Treatment-Wilson disease
chelation w/ D-penicillamine (supplement B6 as D-pen inactivates); alternative is trientine (↓ toxicity w/ ≈ efficacy, but $$), ammonium or bis-choline tetrathiomolybdate (investigational) may ↓ neurologic deterioration compared to trientine. Zinc: ↓ intestinal Cu transport & can help delay disease; best used in conjunction w/ chelation (give 5 h after chelators). Eliminate Cu-rich foods. Transplant for ALF or unresponsive to Rx.