DRUG | SUPPLIED | DOSE | COMMENTS |
---|---|---|---|
PENICILLAMINE (Paeds) (Cuprimine) Mechanism of action: Heavy metal antagonist; chelating agent. Ref: 4, 44, 445 Last update: 2019-08-13 |
capsule: 250 mg A low usage item; not stocked at BCCH but can be obtained in 2 to 3 days. |
Wilson's Disease: Infants & children: 10 mg/kg/dose PO BID or 7 mg/kg/dose PO TID. Adults: 500-750 mg PO BID or 250-500 mg/dose PO TID. Maximum 2 g/24 hours Cystinuria: Infants/Young Children: 7.5 mg/kg/dose PO QID. Maximum 4 g/24 hr Older Children/Adults: 500 mg/dose PO QID (1-4 g/24 hr) |
Penicillamine has been associated with serious hematological and renal adverse effects. Routine monitoring of CBC and urinalysis Q1-2 weeks during first six months of therapy is recommended. Dose adjustment is based on 24 hour urinary copper excretion (Wilson's Disease) or urinary cysteine excretion (cystinuria). Give on empty stomach - give doses one hour before or 2 hours after meals. Avoid concomitant iron, antacids and milk. Patients treated for Wilson's Disease or cystinuria should be supplemented with pyridoxine 25 - 50 mg per day. Standard Prescription: penicillamine __mg PO Q__H (__mg/kg/24 hr) |