ACUTE MYOGLOBINURIC RENAL-FAILURE IN MCARDLES DISEASE - DIAGNOSTIC-SIGNIFICANCE OF P-31-MAGNETIC RESONANCE SPECTROSCOPY

A 44-year-old man developed acute renal failure, associated with reddish brown urine, after severe physical exertion. Since childhood his physical capacity was known to be limited. Muscular indurations, cramps and pains had occurred after muscular work. Serum creatinine level on the day before hospitalization was 10.0 mg/dl, creatinekinase activity 3,790 U/l, lactate dehydrogenase activity 670 U/l, myoglobin level 701 ng/ml. Cause of the rhabdomyolysis was thought to be McArdle's disease (glycogen storage disease type V: abnormal skeletal muscle glycogen utilization). P-31-magnetic resonance spectroscopy, which gives characteristic spectra with anaerobic muscle work, provided the diagnosis, which was confirmed by measurement of myophosphorylase activity in a muscle biopsy (zero activity). After two dialysis sessions and alkalinization of the urine diuresis again occurred and the retention indicators fell to normal in the course of the subsequent 18 months. Creatine kinase activity remained elevated.