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by mtarsi

B - When the Patient is Sick

i. Infections

1. Reaction to Infection

2. Risk Factors for Recurrent Infections

3. Pneumonia and respiratory compromise

Jul 02, 2008 Comments: 0
by mtarsi

Jul 02, 2008 Comments: 0
by mtarsi

Given a mitochondrial patient's severe response to infection, when cultures (bacterial or viral) are obtained as part of an infection work-up, antibiotics or anti-viral intervention should probably be started and discontinued later if cultures are negative.  When treatment is begun only when a culture turns positive 48 hours (or longer) after presentation, the patient's clinical status may have deteriorated, leading to a more prolonged course of illness and perhaps a higher risk of complications.

Jul 02, 2008 Comments: 0
by mtarsi

Questions to ponder in a mitochondrial patient who is getting sick

Jul 02, 2008 Comments: 0
by mtarsi

Many mitochondrial patients have baseline temperatures that are below normal (by as much as 2-5 degrees Fahrenheit), and even lower baseline measurements at night.  Higher-than-normal temperatures also occur.  These baselines might also be variable and should be periodically checked.   It is important to assess fever based on the increase above the patient's unique baseline.  For example, a temperature of 99.5°F may not be worrisome in a typical child but if that represents a 3-4 degree increase above a sick-looking mitochondrial patient's baseline, it is not t

Jul 02, 2008 Comments: 0
by mtarsi

Indwelling central lines offer the potential of great benefit of providing a mechanism for parenteral nutrition in patients with significant gut dysmotility or IV fluids in patients with vascular dysautonomia.  However, the risk of sepsis and other complications including clotting cannot be overstated, and clinicians and patients/parents must carefully weigh the risks and benefits in making a decision.

Jul 02, 2008 Comments: 0
by mtarsi

When bladder dysfunction occurs, patients may complain of frequency, urgency, incomplete emptying ("double-voiding"), and/or frank urinary retention.  The onset of these symptoms may be gradual, or initially may not be an issue except during periods of physiologic stress (e.g., during infections) with a return to regular functioning as the stressor recedes.  When urinary retention is significant, cystitis may occur and with reflux pyelonephritis as well.

Jul 02, 2008 Comments: 0
by mtarsi

Muscle fatigue and weakness are common manifestations of mitochondrial disease.  This can include the muscles of the chest wall and diaphragm.  Intubation and ventilation may be required to rest the patient.  With severe and/or prolonged infections, recovery may be remarkably prolonged, especially if the course of the infection has been drawn out, demanding patience and long-term, aggressive pulmonary toileting. 

Jul 02, 2008 Comments: 0
by mtarsi

Risk factors that contribute to infection in mitochondrial disease include:

Jul 02, 2008 Comments: 0
by mtarsi

During infections, the patient's baseline clinical symptoms may become more severe.  An exacerbation of myalgias, headaches or migraine, and/or sensory symptoms can occur.  Gut motility may worsen, and a reduction in calories and fluids under these conditions can further aggravate the patient's fatigue or trigger those symptoms commonly associated with poor intake (e.g., headaches, dizziness).  The reduced ability to tolerate fluids or food enterally may necessitate intravenous fluid therapy.  A reduced urine output may be due either to dehydration or urinary retenti

Jul 02, 2008 Comments: 0

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