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Triethylene tetramine (trientine)

Wilson disease in cases of penicillamine intolerance.

500-750 mg/ day for pediatricpatients and 750-1250 mg/day for adults given in divided doses two, three or four times daily. This may be increased to a maximum of 2000 mg/day for adults or 1500 mg/day for children.  Adjust the dose by determination of free copper in the serum, which equals the difference between quantitatively determined total copper and  ceruloplasmin.

Adequately treated patients will usually have less than 10 mcg free copper/dL of serum (20, 21).

250 mg capsules

PO

Myasthenia gravis, spasticity, dystonia, contact dermatitis.

1b

20. Torres RJ, Prior C, Puig JG. Efficacy and safety of allopurinol in patients with hypoxanthine-guanine phosphoribosyltransferase deficiency. Metabolism 2007;56(9):1179-86 doi: S0026-0495(07)00150-3 [pii] 10.1016/j.metabol.2007.04.013[published Online First: Epub Date]|.

21. Cameron JS, Moro F, Simmonds HA. Gout, uric acid and purine metabolism in paediatric nephrology. Pediatr Nephrol 1993;7(1):105-18

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