Term
| What are the different types of gross gallbladder malformations? |
|
Definition
| septate, bilobed, diverticular, misplaced |
|
|
Term
|
Definition
| gallbladder malformation with an inward folded fundus |
|
|
Term
| When does a biliary atresia manifest? |
|
Definition
|
|
Term
| One-third of infants with neonatal cholestasis have _________. |
|
Definition
|
|
Term
| What happens in biliary atresia is left untreated? |
|
Definition
| secondary biliary cirrhosis develops within 3-6 months |
|
|
Term
| 50-60% of children referred for liver transplant are referred b/c of _______. |
|
Definition
|
|
Term
| What is the most common cause of death from liver disease in early childhood? |
|
Definition
|
|
Term
| What are the two forms of biliary atresia? |
|
Definition
fetal (aberrant development) 20%
perinatal (secondary damage: viral infection, autoimmune injury) |
|
|
Term
| What are the three types of biliary atresia? |
|
Definition
I: common bile duct atresia with patent proximal duct
II: hepatic duct atresia, with patent or obliterated cystic and common bile ducts
type III: complete extrahepatic biliary atresia (including right and left hepatic ducts) |
|
|
Term
| WHat is the most common type of biliary atresia? |
|
Definition
|
|
Term
| How do you fix type III biliary atresia? |
|
Definition
| biliary enteric anastomosis aka hepatoportoenterostomy or the "kasai procedure" |
|
|
Term
| What do you call congenital cystic dilatations or diverticular of the gallbladder? |
|
Definition
|
|
Term
| What are the seqalae of choledochal cysts? |
|
Definition
| neonatal cholestasis, recurrent biliary colic/jaundice, pancreatitis |
|
|
Term
| When do choledochal cysts manifest? |
|
Definition
|
|
Term
| What population are choledochal cysts more common in? |
|
Definition
|
|
Term
| Choledochal cysts predispose the patient to what complications? |
|
Definition
| cholangiocarcinoma, cholelithiasis, stenosis |
|
|
Term
| What percent of choledochal cysts are single dilatations? |
|
Definition
|
|
Term
| What percent of the population has cholelithiasis? |
|
Definition
|
|
Term
| T/F Most gallstones are clinically silent. |
|
Definition
|
|
Term
| What are the symptoms of gall stones? |
|
Definition
| constant pain or postprandial colicky pain (after fat rich meals) |
|
|
Term
| What type of gall stones are most likely to cause biliary obstruction? |
|
Definition
| very small stones called "gravel" |
|
|
Term
| What are gallstones made of? |
|
Definition
cholesterol, calcium bilirubinate, calcium carbonate
10% pure, 80% mixed, 10% combined (nucleus/shell) |
|
|
Term
| What percent of stones are cholesterol stones? |
|
Definition
|
|
Term
| What are the characteristics of cholesterol versus pigmented stones? |
|
Definition
cholesterol= pale yellow
pigmented= brown/black |
|
|
Term
| What do gallstones look like on xray? |
|
Definition
| pure cholesterol stones are radiolucent; mixed/combined stones are radiopaque depending on their concentration of calcium |
|
|
Term
| What solubilizes cholesterol in the bile? |
|
Definition
|
|
Term
| Nucleation (crystallization) of cholesterol is promoted by... |
|
Definition
| supersaturation of bile wit hcholesterol, microprecipitates of inorganic or organic calcium salts |
|
|
Term
| What are racial, age, sex, etc. risk factors for cholesterol stones? |
|
Definition
| native americans, hispanics, northern europeans, age >55, female sex, oral contraceptives, pregnancy, estrogen replacement therapy, obesity, rapid weight reduction (gastric bypass), hyperlipidemia, ilial dysfunction or bypass, family history, some cholesterol lowering medications (fibrates) |
|
|
Term
| T/F Cholesterol lowering meds lower your risk of cholesterol gall stones. |
|
Definition
| yes and no. some cholesterol lowering medications (fibrates) increase your risk, others (statins) may decrease your risk |
|
|
Term
| What are the risk factors for pigmented gall stones? |
|
Definition
| asian population, hemolytic disorder, bacterial colonization of the biliary tree (bacterial glucuronidases), parasitic infestation of the biliary tree (ascaris lumbricoides, opisthorchis sinensis) |
|
|
Term
|
Definition
