Parents of a 16-year-old child who has tremors and trouble walking bring him to the office. His academic performance has dropped over the past year, and he has become moodier and more aggressive. His parents also observe that he has been sleeping more than normal, and that for the past two months, he hasn't been eating well. The patient's tremor in both hands started around three weeks ago, and it is most noticeable when he reaches for something or tries to write. Over the past week, the tremor has dramatically deteriorated, and he has started to walk with an uneven, wide-based gait. Elevated serum transaminases and 24-hour urine copper excretion are seen in laboratory tests. Therapy with penicillamine is indicated for the patient. This medication primarily works through which of the following mechanisms?

Question

Quizplus · Accepted Answer

Eductional Objective: First-line treatment of Wilson disease is with penicillamine, a copper chelating agent. Penicillamine binds free copper as well as reduces copper bound to other proteins. This reduction preferentially increases affinity of copper to the chelating agent. The chelator-copper complex is soluble, thereby increasing urinary copper excretion.

Parents of a 16-Year-Old Child Who Has Tremors and Trouble