Gastro - Pathology Part 3 Flashcards
What disease is “string sign” associated with?
Crohn’s Disease
A 25 year old female has a history of bloody diarrhoea and weigh loss. Her large intestine shows lesions that have spread from the rectum. What is the diagnosis?
Ulcerative colitis
What is the name of the finding in the eye where there is a brown arcus around the iris? What disease is it associated with?
Kayser-Fleischer ring ; associated with Wilson’s disease
What cancer is associated with Wilson’s disease?
Hepatocellular carcinoma
What movement disorder is associated with Wilson’s disease?
Choreiform movements due to preferential copper deposition in the basal ganglia
With what medication are patients with Wilson’s disease treated?
Penicillamine
With what pattern of inheritance is Wilson’s disease passed?
Autosomal recessive
What specific type of anemia is associated with Wilson’s disease?
Haemolytic anemia (remember ABCD to recall the other common findings: Asterixis, Basal ganglia degeneration, Ceruloplasmin [decreased], Cirrhosis, Corneal deposits, Copper accumulation, Carcinoma (hepatocellular), Choreiform movements, and Dementia)
A 30-year-old patient presents with new-onset dementia, choreiform movements, and flapping hand tremor; he also has severely elevated liver enzymes and discolored rings around his irises on ocular exam. What lab results will confirm the diagnosis? How will you treat the patient?
This patient has Wilson’s disease. Decreased ceruloplasmin; chelation with penicillamine
Hemochromatosis classically affects what three organs?
Liver, skin, and pancreas
Hemochromatosis classically causes what liver finding?
Micronodular cirrhosis
A man presents to the office with new-onset insulin-dependent diabetes. His skin is darkly pigmented and he is found to have elevated liver enzymes. What is his likely diagnosis?
Hemochromatosis
Iron deposition in the heart due to hemochromatosis can lead to what condition?
Congestive heart failure secondary to restrictive cardiomyopathy
Hemochromatosis is associated with an increased risk of what cancer?
Hepatocellular carcinoma
With what pattern of inheritance is primary hemochromatosis passed?
Autosomal recessive
True or False: It is possible to develop hemochromatosis without being genetically predisposed to the disease.
True (secondary hemochromatosis); due to recurrent blood transfusions
What is the most common cause of secondary hemochromatosis?
Chronic transfusion therapy
Describe ferritin capacity, iron capacity, and total iron-binding capacity and transferrin saturation in patients with hemochromatosis.
High; high; low; high
What two treatments are often used for hemochromatosis?
Repeated phlebotomy and deferoxamine chelation
Hemochromatosis is associated with what genetic marker?
Human leukocyte antigen A3
Is primary sclerosing cholangitis intrahepatic, extrahepatic, or both?
Both; the entire biliary tree is affected
What is the underlying change in primary sclerosing cholangitis?
Fibrosis of the bile ducts
What is likely to be found on biopsy of a patient with primary biliary cirrhosis?
Lymphocytic infiltrate and granulomas
What is the appearance of primary sclerosing cholangitis on endoscopic retrograde cholangiopancreatography?
Alternating strictures and dilation of the bile ducts, which is also called “ beading”
Which symptoms are common in the presentation of biliary tract disease?
Jaundice, light stool, pruritus, dark urine
What disease is associated with primary sclerosing cholangitis?
Ulcerative colitis
Which autoantibodies are seen in the serum of patients with primary biliary cirrhosis?
Antimitochondrial antibodies
The biliary stasis caused by PSC can be so severe as to cause liver failure, a process known as what?
Secondary biliary cirrhosis
What abnormal lab results are common to most forms of biliary tract disease?
Increased conjugated bilirubin, cholesterol, and alkaline phosphatase
Primary biliary cirrhosis is commonly associated with what autoimmune disorders?
Rheumatoid arthritis, CREST syndrome, coeliac disease
What class of immunoglobulin is elevated in primary sclerosing cholangitis?
Immunoglobulin M
What causes secondary biliary cirrhosis?
Extrahepatic biliary obstruction
In secondary biliary cirrhosis, increased pressure in the intrahepatic ducts leads to what?
Hepatic injury and fibrosis
Secondary biliary cirrhosis is often complicated by what infectious process?
Ascending cholangitis, a bacterial infection facilitated by biliary stasis
Disproportionately high amounts of _____ and/or _____ in bile tend to favor the formation of gallstones.
Cholesterol; bilirubin
Which substances in bile increase the solubility of bilirubin and cholesterol to prevent formation of gallstones?
Bile acid and lecithin
List the two types of gallstones.
Cholesterol stones and pigment stones
Are most cholesterol stones radiolucent or radiopaque?
Radiolucent; as a result, ultrasound is the preferred method of imaging
About what percent of cholesterol stones are radiopaque?
10% to 20%
Why are some cholesterol stones radiopaque?
