Vitamin E in a mitochondrial myopathy with proliferating mitochondria

Department of Biochemistry , Erasmus Universiteit Rotterdam, Rotterdam, South Holland, Netherlands
The Lancet (Impact Factor: 45.22). 08/1993; 342(8864):175-6. DOI: 10.1016/0140-6736(93)91379-Z
Source: PubMed
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    • "There is some controversy regarding this approach as a systematic review failed to provide clear evidence supporting the use of CoQ10, creatine, dichloroacetate, and dimethylglycine in mitochondrial disorders [4] [5] [6]. However, a partial improvement in clinical features using anti-oxidants including coenzyme Q10, idebenone (a coenzyme Q10 analog), vitamin E, and dihydrolipoate has been reported [4] [5] [6]. Patients with mitochondrial cytopathies taking creatine monohydrate show increased strength of highintensity anaerobic and aerobic type activities [7]. "
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    ABSTRACT: Mitochondrial disorders are genetic diseases that result in a deficiency of energy metabolism (ATP production). A “mitochondrial crisis” can occur in the setting of infection, dehydration, or physiologic stress. The hallmark of a mitochondrial crisis is failure of multiple individual organ systems. The mortality of mitochondrial crisis is high and therapy is supportive but involves a specific strategy of hydration with dextrose-containing IV fluids, avoidance of many medications known to worsen mitochondrial function, and limitations of oxygenation as this can promote free radical production. We report a case of a patient with known mitochondrial disease that presented with a mitochondrial crisis with prominent and life-threatening cardiac manifestations including long QT, ventricular arrhythmias, and acute left ventricular systolic dysfunction in addition to rhabdomyolysis, lactic acidosis, and an acute kidney injury. This patient was managed successfully with a specifically tailored supportive strategy, a high-dose metabolic cocktail, permissive hypoxia, and low-protein diet. At 10 weeks post discharge all electrocardiographic abnormalities resolved and ventricular recovery has been observed. Given the increased survival of this population of patients into adulthood it is important that these adjunctive therapeutic strategies require consideration by clinicians treating this group of patients.
    Preview · Article · Aug 2012 · Journal of Cardiology Cases
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    • "Although the diagnosis itself of the patient was controversial, the effect of this therapeutic approach was dramatic and cardiac condition almost fully recovered at least in this case. Although it will be difficult to clarify the role of each material in this unique cocktail in mitochondrial diseases, this kind of " broad approach " might be effective in various types of mitochondrial diseases [12] [13] [14] and may show a novel pharmacological approach to mitochondrial diseases . However, strong attention should be paid to the dosage and method of administration of these materials because they can cause various types of side effects, including hypotension, peripheral vascular injury, cardiogenic shock, or life-threatening arrhythmias. "

    Preview · Article · Aug 2012 · Journal of Cardiology Cases
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    ABSTRACT: The computational model for skeletal muscle energetics and oxidative phosphorylation is derived from a previously published model applied to cardiac tissue (5). For the current application the cardiac model has been modified in two ways to adapt the model to analyze data from human skeletal muscle. First, the oxygen transport component of the previous model has been removed. It is assumed that the skeletal muscle remains normoxic during the experiments and the cellular oxygen concentration, (O2), which is a variable in the model of Beard (5), appears as a fixed parameter in the current model. Second, the mitochondrial volume of the muscle cell, which is approximately 30% of cell volume in cardiomyocytes (27), is set to Vmito = 0.056 (l mito) (l cell) -1 , a value measured
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