DDSEP® 8 Quick Quiz

September 2020 - Quick Quiz Question 1

Q1. A 19-year-old male with elevated liver enzymes is found to have a serum ceruloplasmin of 3 mg/L, and a urine copper excretion of 210 mcg/24 hours. He is started on trientine to treat his illness.

What level of urine copper is the goal of this therapy? 

No urine copper should be found if therapy is effective

Urine copper should be 200-500 mcg/24 hours

Urine copper should be 500-1000 mcg/24 hours

Urine copper should be 1000-1500 mcg/24 hours

Urine copper should be more than 1500 mcg/24 hours

Q1: Correct answer: B

Rationale
A serum ceruloplasmin less than 5 mg/L and a 24-hour urine copper excretion greater than 100 mcg/24 hours are both highly suggestive of Wilson's disease, a disorder of copper metabolism caused by a mutation in a P-type ATP-ase that mediates the excretion of copper into the bile. Treatment of Wilson's disease consists of copper chelation therapy. Commonly used therapies include D-penicillamine, trientine, and zinc. Patients on therapy should have 24-hour urine copper determination every 6-12 months. Patients on maintenance trientine or D-penicillamine should have urine copper excretion of 200-500 mcg/24 hours. Patients on zinc therapy should have much lower copper excretion, in the range of 75 mcg/24 hours.

References
1. European Association for the Study of the Liver. EASL clinical practice guidelines: Wilson's disease. J Hepatol. 2012;56:671-85.
2. Roberts EA, Schilsky ML. Diagnosis and treatment of Wilson disease: An update. Hepatology. 2008;47:2089-111.

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