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Mitochondrial patients often have elevations of CPK, a muscle enzyme. Chronically elevated CPK levels are reflective of muscle disease and muscle breakdown. Although mildly elevated levels are of no concern, levels greater than 200 can be reflective of chronic muscle breakdown and ultimately muscle mass loss. In addition, some patients have intermittent episodes of acute, severe muscle breakdown known as rhabdomyolysis with CPK levels climbing into the thousands, which results in muscle protein, myoglobin, being dumped into a patient's urine turning it coca cola colored and clogging their kidneys with muscle debris. Rhabdomyolysis is a medical emergency that can lead to acute kidney failure requiring treatment with intravenous fluids and bicarbonate. All mitochondrial patients should have CPK levels monitored.
Fran D. Kendall, M.D.
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