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The efficacy of coenzyme Q10 treatment in alleviating the symptoms of primary coenzyme Q10 deficiency: A systematic review

Ying Wang, Siegfried Hekimi
Journal of Cellular and Molecular Medicine 2022 26(17):4635-4644

Bibliography

PubMed
PMID 35985679
PubMed Central
PMC9443948
Funding
Detailed funding is reported in the full text; the PubMed abstract does not summarize it.
Competing interests
Detailed disclosures are reported in the full text; the PubMed abstract does not summarize them.

Study snapshot

DesignSystematic review of primary CoQ10-deficiency case reports and series
ModelGenetically defined primary CoQ10 deficiency
Sample303 patients identified; 89 evaluable treated cases; 24 reported improvement
InterventionOral CoQ10 replacement therapy
DurationVaried widely across reports
EndpointsOverall symptomatic response; Ataxia; Proteinuria; Partial symptom-specific improvement; Response after stopping therapy

What the study showed, in plain terms

Primary CoQ10 deficiency is a real genetic mitochondrial disorder in which CoQ10 replacement is biologically logical. Yet this systematic review found reported improvement in only 24 of 89 evaluable treated cases, usually partial and symptom-specific, showing that the clinical evidence is much weaker than the replacement rationale might suggest.

Key findings

Twenty-four of 89 evaluable cases (27.0%) reported improvement. Ataxia improved in 12 reported cases and proteinuria in five. All responses were partial, and evidence quality was very weak.

What this study can and cannot tell us

Case-report evidence, heterogeneous genes and phenotypes, inconsistent doses and durations, publication bias and lack of standardized controls severely limit certainty.

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