Genetics of Glycogen-Storage Disease Type V Follow-up

Updated: Nov 10, 2015
  • Author: Edward J Cupler, MD, FAAN; Chief Editor: Maria Descartes, MD  more...
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Follow-up

Further Inpatient Care

If acute renal failure occurs in patients with McArdle disease (glycogen-storage disease type V), hemodialysis and appropriate monitoring of fluid and electrolyte status may be necessary.

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Deterrence/Prevention

Because acute renal failure is precipitated by rhabdomyolysis in McArdle disease, avoidance of strenuous and/or isometric exercise may be indicated.

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Complications

Acute renal failure may complicate muscle necrosis and myoglobinuria following vigorous exercise.

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Prognosis

McArdle disease typically has a relatively benign nature when severe rhabdomyolysis is avoided. Limitation or adaptation of exercise to avoid symptoms may be necessary. Acute renal failure requires appropriate treatment. Progression to chronic renal disease has not been described, but acute renal failure due to myoglobinuria is potentially life threatening.

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Patient Education

Educate patients about modifying their activity in order to prevent rhabdomyolysis. Patients should avoid extreme isometric exercise.

Educate patients about the “second wind” phenomenon.

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