Contradictory outcomes have been described regarding the effect of supplementation with the CoQ10 analogue idebenone on cardiac function or neurological function in younger Friedreich’s ataxia patients. Thus, in an open study, supplementation with idebenone (5 mg/kg/day for 6 months) in eight children (age range 4–12 years) with Friedreich’s ataxia resulted in improved cardiac indices (left ventricular mass, left ventricular posterior wall thickness, shortening fraction). An open study of Friedreich’s ataxia patients, including 15 children (ages 4–12 years), found improved cardiac indices (left ventricular posterior wall thickness, interventricular septum thickness) following supplementation with idebenone (20 mg/kg/day). However, in a randomised controlled trial comprising 70 paediatric patients, supplementing idebenone (450–2250 mg/day for 6 months) did not result in any significant benefit on left ventricular hypertrophy or other cardiac function parameters.
Similarly, with regard to neurological function, an open study by Pineda et al. comparing younger (ages 8–18 years) and adult (ages 18–46 years) patients with Friedreich’s ataxia found supplementation with idebenone (5–20 mg/kg/day for 3–5 years) stabilised neurological function in younger subjects, but not in adults. However, a randomised controlled trial supplementing idebenone (450–2250 mg/day for 6 months) in a series of 70 children/adolescents (aged 8–18 years) did not result in any significant improvement in neurological function. In addition, a randomised controlled trial, supplementation with idebenone (5–45 mg/kg/day for 6 months) in a cohort of 42 children/adolescents (aged 9–17 years) with Friedreich’s ataxia did not result in any improvement in exercise capacity.
Monday, June 3, 2024
Efficacy and Safety of Coenzyme Q10 Supplementation in Neonates, Infants and Children: An Overview
Mantle D, Hargreaves IP. Efficacy and Safety of Coenzyme Q10 Supplementation in Neonates, Infants and Children: An Overview. Antioxidants (Basel, Switzerland). 2024 Apr;13(5):530. DOI: 10.3390/antiox13050530. PMID: 38790635; PMCID: PMC11117623.
