title: Monitoring cardiac function during idebenone therapy in Friedreich's ataxia.
abstract: Friedreich's ataxia (FA) is associated with progressive cardiac hypertrophy resulting from a genetic abnormality in the frataxin gene. Cardiac involvement is the most common cause of death (59%) in FA patients. Cardiac related death occurs at a significantly younger age than non-cardiac related death. Idebenone is a short-chain quinone analogue with a potent free-radical scavenger action. This drug has the potential to preserve and even improve mitochondrial function.Studies on Idebenone treatment showed rather conflicting results on FA cardiomyopathy. The present article reviews the clinical features of FA cardiomyopathy, imaging techniques used to diagnose, follow and monitor therapy which aimed to revert FA cardiomyopathy.
authors.forename: Di Salvo
authors.initials: DS
authors.lastname: Giovanni
authors.forename: Pergola
authors.initials: P
authors.lastname: Valeria
authors.forename: Fadel
authors.initials: F
authors.lastname: Bahaa
authors.affiliation: HEART CENTRE (Dpt) - F, P.O. Box 3354 Riyadh 11211 MBC - 16, Saudi Arabia. giodisal@yahoo.it.
authors.forename: Al Fayyadh
authors.initials: AF
authors.lastname: Majid
mesh_terms: D005621:Friedreich Ataxia / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology*; D006321:Heart / Q000187:drug effects / Q000503:physiopathology; D006334:Heart Function Tests / Q000187:drug effects*; D006801:Humans; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use
chemical_list: D014451:Ubiquinone; C036619:idebenone