Primary Myoglobinuria

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Abstract
Primary paroxysmal myoglobinuria precipitated by exercise or asthmatic attacks is reported in a man aged 37 years. This represents the 57th case of this disorder recorded in the medical literature. The presence of muscle pain and dark urine in this patient led us to suspect the diagnosis of primary myoglobinuria. The urine at this time contained no red blood cells or casts, but contained a benzidine positive pigmented protein while the plasma was free of hemoglobin, a situation strongly suggestive of myoglobinuria. Myoglobinuria was demonstrated conclusively during a typical attack by urine electrophoresis using the benzidine spray technique. Its presence was confirmed by ultracentrifugation and ultrafiltration of the patient''s urine. Spectroscopic examination of the urine was unsatisfactory and the ammonium sulfate test of Blondheim was negative for myoglobin on two occasions, representing the first recorded failure of the Blondheim test to confirm the presence of myoglobin in a known instance of myoglobinuria. The onset of primary myoglobinuria is usually in the second or third decade of life. Seventy-five % of the reported cases have been in white men although 2 instances have been noted in negro women. The disease has been reported in families. The attacks begin with pain and weakness in the extremities followed by the passage of dark urine. Muscle wasting and myotonia have been recorded as manifestations of the disorder. Death due to acute renal failure has been noted in 22% of the reported cases.