| accumulation of cholesterol esters in foamy histiocytes within the lamina propria of the gall bladder. Related to "cholesterol hypersecretion" |
|
|
Term
| What is the gross appearance of cholesterosis? |
|
Definition
|
|
Term
| What causes 90% of acute cholecystitis? |
|
Definition
| gallstones in the neck or cystic duct |
|
|
Term
| Acute acalculous cholecystitis is due to... |
|
Definition
| ischemia/shock (cystic artery is an end artery with no collaterol circulation), primary bacterial infection, HIV/AIDS |
|
|
Term
| What are the symptoms of acute cholecystitis? |
|
Definition
| right upper quadrant pain, nausea, vomiting, mild to moderate fever, leukocytosis |
|
|
Term
| What symptoms are NOT associated with acute cholecystitis and are instead associated with cholangitis? |
|
Definition
| high fever, chills, jaundice or hyperbilirubinemia |
|
|
Term
| How long does acute cholecystitis last? |
|
Definition
| usually subsides within days |
|
|
Term
| T/F Acute cholecystitis may require immediate surgery. |
|
Definition
|
|
Term
| Patient with a history of an episode of acute cholecystitis wants to know if she'll have another episode. What do you tell her? |
|
Definition
|
|
Term
| Acalculous cholecystitis can hoften have dangerous complications such as... |
|
Definition
|
|
Term
| What percent of chronic cholecystitis is due to cholelithiasis? |
|
Definition
|
|
Term
| What are the symptoms of chronic cholecystitis? |
|
Definition
| recurrent attacks of epigastric pain |
|
|
Term
| What are the microscopic findings that correlate with chronic cholecystitis? |
|
Definition
| chronic inflammation and fibrosis with outpouchings of the mucosa known as rokitansky aschoff sinuses |
|
|
Term
| What do you call calcification of the gallbladder thought to be brought on by chronic gallstones? |
|
Definition
|
|
Term
| What are the complications of cholecystitis? |
|
Definition
| choledocholithiasis/cholangitis, sepsis, perforation, abscess, peritonitis, cholecystenteric fistula, gallstone ileus |
|
|
Term
| Most stones within the common bile duct originate in the ________. |
|
Definition
|
|
Term
| Stones that form WITHIN the bile duct are associated with... |
|
Definition
| cysts, bacterial and parasitic infections |
|
|
Term
| What are complications of obstruction of the common bile duct? |
|
Definition
| colic, jaundice, cholangitis, secondary biliary cirrhosis, pancreatitis |
|
|
Term
| What is the term for inflammation of the biliary tract? |
|
Definition
|
|
Term
| What are the most common causes of cholangitis? |
|
Definition
| choledocholithiasis and enteric gram negatives (ascending infection) |
|
|
Term
|
Definition
| clinical findings of cholangitis: RUQ pain, fever/chills, jaundice/hyperbilirubinemia |
|
|
Term
| How do you treat cholangitis? |
|
Definition
| medical emergency! prompt antibiotic therapy, endoscopic biliary drainage, surgical evacuation |
|
|
Term
| What are complications of cholangitis? |
|
Definition
|
|
Term
| What are the neoplasms of the gallbladder? |
|
Definition
| gallbladder carcinoma, gallbladder adenoma |
|
|
Term
| Describe the appearance of gallbladder adenomas? |
|
Definition
| similar to colonic adenomatous polyps; tubular, tubulovillous, villous |
|
|
Term
| What predisposes you to gallbladder cancer? |
|
Definition
| gallstones in 60-90% of gallbladder ca, female/estrogen, native american, hispanics, older age, obesity, salmonella typhi carriers, smoking, alcoholism |
|
|
Term
| Most gallbladder cancers are _____. |
|
Definition
|
|
Term
| How do you clinically diagnose gallbladder adenocarcinomas? |
|
Definition
| very difficult; symptoms are those associated with cholelithiasis |
|
|
Term
| T/F Gallbladder cancer has a dismal survival rate. |
|
Definition
|
|
Term
| Most carcinomas of the extrahepatic bile ducts are _____. |
|
Definition
|
|
Term
| Carcinomas of the extrahepatic bile ducts are more common in ___. |
|
Definition
|
|
Term
| Carcinomas of extrahepatic bile ducts are associated with... |
|
Definition
| primary sclerosing cholangitis (IBD), choledochal cysts, liver flukes |
|
|
Term
| What is courvoisier's sign? |
|
Definition
| painless, progressive jaundice and palpable gallbladder that indicates carcinoma of the extrahepatic bile ducts |
|
|
Term
