Select a Community
Are you sure you want to trigger topic in your Anconeus AI algorithm?
You are done for today with this topic.
Would you like to start learning session with this topic items scheduled for future?
Secretion into bile
68%
192/283
Loop of Henle secretion into lumen of kidney
8%
23/283
Glomerular filtration
11%
32/283
Bleeding
3%
9/283
Duodenal secretion
6%
18/283
Select Answer to see Preferred Response
This patient is suffering from Wilson disease, a disorder that results from copper deposition in various tissues. The main route of copper elimination, and that impaired in Wilson disease, is via hepatocyte-mediated secretion of copper into the bile. In Wilson disease, also known as hepatolenticular degeneration, a defect in the ATP7B gene product, P-type copper ATPase, leads to impaired copper trafficking and secretion into the bile. This results in copper accumulation within liver cells and the eventual release of free copper into circulation. Free copper generates free radicals that damage tissues. Heidelbaugh and Bruderly discuss the diagnostic evaluation of patients with cirrhosis and chronic liver failure. If one suspects Wilson disease (usually in a patient younger than 40 years with family history of liver disease), serum ceruloplasmin and copper levels should be measured. However, the authors note that screening all patients with chronic hepatic injury for Wilson disease is not indicated. Treatment for Wilson disease consists of a low-copper diet and lifelong copper chelating agents. Wiggelinkhuizen et al. conducted a systematic review of 1 randomized trial and 12 observational studies evaluating D-penicillamine, trientine, tetrathiomolybdate, or zinc monotherapy as initial treatment for Wilson disease. The authors concluded that insufficient evidence existed to determine relative treatment effects of the various drugs. Illustration A depicts copper metabolism in patients with Wilson disease. Note that there is reduced excretion and, thus, retention of copper. Illustration B shows an histology image of hepatocytes in Wilson's disease. The rhodadine stain highlights the copper deposition. Incorrect Answers: Answer 2: Copper is not secreted by the Loop of Henle. Answer 3: Though copper is filtered by the kidneys, this is not the major route of copper elimination as less than 20% is eliminated this way. Answer 4: Though bleeding may lead to copper loss, this is not the body's major pathway of copper elimination. Answer 5: Copper is not secreted by the duodenum, instead, copper is absorbed in the duodenum.
4.5
(4)
Please Login to add comment