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JIPMER Nursing Officer - 2020
Medical Surgical Nursing
Easy

Abnormal copper accumulation seen in which disease condition?

Appeared in: JIPMER Nursing Officer - 2020

Explanation

  • Wilson's disease is an autosomal recessive genetic disorder characterized by the abnormal accumulation of copper in the body.
  • It is caused by mutations in the ATP7B gene, which impairs the liver's ability to excrete excess copper into the bile.
  • This leads to copper buildup in vital organs, most notably the liver, brain, and corneas of the eyes.
  • A classic diagnostic sign is the presence of Kayser-Fleischer rings, which are brownish-gold rings in the cornea visible on a slit-lamp exam.

Why Other Options Were Wrong

  • Option B: Guillain-Barré syndrome (GBS) is an autoimmune disorder where the immune system attacks the peripheral nerves. It is not related to metal metabolism.
  • Option C: Compartment syndrome is a condition of increased pressure within a confined muscle space, usually after trauma, which compromises circulation and function. It does not involve copper accumulation.
  • Option D: Dressler syndrome is a form of secondary pericarditis that occurs as an immune response after damage to heart tissue, such as a heart attack or cardiac surgery. It is an inflammatory condition, not a metabolic one.

Related Visual

Visual explanation — Related Visual
Clinical Relevance
  • Nursing practice connection: Knowing Pathophysiology of Wilson's Disease helps nurses interpret findings accurately and avoid errors in routine assessment, medication administration, and patient teaching.
  • Nurses play a crucial role in managing Wilson's disease through patient education on dietary restrictions (avoiding high-copper foods) and ensuring strict adherence to lifelong chelation therapy.
  • Monitoring for adverse effects of chelating agents (e.g., D-penicillamine can cause bone marrow suppression) and assessing liver function and neurological status are key nursing responsibilities.
  • What if? A patient with Wilson's disease presents with sudden jaundice and confusion. The nurse should recognize these as signs of acute liver failure, a medical emergency requiring immediate escalation to the provider and preparation for potential intensive care.
How to Approach the Question
  • First, identify the key phrase in the question stem: "abnormal copper accumulation."
  • This is a factual recall question that tests your knowledge of specific metabolic diseases.
  • Systematically review each option, linking it to its known pathophysiology.
  • Recall that Wilson's disease is the classic example of a disorder of copper metabolism.
  • Eliminate the other options by identifying their distinct causes: GBS (autoimmune), Compartment syndrome (pressure/ischemia), and Dressler syndrome (post-MI inflammation).
  • Confirm the choice by remembering hallmark signs of Wilson's disease, such as Kayser-Fleischer rings, which are directly caused by copper deposition.
Concept Tested & Keywords
  • Concept Tested: Pathophysiology of Wilson's Disease
  • Stem keywords: Abnormal copper accumulation, disease condition
  • Lead-in keywords: which
  • Negative lead-in flag: false

Question ID

QZfeAGCDdHAJxc4_5eZy4N

Reference Book

E6 Robert Boland, Marcia L. Verduin - Kaplan and Sadock's Comprehensive Text of Psychiatry-Wolters Kluwer Health (2024) (pp 1-16525 of 16525) pp. 2294-2296, 1884-1886

E6 Physiology Ganong 27e Vol 1 Part 2 p. 22-24

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