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12 Ιουν 2024 · Mitochondrial disease results from failure of mitochondria to function properly. This can lead to less energy, cell injury and cell death. The most common organs that may experience damage are the brain, heart, liver, muscles, kidneys and the endocrine system.
Someone with Mitochondrial myopathy who is generally on a high protein, low sugar diet finds that when she needs an IV, switching to dextrose (sugar) and water rather than saline makes her feel better - why is this?
Safe and effective use of the ketogenic diet has been reported in patients with RC defects; in one study, 10 of 14 patients placed on the ketogenic diet were seizure free or had a greater than 50% reduction in seizures .
Diet: Dietary needs are extremely variable from patient to patient with mitochondrial myopathies. It is required to evaluate a patient’s nutritional deficiencies. Potential therapies include supplementing caloric intake, enteral feeding, increasing the frequency of meals, limited fasting, and parenteral nutrition ( Parikh et al., 2009 ).
In five patients with mitochondrial myopathy associated with complex I deficiency, 9 mg/day for patients younger than 12 years and 36 mg/day for older patients for 3–17 months improved muscle strength in two patients and reduced fatigue in one patient.
26 Νοε 2015 · Overall, these recent findings support the original view that mtDNA mutations are the major cause of mitochondrial disease in adults, but demonstrate clearly the importance of a diverse array of nuclear gene defects that can present to the clinic in adult life.
14 Φεβ 2024 · We performed a retrospective chart review of adult patients diagnosed with mitochondrial myopathy at Mayo Clinic (2005–21). We identified 94 patients. Median time from symptom onset to diagnosis was 11 years (interquartile range 4–21 years). Median age at diagnosis was 48 years (32–63 years).
