title: Myocardial dysfunction in mitochondrial diabetes treated with Coenzyme Q10.
abstract: Maternally-inherited diabetes and deafness (MIDD) has been related to an A to G transition in the mitochondrial tRNA Leu (UUR) gene at the base pair 3243. Although some previous articles have reported that this mutation may be a cause of cardiomyopathy in diabetes, the degree of cardiac involvement and a specific treatment has not been established. Here, we reported a case of a patient with MIDD who developed congestive heart failure and the therapeutic usefulness of Coenzyme Q10 (CoQ10). In our patient, after the introduction of Coenzyme Q10 150 mg/day, there was a gradual improvement on left ventricular function evaluated by echocardiography. The fractional shortening (FS) and ejection fraction (EF) increased from 26 to 34% and from 49 to 64%, respectively. No side effects were noted. Three months after CoQ10 discontinuation, the parameters of systolic function evaluated by echocardiography decreased, suggesting that CoQ10 had a beneficial effect. Identification of diabetes and cardiomyopathy due to mitochondrial gene mutation may have therapeutic implications and Coenzyme Q10 is a possible adjunctive treatment in such patients.
authors.affiliation: Division of Endocrinology, Department of Medicine, Federal University of São Paulo, Rua Botucatu, 740 0434-970 São Paulo, SP, Brazil.
authors.forename: João Eduardo
authors.initials: JE
authors.lastname: Salles
authors.forename: Valdir A
authors.initials: VA
authors.lastname: Moisés
authors.forename: Dirceu R
authors.initials: DR
authors.lastname: Almeida
authors.forename: Antonio R
authors.initials: AR
authors.lastname: Chacra
authors.forename: Regina S
authors.initials: RS
authors.lastname: Moisés
mesh_terms: D000328:Adult; D000975:Antioxidants / Q000627:therapeutic use; D003067:Coenzymes; D004272:DNA, Mitochondrial / Q000235:genetics; D016883:Diabetic Ketoacidosis / Q000150:complications / Q000188:drug therapy*; D005260:Female; D006321:Heart / Q000503:physiopathology*; D006334:Heart Function Tests; D006801:Humans; D007328:Insulin / Q000627:therapeutic use; D020125:Mutation, Missense; D012356:RNA, Transfer, Leu / Q000235:genetics; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use; D014927:Wolff-Parkinson-White Syndrome / Q000150:complications
chemical_list: D000975:Antioxidants; D003067:Coenzymes; D004272:DNA, Mitochondrial; D007328:Insulin; D012356:RNA, Transfer, Leu; D014451:Ubiquinone; C024989:coenzyme Q10