Internal Medicine

0521779407-23 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:24

1556 Wilson’s Disease

Treatment Options

■D-penicillamine: give vitamin B6 also

➣Initial therapy: 250–500 mg PO daily; gradually increase to 1,000–

1,500 mg daily, in 3–4 divided doses

➣Maintenance: 750–1,250 mg daily, in 3–4 divided doses

■Trientine

➣Initial therapy: 1–2 grams PO daily, in 3–4 divided doses

➣Maintenance: 750–1,000 mg daily, in 3–4 divided doses

■Zinc salts

➣Maintenance: 150 mg PO daily, in 3 divided doses

■Ammonium tetrathiomolybdate

➣Initial therapy: 120–420 mg per day for 8 weeks

➣Maintenance: use zinc salts

Side Effects & Contraindications

■D-penicillamine

➣Side effects: hypersensitivity reaction, bone marrow suppression,

proteinuria, lupus-like reaction, myasthenia, may worsen neu-

rologic symptoms, pemphigoid-type skin reaction

➣Contraindications

Absolute: history of penicillamine-related aplastic anemia or

agranulocytosis

Relative: concomitant therapy with gold salts, antimalarial or

cytotoxic drugs, oxyphenbutazone, phenylbutazone

■Trientine

➣Side effects: sideroblastic anemia, rhabdomyolysis

➣Contraindications

Absolute: known hypersensitivity

■Zinc salts

➣Side effects: gastric intolerance, headache

➣Contraindications: none

■Ammonium tetrathiomolybdate

➣Side effects: bone marrow suppression, elevation of aminotrans-

ferases

follow-up

During Treatment & Routine

■With penicillamine and trientine, aim for 24-hour urine copper >250

mcg per day or serum free copper <0.1 mg/L, checking every 3–6

months; with zinc therapy, urine copper usually <150 mcg per day &

urine zinc >1,000 mcg per day.