Kayser-Fleischer rings are seen in Wilson disease due to copper deposition; which organ system is primarily affected?

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Study for the NBME Form 13 Test with our comprehensive quiz collection, featuring flashcards and multiple-choice questions to ensure your readiness. Enhance your knowledge with detailed explanations for each question.

Multiple Choice

Kayser-Fleischer rings are seen in Wilson disease due to copper deposition; which organ system is primarily affected?

Copper handling defects in Wilson disease cause copper to build up in the liver first. A mutation in ATP7B impairs excretion of copper into bile and its incorporation into ceruloplasmin, so hepatocytes accumulate copper, leading to liver injury that can range from mild enzyme elevations to hepatitis and cirrhosis. As copper overload progresses, it can spill into the bloodstream and deposit in other tissues, including the cornea (Kayser-Fleischer rings) and brain, causing neuropsychiatric symptoms. Because hepatic copper accumulation is the initiating and primary event, the liver is the organ system most affected.

Kayser-Fleischer rings are seen in Wilson disease due to copper deposition; which organ system is primarily affected?

Study for the NBME Form 13 Test with our comprehensive quiz collection, featuring flashcards and multiple-choice questions to ensure your readiness. Enhance your knowledge with detailed explanations for each question.

Kayser-Fleischer rings are seen in Wilson disease due to copper deposition; which organ system is primarily affected?

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