Mitochondrial disease

Inherited disorders of the mitochondrial respiratory chain affecting energy-dependent tissues: muscle, brain, heart and liver. Many clinical forms exist – MELAS, MERRF, Leigh syndrome, Kearns-Sayre. No specific therapy; «mitochondrial cocktails» of varying evidence are used.

ICD-10 G71.31 drug

Also known as: Mitochondrial myopathy, MELAS, MERRF, Leigh syndrome

Marketing claims without evidence base

The drug is promoted for these uses outside international guidelines. Each entry below is analyzed against AEMPS, FDA, EMA, Cochrane and major RCTs.

DD-riboseNot recommended · Pentose monosaccharide; complemento alimenticio with a claimed role in energy metabolismD-ribose is included in empirical mitochondrial cocktails for MELAS, MERRF and other mitochondriopathies based on a theoretical role in ATP synthesis. For mitochondrial diseases themselves there are no controlled RCTs with D-ribose, and clinical guidelines do not list it. In most mitochondrial patients standard support relies on coenzyme Q10, riboflavin, L-carnitine and thiamine. D-ribose was separately studied in myoadenylate deaminase deficiency (AMPD1), a different, non-mitochondrial disorder, see its own card.

How to read tiers A–F

Evidence tiers belong to the drug-indication pair, not to the drug as a whole: the same substance can be A for one condition and F for another.

AStrong evidence – first or second line in international guidelines
BRecommended in selected cases
CStudy data contradict each other
DBenefit not proven, no documented harm
FDocumented harm, or risk without benefit