A 22-year-old man presents with tremor, dysarthria, psychiatric symptoms, and elevated serum copper. Slit-lamp examination reveals Kayser-Fleischer rings. Serum ceruloplasmin is 8 mg/dL. He is diagnosed with Wilson's disease. Which initial treatment is preferred for symptomatic Wilson's disease with neurological involvement?
- A D-penicillamine
- B Zinc acetate monotherapy
- C Ammonium tetrathiomolybdate
- D Trientine (triethylene tetramine) ✓
Explanation
Current European Association for Study of the Liver (EASL 2022) guidelines prefer trientine over D-penicillamine as first-line for newly diagnosed symptomatic Wilson's disease, particularly neurological presentation, because D-penicillamine can precipitate neurological worsening in 25–50% of neurologically symptomatic patients. Trientine chelates copper with less risk of neurological deterioration. Zinc maintains copper balance and is used for maintenance or asymptomatic patients. Ammonium tetrathiomolybdate is under investigation as an alternative chelator with lower neurological deterioration risk.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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