A 22-year-old woman presents with jaundice, behavioral changes, and kayser-Fleischer rings on slit-lamp examination. Serum ceruloplasmin is 8 mg/dL (low). 24-hour urine copper is 280 μg/day. LFTs show AST 320, ALT 290, bilirubin 9.2 mg/dL. She is diagnosed with Wilson's disease and started on D-penicillamine. Which critical monitoring is required early in treatment?
- A Serum ferritin for iron overload
- B Pulmonary fibrosis from D-penicillamine
- C Neurological worsening (paradoxical deterioration) ✓
- D Cardiac arrhythmias from copper mobilization
Explanation
A well-recognized and clinically important adverse effect of initiating D-penicillamine in Wilson's disease, particularly when neurological symptoms are present, is paradoxical neurological worsening in approximately 10–50% of patients. This occurs due to acute copper redistribution from the liver into the systemic circulation and CNS. This deterioration may be irreversible in some patients, prompting many experts to prefer zinc or trientine as first-line therapy in neurological Wilson's disease. The risk is substantially lower with trientine. Pyridoxine supplementation is required with D-penicillamine to prevent vitamin B6 antagonism.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.