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by mtarsi

During sepsis, physiologic changes in pH and temperature and structural changes in the respiratory chain enzymes impair cellular bioenergetic capacity which can lead to organ dysfunction and failure (Muravchick, 2006).  In the setting of sepsis, there is depression of the microcirculation causing regional hypoxia and problems with oxygen extraction by the cell even when systemic oxygenation is maintained.  During hypoxia, cells may adopt a "hibernation-like state."  Initially this state is reversible but if the septic stress is prolonged and the microcirculation left unco

Jul 02, 2008 Comments: 0
by mtarsi

Axelrod FB, Chelimsky G, Weese-Mayer DE.  Pediatric autonomic disorders.  Pediatrics 2006;118:309-21.Bennett MJ, Sherwood WG, Gibson KM, Burlina AB.  Secondary inhibition of multiple NAD-requiring dehydogenases in respiratory complex I deficiency: Possible metabolic markers for the primary defect.  J Inher Metab Dis 1993;16:560-62.Davis MD, Sandroni P, Rooke T, Low P.  Arch Derm 2002;139(10):1337-43.

Jul 02, 2008 Comments: 0
by mtarsi

An under-appreciated aspect of mitochondrial disease is the autonomic dysfunction (Zelnik, 1996; Axelrod, 2006) which can impact life on a daily basis. Patients often have some dysregulation of temperature, in which the baseline body temperature often measures in the 96s and 97s and sometimes lower; temperatures can drop even lower during the night. Less often, temperature values at baseline run higher than normal. This is a particularly important piece of information when assessing a mitochondrial patient who is sick with infectious symptoms.

Jul 02, 2008 Comments: 0
by mtarsi

A restorative process, adequate sleep (in its duration and quality) is a critically important factor in the wellbeing of patients with energy problems.  Inadequate sleep can be associated with fatigue, weakness, lethargy, behavioral issues, and difficulty with memory, learning, and concentration.  Such a scenario can exacerbate the severity of symptoms that a patients typically experiences.

Jul 02, 2008 Comments: 0
by mtarsi

Most patients with mitochondrial disease don't have an immunodeficiency.  However the stress of infectious illnesses can be associated with a prolonged period of debilitating weakness and fatigue.  Therefore preventive measures to reduce the chance of infections are worthwhile.  This can include routine and seasonal vaccinations.  The fever and discomfort associated with inoculations could be difficult for some patients with energy disorders to endure.  These can be effectively treated using prophylactic anti-pyretics.

Jul 02, 2008 Comments: 0
by mtarsi

When calorie intake drops, as during infections or with prolonged fasting, glycogen is first mobilized to provide glucose as a source of energy.  When glycogen stores are used up, gluconeogenesis and fatty acid ß-oxidation are initiated.  A proportion of patients with primary mitochondrial disease show secondary fatty acid ß oxidation dysfunction (Bennett, 1993; Hagenfeldt, 1998; Enns, 2000).  This may present as fatigue or lethargy or vomiting with prolonged fasting or long periods with inadequate calorie intake.  When an affected patient becomes ill, energy requireme

Jul 02, 2008 Comments: 0
by mtarsi

Inadequate fluid intake, if persistent and significant, can result in dehydration and associated fatigue and/or malaise.  However, patients with autonomic dysregulation are at risk for vascular dysautonomia and may experience orthostatic changes in blood pressure and heart rate.  An inadequate intake of fluids can result in dizziness or lightheadedness, syncope, and significant fatigue, and may be associated with chronic nausea and vomiting, as well as an increase in migraine frequency and severity.

Jul 02, 2008 Comments: 0
by mtarsi

With few exceptions, there is no specialized dietary treatment for mitochondrial disease although some recommend a lower carbohydrate diet (Munnich, 2006).  In pyruvate dehydrogenase complex deficiency, implementation of a ketogenic diet may provide some biochemical stability and when implemented in early infancy might improve long term neurologic outcome (Wexler, 1997).  

Jul 02, 2008 Comments: 0
by mtarsi

Patients with mitochondrial disease have many reasons for not eating well, including GI issues, neurologic disease, and metabolic instability.  The result is the same - an inappropriately low intake of calories and nutrients.  This can result in fatigue and lethargy; when persistent, the patient's weakened state can appear as if the disease course is progressing or accelerating.  Furthermore, a malnourished or undernourished condition can be associated with an exacerbation of symptoms (such as muscular pain, headaches, and/or migraine).  

Jul 02, 2008 Comments: 0
by mtarsi

Jul 02, 2008 Comments: 0

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