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

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.
AuthorsT Kroepfl, E Mair, J Deutsch, M Brunner-Krainz, E Paschke, B Plecko
JournalJournal of inherited metabolic disease (J Inherit Metab Dis) Vol. 29 Issue 4 Pg. 593 (Aug 2006) ISSN: 1573-2665 [Electronic] United States
PMID16786254 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Organometallic Compounds
  • Histidine
  • Copper
  • copper histidine
Topics
  • Child
  • Child, Preschool
  • Copper (analysis, blood, cerebrospinal fluid)
  • Histidine (administration & dosage, analogs & derivatives, therapeutic use)
  • Humans
  • Infant
  • Liver (chemistry)
  • Menkes Kinky Hair Syndrome (drug therapy, metabolism)
  • Organometallic Compounds (administration & dosage, therapeutic use)
  • Time Factors

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