coenzyme-q10 and Acidosis

coenzyme-q10 has been researched along with Acidosis* in 1 studies

Other Studies

1 other study(ies) available for coenzyme-q10 and Acidosis

ArticleYear
Successful treatment of infantile-onset ACAD9-related cardiomyopathy with a combination of sodium pyruvate, beta-blocker, and coenzyme Q10.
    Journal of pediatric endocrinology & metabolism : JPEM, 2019, Oct-25, Volume: 32, Issue:10

    Mitochondrial acyl-CoA dehydrogenase 9 (ACAD9) deficiency is one of the common causes of respiratory chain complex I deficiency, which is characterized by cardiomyopathy, lactic acidemia, and muscle weakness. Infantile cardiomyopathy is the most common phenotype and is usually lethal by the age of 5 years. Riboflavin treatment is known to be effective in ~65% of the patients; however, the remaining are unresponsive to riboflavin and are in need of additional treatment measures. In this report, we describe a patient with ACAD9 deficiency who developed progressive cardiomyopathy at 8 months of age. As the patient's left ventricular ejection fraction (LVEF) kept decreasing to 45.4% at 1 year 8 months, sodium pyruvate treatment was introduced together with a beta-blocker and coenzyme Q10. This resulted in a steady improvement, with full and sustained normalization of cardiac function without riboflavin. The therapy, therefore, might be a useful addition for the treatment of ACAD9 deficiency.

    Topics: Acidosis; Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenases; Adrenergic beta-Antagonists; Amino Acid Metabolism, Inborn Errors; Cardiomyopathies; Cardiomyopathy, Hypertrophic; Carvedilol; Drug Therapy, Combination; Female; Humans; Infant, Newborn; Mitochondrial Diseases; Muscle Weakness; Prognosis; Pyruvates; Ubiquinone; Vitamins

2019