| What is the prognosis of extrahepatic bile duct carcinoma? |
|
Definition
| most are not resectable= short survival |
|
|
Term
| What is a Klatskin's tumor? |
|
Definition
| tumor that is proximal to the cystic duct |
|
|
Term
| What is the embryologic origin of the different ducts of the pancreas? |
|
Definition
| ventral pancreatic bud forms the main duct and the dorsal pancreatic bud forms the accesory duct if there is one |
|
|
Term
| What do you call it when the ventral pancreatic bud ends up strangulating the duodenum? |
|
Definition
|
|
Term
| Where are ectopic/accessory pancreases found? |
|
Definition
| stomach, duodenum, jejunum, ileum, meckel's diverticulum, mesentery |
|
|
Term
| What are the complications of ectopic/accessory pancreases? |
|
Definition
| inflammation, bleeding, tumors |
|
|
Term
| What are the two main causes of acute pancreatitis that account for 80% of cases? |
|
Definition
| alcohol in males and gallstones in females |
|
|
Term
| What are minor causes of acute pancreatitis? |
|
Definition
| hypertriglyceridemia, hypercalcemia, hyperparathyroidism, drugs, trauma (blunt, surgical), infections (mumps), pancreas divisum, choledochocele, periampullary tumors, mutations in trypsinogen and serine protease inhibitor, abnormal bicarb secretion |
|
|
Term
| Which drugs can cause acute pancreatitis? |
|
Definition
|
|
Term
| 10-20% of the cases of acute pancreatitis are due to... |
|
Definition
| cryptogenic (biliary sludge) |
|
|
Term
| How does alcohol cause pancreatitis? |
|
Definition
| protein rich secretion, protein plugs, contraction of sphincter of Oddi, direct acinar injury |
|
|
Term
| What is the morphology of acute pancreatitis? |
|
Definition
| inflammation, fat necrosis, released fatty acids combine with calcium to form dark precipitates, destruction of the parenchyma and vessels/hemorrhage |
|
|
Term
| Classically, pts with acute pancreatitis have their disease precpitated by... |
|
Definition
| alcoholic binge, overeating, drugs (opiates) |
|
|
Term
| What is the classic description of pain from acute pancreatitis? |
|
Definition
| stabbing, severe, referred to the upper back |
|
|
Term
| What should you look for on physical exam if you suspect acute pancreatitis? |
|
Definition
| periumbilical or flank ecchymosis, nodules of subcutaneous fat necrosis |
|
|
Term
|
Definition
| periumbilical ecchymosis assoc with acute pancreatitis |
|
|
Term
|
Definition
| flank ecchymosis assoc with acute pancreatitis |
|
|
Term
| What would you expect to find on labs for a patient with acute pancreatitis? |
|
Definition
| increased amylase and lipase, hypocalcemia, hyperglycemia, leukocytosis, electrolyte disturbances |
|
|
Term
| How do you tx acute pancreatitis? |
|
Definition
| restriction of food and fluids (pancreatic resting) and "supportive" therapy |
|
|
Term
| What are complications of acute pancreatitis? |
|
Definition
| DIC, ARDS, shock/acute renal failure, pancreatic pseudocyst |
|
|
Term
| What is the mortality rate of acute pancreatitis? |
|
Definition
|
|
Term
| What is a pancreatic pseudocyst? |
|
Definition
| walled-off areas of pancreatic fat necrosis (collection of fluid and debris), lack epithelial lining, wall made of fibrosis and granulation tissue |
|
|
Term
| What casues pancreatic pseudocysts? |
|
Definition
| acute pancreatitis or trauma |
|
|
Term
| What are the causes of chronic pancreatitis? |
|
Definition
| repeated bouts of acute pancreatitis, alcoholism, longstanding pancreatic duct obstruction (gallstones, neoplasms), pancreas divisum, tropical pancreatitis, idiopathic, hereditary pancreatitis |
|
|
Term
| What is the most common cause of chronic pancreatitis? |
|
Definition
|
|
Term
| What percent of chronic pancreatitis is idiopathic? |
|
Definition
|
|
Term
| What are the mutations that cause hereditary pancreatitis? |
|
Definition
| autolysis resistant trypsin, altered trypsin inhibitor (serine protease), decreased bicarbonate secretion |
|
|
Term
| What are the gross characteristics of chronic pancreatitis? |
|
Definition
| fibrosis and calcified concretions |
|
|
Term
| What are the complications of chronic pancreatitis? |
|
Definition
| malabsorption (pancreatic insufficiency), DM, severe chronic pain, pancreatic cancer |
