Copper metabolism and pediatric cholestasis.

Objective: Copper is an essential trace mineral but both deficiency and toxicity need to be avoided. Copper is regulated via excretion by the biliary system and caution was recommended when administered in patients with cholestasis. Recent clinical reports indicate that despite the cholestasis, copper should not be withheld from parenteral nutrition.

Results: Transporters involved in regulating copper levels have been identified. This explains the processes that regulate copper levels and the diseases that result from transporter defects. Monitoring copper ideally requires a liver biopsy but there are reports that in infants serum copper levels correlate with the liver copper. The published cautions about copper in cholestatic patients on parenteral nutrition led to the removal of copper from the solutions. Subsequently, multiple reports of clinical copper deficiency developing in these patients including infants were published. Newer literature indicates no elevation in infant copper levels despite normal copper parenteral nutrition supplementation in the presence of cholestasis.

Conclusions: Copper is essential and levels are regulated in response to an individual's needs. The liver excretion of copper is the primary regulating method but clinically cholestasis does not result in elevated levels in infants. The best clinical approach to parenteral nutrition copper is careful monitoring even in the presence of cholestasis.