treatment of mitochondrial cytopathies

نویسندگان

marjan shakiba pediatric endocrinology and metabolism department, mofid children hospital, faculty of medicine, shahid beheshti university of medical sciences, tehran, iran

چکیده

pls see pdf.

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Commentary: Human mitochondrial cytopathies.

Mitochondria provide energy (ATP) for all eukaryotic cells except mature erythrocytes and keratinocytes. They are abundant in cells that expend much energy, such as muscle, exocrine pancreas, nervous system, and heart cells, and motile sperm. Many mitochondrial enzymes are encoded by nuclear DNA and imported into the mitochondria. Like bacteria, mitochondria possess their own DNA and ribosomes....

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Mitochondrial respiratory chain disorders account for significant and varied presentations in paediatric practice. The true prevalence of these disorders in the paediatric population is still not well documented with predicted geographic variation. We report a retrospective analysis over a seven year period of cases presenting to a tertiary care centre and associated clinical features. The over...

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Mitochondrial cytopathies ultimately lead to a reduction in aerobic energy transduction, depletion of alternative energy stores, increased oxidative stress, apoptosis and necrosis. Specific combinations of nutraceutical compounds can target many of the aforementioned biochemical pathways. Antioxidants combined with cofactors that can bypass specific electron transport chain defects and the prov...

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Acute tubular dysfunction with Fanconi syndrome: a new manifestation of mitochondrial cytopathies.

S b es itochondrial diseases are a heterogeneous group of energy metabolism disorders hat can affect almost any organ. Kidney disease s well-known in patients with mitochondrial isorders, most commonly manifesting as proxial tubulopathy with chronic tubular acidosis, ypically a minor feature in pediatric patients ith severe involvement of brain, liver, heart, or ther organs. Other renal present...

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عنوان ژورنال:
iranian journal of child neurology

جلد ۷، شماره ۴، صفحات ۱۶-۱۷

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