The Elusive Magic Pill: Finding Effective Therapies for Mitochondrial Disorders

Association of Hearing Impairment With Incident Frailty and Falls in Older Adults

“…The relationship between frailty, disability, and comorbidity is complex and overlapping (Fried et al, 2004). Shared pathological pathways (e.g., systemic inflammation, neurodegeneration, microvascular disease) could contribute to both greater HI and onset of frailty (Dinarello, Simon, & van der Meer, 2012; Gates, Cobb, D’Agostino, & Wolf, 1993; Goldstein & Wolfe, 2013; Liew et al, 2007). Alternatively, a study by Fried et al (2009) found that older adults having a greater number of abnormal physiological systems had a greater likelihood of developing frailty.…”

Therapeutic Strategies for Mitochondrial Disorders

“…There are, however, also a number of disease-modifying remedies which are ineffective or at a stage far away from clinical applicability. 30,[109][110][111] Symptomatic measures are still the mainstay of treating mitochondrial disorders. Nevertheless, there are several reasons why therapeutic nihilism is not justified.…”

Kearns-Sayre syndrome presenting as isolated growth failure

“…The ubiquinol was given to minimise any redox block between complexes I and II or III. 15 The vitamin E was prescribed with the objective of scavenging oxygen-free radicals arising from mitochondrial dysfunction, and the creatine was given to improve muscle function through increasing muscle phosphocreatine content. 15 …”

“…α-Lipoic acid is a cofactor of the pyruvate dehydrogenase complex (PDHC) and by improving PDHC function lowers plasma lactic acid levels. 15 Carnitine functions both as an antioxidant and as a covalent acceptor of organic acids; the latter facilitates excretion of excess organic acids that accumulate from poor mitochondrial function. 15 In addition, her diet was supplemented with selenium and zinc based on low plasma taurine 42 (55-204 mmol/l) and zinc 61 (66-110 mg/dl) levels.…”

“…15 Carnitine functions both as an antioxidant and as a covalent acceptor of organic acids; the latter facilitates excretion of excess organic acids that accumulate from poor mitochondrial function. 15 In addition, her diet was supplemented with selenium and zinc based on low plasma taurine 42 (55-204 mmol/l) and zinc 61 (66-110 mg/dl) levels. Within 3 months, she had less fatigue, greater strength, improved weight gain, more tolerance for cold and warm temperatures, increased sweating and an improved sense of well-being.…”