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Abnormalities In The Urine

Abnormalities in the urine can occur for one of several reasons:


  1. Concentrated -
    1. Appropriate, due to low fluid intake
    2. Inappropriate, due to autonomic dysregulation. If there is a history of autonomic dysfunction +/- a history of adequate fluid intake, the patient might have a higher fluid requirement. Assess urine concentration and provide extra fluids (either enterally or intravenously). If the patient's gut cannot accommodate an appropriate increase in fluid (because of limited motility), IV fluids should be considered. Infusions of fluid over several hours might provide a long-lasting effect than boluses over 1-2 hours.
  2. Hematuria -
    1. Consider urinary retention and stasis with the risk of infection.
    2. Myoglobinuria may show as hematuria on a urinalysis dipstick without an increase in red cells on microscopic analysis.
  3. Myoglobinuria -
    1. A subset of patients with mitochondrial myopathy will manifest myoglobinuria (DiMauro, 1999), usually in the presence of some physiologic stress (e.g., infection) or following strenuous exercise or over-exertion.


  1. Cells -
    1. Infections
    2. Kidney disease due to mitochondrial disease
  2. Glycosuria -
    1. Kidney disease due to mitochondrial diabetes mellitus
    2. Renal Fanconi syndrome
  3. Proteinuria -
    1. Kidney disease due to mitochondrial disease
  4. Ketonuria -
    1. Appropriate (with low calorie intake or with fasting)
    2. Inappropriate. Ketone production is a mitochondrial function. In some cases, ketone production is not properly regulated and a post-prandial (paradoxical) ketosis may be observed ( ). This implies significant mitochondrial dysfunction.
  5. High pH -
    1. Renal Fanconi syndrome


  1. Concentrated urine
  2. Effect of medications (L-carnitine - a fishy odor)


DiMauro S. Exercise intolerance and the mitochondrial respiratory chain. Ital J Neurol Sci 1999:20:387-93.

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