Copper concentration of liver tissue under long-term copper-histidine therapy in a patient with Menkes disease.
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.
Kroepfl
T
T
Department of General Paediatrics, University Hospital Graz, Graz, Austria.
Mair
E
E
Deutsch
J
J
Brunner-Krainz
M
M
Paschke
E
E
Plecko
B
B
D002648:Child; D002675:Child, Preschool; D003300:Copper / Q000032:analysis* / Q000097:blood / Q000134:cerebrospinal fluid; D006639:Histidine / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008099:Liver / Q000737:chemistry*; D007706:Menkes Kinky Hair Syndrome / Q000188:drug therapy* / Q000378:metabolism*; D009942:Organometallic Compounds / Q000008:administration & dosage / Q000627:therapeutic use*; D013997:Time Factors
D009942:Organometallic Compounds; D006639:Histidine; D003300:Copper; C098468:copper histidine