTEIN.
Get increased CB in serum and present with a very DARK LIVER.
Rotor syndrome is similar but lack dark liver


Biliary Tract Obstruction

gallstones, pancreatic carcinoma, cholangiocarcinoma, parasites, and liver fluke (clonorchis sinensis).
Get increased serum CB, decreased urine urobilinogen and increased alkaline phosphatase
Dark urine due to bilirubinuria and pale stool
pruritis due to increased bile acids in plasma
Hypercholesterolemia with xanthomas
Steatorrhea with malabsoprtion of fat soluable vitamins


Viral Hepatitis as it relates to Jaundice

Inflamation disrups hepatocytes and smill bile ductules
Increase in bot hserum UCB and CB
Dark urine due to increased bilirubinuria
Urine urobilinogen is normal or decreased (less CB being delivered to duodenum and more in blood)


Viral Hepatitis

Inflammation of liver parenchyma.
Usually due to hepatitis but also can occur with EBV and CMV
Presents as jaundice (miced UCB and CB) with dark urine (CB), fever, malaise, nausea, and elevated liver enzymes (ALT>AST)
Inflammation involves lobules of liver and portal tracts and characterized by cytotoxic apoptosis of heaptocytes
Acute = symptoms 6 months and inflmmation involves predominantly the portal tract. Risk of progression to cirrhosis


Hepatitis A

Fecal-oral transmission
Commonly acquired by travelers
only acute hepatitis
IgM marks active infection while IgG indicates immunization or prior infection
Vaccine exists


Hepatitis E

Fecal-oral transmission
Only acute hepatitis
Commonly aquired from contaminated water or undercooked seafood
no vaccine
IgM marks active infection while IgG indicates prior infection
HEV in preganant women is very troublesome as it can cause fulminant hepatitis (liver failure with massive liver necrosis)


Hepatitis C

Parenteral Transmission
Acute hepatitis and chronic diseases occurs in MOST
HCV-RNA test confirms infection.
Decreased RNA indicates recovery whereas persistence = chronic


Hepatitis D

Depends on HBV for infection
Superinfection (hep D on top of preexisting Hep B infection) is more severe than coinfection (Hep B and D infect at same time)


Hepatitis B

Parenteral transmission
Acute hepatitis that progresses to chronic in 20% of patients
Hep B surface antigen (HBsAG) is first serologic marker to rise in infection and its persistence >6 months indicates chronicity
Hep B envelope Antigen (HBeAG) and HBV DNA = indicates that person is infected and capable of transmitting the virus to others
Hep B Core Antibody (HBcAB) = IgM is initially used in indicates Acute or window phase to fight the virus while switch to IgG indicates the infection is resolves or has become chronic
Hep B surface Antibody = IgG is protective of future infections and seen in patients that have been vaccinated



End stage liver damage characterized by disruption of normal hepatic parenchyma by BANDS of FIBROSIS and regenerative NODULES of hepatocytes
Fibrosis is mediated by STELLATE CELLS that release TGF-BETA
Clinical features
1) Portal hypertension = ascites, congestive splenomegaly, portosystemic shunts (esophageal varices, hemorrhoids, caput medusae), and hepatorenal syndrome (rapidly developing kidney failure)
2) Decreased detoxification = Excess serum amonia reversible mental status changes, asterixis, and coma. Excess estrogen leads to gynecomastia, spider anigomata, and palmar erythema. Also get jaundice
3) Decreased protein synthesis = hypoalbuminemia w/ edema, and caogulopathy that you follow with monitoring PT


Alcohol Related Liver disease

Alcohol damages hepatic parenchyma
1) Fatty liver = accumulation of fat in the hepatocytes
2) Alcoholic Hepatitis = metabolite acetaldehyde damages hepatocytes and causes them to swell with formation of Mallory Bodies (damaged intermediate fillaments) necrosis and acute inflammation. Painful hepatomegaly and elevated liver enzymes (AST>ALT). AST is elevated because alcohol attacks mitochondria where AST is found.
3) Cirrhosis


Nonalcoholic fatty liver disease

Fatty change, hepatitis, and/or cirrhosis that develops without exposure to alcohol
Associated with obesity



Excess body iron leading to deposition in tissues (hemosiderosis) and organ damage (hemochromatosis). Tissue damage is medaited by generation of free radicals via fenton reaction
Primary = auto recessive defect in iron absoprtion where HFE gene is mutated to C282Y
Secondary -= chronic blood transfusions
Presents in late adulthood with triad of 1)cirrhosis 2)secondary DM, 3) bronze skin....can also see dilated cardiomyopathy, cardiac arrhythmias, and gonadal dysfunction
Labs show increased ferritin, decreased TIBC, increased serum iron and % saturation
Liver biopsy shows accumulation of brown pigment in hepatocytes that is distinguished fro mthe wear and tear brown pigment of lipofuscin in that it iron stains blue with prussian blue stain
treatment is phlebotomy


Wilson Disease

Auto recessive defect (ATP7B gene) in ATP mediated hepatocyte copper transport. Copper isnt transported to bile and isnt incorporated into ceruloplasmin.
Copper builds up in the hepatocytes, leaks into serum, and deposits in tissues where it generates hydroxyl free radicals and damages tissue.
Presents in childhood with 1)cirrhosis 2) neuro (behavioral changes, dementia, chorea, parkinsonian) 3) kayer-fleisher rings in cornea
Labs show increased urinary copper and copper in liver, decreased serum ceruloplasmin
Treatment is D-penicillamine (chelates copper)


Primary Biliary Cirrhosis

Autoimmune granulomatous destruction of intrahepatic bile ducts. Classically seen in women in 40s
Etiology unknown but see ANTIMITOCHONDRIAL ANTIBODY in serum
presents with features of obstructive jaundice
Cirrhosis is late complication


Primary Sclerosing Cholangitis

Inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. Periductal fibrosis with "onion skin" sppearance and uninvolved regions become dilated giving "beaded" appearance on contrast imaging
Etiology unknow but highly associated with ULCERATIVE COLITIS and p-ANCA is typically +
presents with features of obstructive jaundice
Cirrhosis is late complication
increased risk for CHOLANGIOCARCINOMA


Reye Syndrome

Fulminant liver failure and encephalopathy in children with viral illness that are given aspirin (prolly damages mitochondira of hepatocytes)
Presents with hypoglycemia, elevated liver enzymes, and nausea with vommiting.
May progress to coma and death


Hepatic Adenoma

Benign tumor of hepatocytes
Associated with oral contraceptives and regresses with cessation
Risk of rupture and intraperitoneal bleeding especially during pregnancy. Tumors are subcapsular and grow with exposure to estrogen


Hepatocellular Carcinoma

Malignant tumor of hepatocytes
Risk factors include:
-chronic hepatitis (HBV HCV)
- cirrhosis
-aflatoxins derived from aspergillus found on stored grains
Increased risk for Budd Chiari syndrome = liver infarction secondary to hepatic vein obstruction. presents with painful hepatomegally and ascites
Often found late and have poor prognosis
Serum tumor marker is ALPHA-FETOPROTEIN


Mets to Liver

More common than primary liver cancer
Colon, pancreas, lung and breasts are common sources
see multiple nodules in the liver
Get heptomegaly with a nodular free edge of the liver