Inborn errors of metabolism:
Indications for SYPRINE:
Wilsons disease in patients who are intolerant of penicillamine.
Swallow whole. Take on an empty stomach. 750–1250mg/day in 2–4 divided doses; max 2000mg/day. Increase dose if clinical response not adequate or free serum copper is persistently >20mcg/dL. Determine maintenance dose at 6–12 month intervals.
<6yrs: not recommended. Swallow whole. Take on an empty stomach. 6–12yrs: 500–750mg/day in 2–4 divided doses; max 1500mg/day. Increase dose if clinical response not adequate or free serum copper is persistently >20mcg/dL. Determine maintenance dose at 6–12 month intervals.
Not for treatment of biliary cirrhosis. Monitor for iron deficiency anemia (esp. women). Monitor for symptoms of fever or skin eruption; take body temperature nightly during 1st month of treatment. Any site of exposure to capsule contents must be washed with water. Do 24-hour urinary copper analysis periodically (eg, every 6–12 months). Pregnancy (Cat.C). Nursing mothers.
Absorption may be inhibited by mineral supplements; separate dosing of iron by at least 2 hours. Separate dosing of any other drug, food, or milk by at least 1 hour.
Iron deficiency, systemic lupus erythematosus, dystonia, muscular spasm, myasthenia gravis, contact dermatitis.