title: [Treatment of mitochondrial diseases in childhood and adolescence].
abstract: The advances made in recent years in understanding this large group of disorders has led to the development of ways of approaching their treatment, although in most cases it is neither definitive nor curative. In this article we review current aspects of the treatment of defects: of substrate transport, substrate use, the Krebs cycle, the mitochondrial respiratory chain and oxidation-phosphorylation coupling. We conclude with comments on possible future treatment.
authors.affiliation: Departamento de Pediatría, Hospital General de Galicia, Complejo Hospitalario Universitario de Santiago de Compostela, Sergas, España. pdcastro@uscmail.usc.es
authors.forename: M
authors.initials: M
authors.lastname: Castro-Gago
authors.forename: M I
authors.initials: MI
authors.lastname: Novo-Rodríguez
authors.forename: J
authors.initials: J
authors.lastname: Eirís-Puñal
mesh_terms: D000293:Adolescent; D002331:Carnitine / Q000378:metabolism; D002334:Carnitine O-Palmitoyltransferase / Q000378:metabolism; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D017240:Mitochondrial Myopathies / Q000178:diet therapy* / Q000188:drug therapy* / Q000201:enzymology; D009097:Multienzyme Complexes / Q000378:metabolism; D011687:Purines / Q000378:metabolism
chemical_list: D009097:Multienzyme Complexes; D011687:Purines; D002334:Carnitine O-Palmitoyltransferase; D002331:Carnitine