|
|
Term
| What are the different types of pancreatic cystic neoplasms? |
|
Definition
| serous, mucinous, intraductal papillary mucinous |
|
|
Term
| Describe serous cystadenomas of the pancreas? |
|
Definition
| microcystic (numerous small), clear, thin, straw-colored fluid, low cuboidal epithelium, almost always benign |
|
|
Term
| Pancreatic serous cystadenomas are more common in... |
|
Definition
|
|
Term
| Describe pancreatic mucinous cystic neoplasms? |
|
Definition
| macrocystic (few large cysts), thick, mucinous fluid, tall columnar epithelium, can be benign "borderline" or malignant |
|
|
Term
| What pancreatic neoplasm occurs almost exclussively in women? |
|
Definition
|
|
Term
| Pancreatic intraductal papillary mucinous neoplasm is more common in... |
|
Definition
|
|
Term
| What is an intraductal papillary mucinous neoplasm? |
|
Definition
| intraductal proliferation of mucinous cells in papillary arrangements, mucin accumulation leads to cystic dilatation of the ducts. Can be benign, borderline, or malignant |
|
|
Term
| Where are intraductal papillary mucinous neoplasms usually located? |
|
Definition
| in the head of the pancreas |
|
|
Term
| What is the incidence and mortality of pancreatic (ductal) adenocarcinoma? |
|
Definition
| greater in men than in women, greater in blacks than in whites, greater in acadiana |
|
|
Term
| What is the 5 year survival rate of pancreatic ductal adenocarcinoma? |
|
Definition
|
|
Term
| What % of cancer is pancreatic cancer? |
|
Definition
|
|
Term
| What is the % of cancer deaths are due to pancreatic cancer? |
|
Definition
|
|
Term
| What are the risk factors for pancreatic adenocarcinoma? |
|
Definition
| smoking, highprotein/fat diets, alcohol, chronic pancreatitis, diabetes, exposure to gasoline products/pesticides, hereditary pancreatitis, hereditary nonpolyposis colorectal cancer, peutz-jeghers syndrome, BRCA2 |
|
|
Term
| What are mutations commonly found in pancreatic adenocarcinoma? |
|
Definition
|
|
Term
| Where are the locations of pancreatic adenocarcinomas? |
|
Definition
60% head, 15% body, 5% tail
20% diffusely |
|
|
Term
| What are the microscopic features of pancreatic (ductal) adenocarcinoma? |
|
Definition
| high invasiveness (perinerual invation and lymphatic invasion), desmoplastic (scirrhous, fibrous) |
|
|
Term
| What are the symptoms of pancreatic ductal adenocarcinoma? |
|
Definition
| pain, obstructive jaundice, migratory thrombophlebitis (Trousseau's signs) due to tumor procoagulants |
|
|
Term
| What is the physical exam finding for pancreatic (ductal) adenocarcinoma? |
|
Definition
| palpable gallbladder (courvoisier's sign) |
|
|
Term
| What percent of pancreatic (ductal) adenocarcinomas are resectable? |
|
Definition
|
|
Term
| What percent of pancreatic neoplasms are pancreatic endocrine neoplasms? |
|
Definition
|
|
Term
| What are pancreatic endocrine neoplasms? |
|
Definition
| Similar to carcinoids; some elaborate hormones and some are non-functional. May be associated with MENI |
|
|
Term
| What is the most common type of pancreatic endocrine neoplasm? |
|
Definition
| insulinoma (beta cell tumor), may cause severe hypoglycemia |
|
|
Term
|
Definition
| fatigue, confusion, stupor, coma, convulsions, glucose <50 mg/dL, attacks precipitated by fasting glucose/exercise, associated with insulinoma |
|
|
Term
| What percent of insulinomas metastasize? |
|
Definition
|
|
Term
| What is zollinger-Ellison syndrome? |
|
Definition
| G cell tumor hypergastrinemia that causes multiple peptic ulcers (gastric, duodenal, jejunal) that are unresponsible to therapy, and diarrhea |
|
|
Term
| What's the risk of gastrinomas metastasizing? |
|
Definition
| over half are locally invasive or have metastasized at the time of diagnosis |
|
|
Term
| What is glucagonoma syndrome? |
|
Definition
| caused by alpha cell tumors (glucagonoma); mild DM, necrolytic migratory erythema, deep vein thrombosis, anemia,tendency to develop overwhelming infections. |
|
|
Term
| What types of patients usually have glucagonomas/alpha cell tumors? |
|
Definition
| peri and post menopausal women |
|
|
Term
| Do glucagonomas metastasize? |
|
Definition
| approx 50% have metastases at the time of diagnosis |
|
|