Calcified stones are radiopaque
Why does Crohn#039;s disease predispose patients to gallstones?
Because of the inability of the diseased terminal ileum to absorb bile salts
List the risk factors associated with formation of cholesterol stones.
Obesity, Crohn’s disease, cystic fibrosis, advanced age, clofibrate, estrogens, multiparity, rapid weight loss, and Native American origin
What percentage of gallstones are cholesterol stones?
80%
Are pigment stones radiolucent or radiopaque?
Radiopaque
A patient presents with right upper quadrant pain, jaundice, and fever. He is found to have gallstones on ultrasound and a high white blood cell count. What is the likely diagnosis?
Acute cholangitis
In addition to biliary infections, what two other medical conditions are associated with pigment stone formation?
Chronic RBC hemolysis and alcoholic cirrhosis
Gallstones are best diagnosed by what radiologic modality?
Ultrasound
Symptomatic gallstones are treated with _____.
Cholecystectomy
Gallstones can cause what four major complications?
Ascending cholangitis, acute pancreatitis, bile stasis, and cholecystitis
Define biliary colic.
Pain caused by gallstones interfering with bile flow, causing bile duct contraction
In what population of patients is biliary colic uncommon?
Diabetic patients; neuropathy can reduce the sensation of pain from gallstones
What radiographic finding is associated with obstruction of the ileocecal valve by a gallstone?
Air in the biliary tract (also known as pneumobilia)
Name the “four F’s” associated with increased risk of developing gallstones.
Female, fat, fertile, and forty
Name the components of Charcot”s triad of symptoms of cholangitis.
Jaundice, fever, and right upper quadrant pain
A patient presents complaining of abdominal pain. On deep palpation of the right upper quadrant, she appears to hold her breath. Name this finding.
The patient has a positive Murphy’s sign, which is inspiratory arrest on deep palpation
Name three possible causes of cholecystitis.
Gallstones (most common), infection (cytomegalovirus), ischemia
Define the pathophysiology of acute pancreatitis.
Pancreatic enzymes become activated leading to the autodigestion of the pancreas
List the causes of acute pancreatitis.
Gallstones, ethanol, trauma, steroids, mumps, autoimmune disease, scorpion sting, hypercalcemia/hyperlipidemia, drugs (sulfa drugs) (remember: GET SMASHeD)
What pattern of pain is characteristic of acute pancreatitis?
Epigastric pain that radiates to the back
What two laboratory findings are classically diagnostic of acute pancreatitis?
Elevated amylase and lipase
Which has higher specificity for acute pancreatitis: amylase or lipase?
Lipase
What are complications of acute pancreatitis?
Disseminated intravascular coagulation, acute respiratory distress syndrome, hypocalcemia, diffuse fat necrosis, pseudocyst formation, hemorrhage, infection, multisystem organ failure
Chronic calcifying pancreatitis is strongly associated with what condition?
Alcoholism
What is the mechanism causing hypocalcemia in acute pancreatitis?
Ca2+ collects in pancreatic calcium soap deposits, causing hypocalcemia
Chronic pancreatitis leading to pancreatic insufficiency results in what constellation of symptoms?
Steatorrhea, fat-soluble vitamin deficiency, and diabetes mellitus
Is acute or chronic pancreatitis associated with an increased risk of pancreatic cancer?
Chronic
What is the average survival of a person newly diagnosed with pancreatic adenocarcinoma?
Six months or less
A patient presents with weight loss, night sweats, and painless jaundice. He is found to have a mass on his pancreas suspicious for malignancy. What is the most likely location of the mass?
The head of the pancreas
A mass is found on the head of the pancreas of a patient. What abnormal lab findings would be found in this patient?
Increased alkaline phosphatase and bilirubin levels indicating obstructive jaundice
True or False? Pancreatic adenocarcinoma often presents with inguinal lymphadenopathy.
False; presentation typically includes abdominal pain that radiates to the back, weight loss, migratory thrombophlebitis, and obstructive jaundice
What is an abdominal exam finding in a patient with pancreatic adenocarcinoma?
Courvoisier’s sign (palpable gallbladder)
When migratory thrombophlebitis is noted in patients with pancreatic adenocarcinoma, it is called what?
Trousseau’s syndrome
What two ethnic groups have an increased risk of pancreatic cancer? Male or female?
Jewish and African-American males
Name two tumor markers associated with pancreatic cancer.
Carcioembryonic antigen and carbohydrate antigen 19-9 (ie CEA and Ca19-9)
True or False? Pancreatic cancer has a strong association with alcohol abuse.
False; pancreatic cancer has been linked to cigarette smoking
What is the inheritance pattern for familial adenomatous polyposis ?
It is inherited in an autosomal dominant fashion. Afflicted individuals inherit one faulty copy of the gene and lose the other through an acquired mutation; this is two-hit hypothesis
