Copper concentration of liver tissue under long-term copper-histidine therapy in a patient with Menkes disease

T Kroepfl; E Mair; J Deutsch; M Brunner-Krainz; E Paschke; B Plecko

doi:10.1007/s10545-006-0312-1

Copper concentration of liver tissue under long-term copper-histidine therapy in a patient with Menkes disease

J Inherit Metab Dis. 2006 Aug;29(4):593. doi: 10.1007/s10545-006-0312-1. Epub 2006 Jun 19.

Authors

T Kroepfl¹, E Mair, J Deutsch, M Brunner-Krainz, E Paschke, B Plecko

Affiliation

¹ Department of General Paediatrics, University Hospital Graz, Graz, Austria.

PMID: 16786254
DOI: 10.1007/s10545-006-0312-1

Abstract

Copper-histidine is the treatment of choice in Menkes disease but bears the potential risk of copper overload and induced liver cirrhosis. We report normal copper concentrations of liver tissue over an 8-year treatment period with copper-histidine.

Publication types

Case Reports

MeSH terms

Child
Child, Preschool
Copper / analysis*
Copper / blood
Copper / cerebrospinal fluid
Histidine / administration & dosage
Histidine / analogs & derivatives*
Histidine / therapeutic use
Humans
Infant
Liver / chemistry*
Menkes Kinky Hair Syndrome / drug therapy*
Menkes Kinky Hair Syndrome / metabolism*
Organometallic Compounds / administration & dosage
Organometallic Compounds / therapeutic use*
Time Factors

Substances

Organometallic Compounds
Histidine
Copper
copper histidine