Liver, Pancreas & Gallbladder Flashcards
What is biliary atresia?
Biliary atresia is the closure or absence of some or all of the major bile ducts.
What is cholestasis?
Cholestasis is arrest of the flow of bile. This may be due to intraheptaic causes, obstruction of the bile duct by gallstones, or any process that blocks the bile duct, e.g. cancer.
What is cholangitis?
Cholangitis is inflammation of the bile ducts.
What is primary sclerosing cholangitis?
Primary sclerosis cholangitis is a chronic liver disease of unknown origin marked by inflammation and obliteration of the intrahepatic and extraheptaic bile ducts. The disease progresses silently and steadily and in most patients leads to cirrhosis, portal hypertension, and liver failure. Seventy percent of patients are men and mean age at diagnosis is 39. Liver transplant can be used treat patients who develop cirrhosis from this disease.
What is Gilbert syndrome?
Gilbert syndrome is a benign, autosomal dominant form of jaundice with variable penetrance, secondary to glucoronyl-transferase deficiency, and resulting in elevated levels of unconjugated bilirubin.
Incidence: The condition is common, present in about 5% to 10% of people of European ancestry.
Causes: it is caused by a deficiency in the enzyme system that conjugates bilirubin (bilirubin-uridine disphosphate glucoronyl transferase) making it insoluble in water. In the absence of this enzyme, unconjugated bilirubin accumulates in the body.
Symptoms: Most patients have no symptoms; some have nonspecific abdominal pain. Patients may have mild elevations of serum bilirubin levels that may or may not be visible on casual examination of the skin.
What is galactosemia?
Galactosemia is an autosomal recessive disorder marked by an inability to metabolism galactose because of a congenital absence of one of two enzymes needed to convert galactose to glucose. The diagnosis is confirmed by testing the newborn’s uterine for non carbohydrate reducing substances or more accurately by tests for the missing enzymes in blood cell.s The infant with galactosemia will fail to thrive within a week after birth due to anorexia, vomiting, and diarrhea unless galactose and lactose are removed from his or her diet.
Glactosemia can lead to both direct and indirect hyperbilirubenemias.
What is breast milk jaundice (breast-feeding jaundice)?
Breast milk jaundice is a hyperbilirubenemia resulting from prenanediol or free fatty acids that inhibit bilirubin conjugation. Serum bilirubin level usually peaks above 20 ml/dl by 14 to 21 days of age. Some pediatricians recommend stopping breast-feeding for 24-36 hours if the level exceeds 20 ml/dl. If the infants bilirubin level drops rapidly, the mother may resume nursing. It typically lasts for the first week of life or until the mother produces adequate milk.
What is hemochromatosis?
Hemochromatosis is a genetic disease marked by excessive absorption and accumulation of iron in the body. The disease is caused by one of several recessive mutations that result in excessive absorption of iron from the gastrointestinal tract. It is not caused by secondary iron overload.
Symptoms: At the time of diagnosis the patient may be asymptomatic. Symptomatic patients may experience weakness, fatigue, arthralgias, abdominal pain, liver failure (cirrhosis), symptoms of diabetes mellitus or heart failure, thyroid disorders, or impotence. These symptoms are caused by the deposition of excess iron into multiple organ systems.
Diagnosis: Physical findings include gray or bronzed skin pigmentation changes, enlarged liver, arthritis, signs of congestive heart failure, and it males, testicular atrophy.
Treatment: Treatment consists of serial phlebotomy.
What is cholecystitis?
Cholecystitis is inflammation of the gallbladder wall, usually caused by obstruction of the bile ducts by bile stones. The disease is marked by colicky pain developing shortly after a meal in the right upper quadrant of the abdomen.
Causes: Acute cholecystitis is usually caused by obstruction of the bile ducts, with chemical irritation and often infection of the gallbladder.
Symptoms & Signs: Cholecystitis caused by gallstones results in right upper quadrant pain that occurs after a a fatty meal, as well as fever, chills, nausea, and vomiting. The pain of cholecystitis often radiates into the right shoulder or right side of the back. Jaundice is present in about 20% of patients, usually related to obstruction of the common bile duct by a gallstone.
Diagnosis: Ultrasonography of the right upper quadrant, the diagnostic procedure of choice, reveals cholecystitis in about 90% of patients.
Treatment: Gallbladder drainage (cholecystectomy) is the usual treatment; it is sometimes used as a temporizing procedure in unstable patients. Gallstones lodged in the ampulla of Vater can sometimes be removed with endoscopic retrograde cholangiopancreastography.
What is a cholecystectomy?
Cholecystectomy is removal of the gallbladder by laparoscopic or abdominal surgery. The procedure is performed for symptomatic gallbladder and bile duct disease.
What is a calculus, and what is biliary colic?
Calculus is a stone, usually composed of mineral salts. Stones can occur in the gallbladder, kidneys, uretersm bladder, or urethra.
Biliary colic refers to a gallstone.
What is the cholecystokinin-secretin test (secretin stimulation test)?
The secretin stimulation test is a direct test of pancreatic function that assesses both the endocrine and exocrine functions of the pancreas. A double-lumen tube is inserted into the patient’s gastrointestinal tract. One lumen samples the duodenal juices, the other removes gastric secretions. First secretin and the cholecystokinin are given to the patient intravenously; then the duodenal juices are analyzed to determine whether adequate levels of bicarbonate and trypsin are secreted.
What is primary biliary cholangitis (PBC)?
Primary biliary cholangitis (PBC) is an autoimmune disorder that leads to the gradual destruction of intrahepatic bile ducts, resulting in periportal inflammation and cholestasis. Primary biliary cholangitis is associated with highly specific antibodies. The anti-mitochondrial antibody is found in 85% of the cases.
What is Dubin-Johnson syndrome?
Dubin-Johnson syndrome is an inherited defect of bile metabolism that causes retention of conjugated bilirubin in hepatic cells. The patient is asymptomatic except for mild intermittent jaundice. No treatment is required.
What is Rotor syndrome?
Rotor syndrome is a benign form of hyperbilirubinemia transmitted as an autosomal recessive trait, in which there is jaundice, but normal aminotransferase levels and normal hepatic synthesis of albumin and clotting factors.
