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At a glance

It is a rare metabolic disorder characterized by an idiopathic primary myoglobinuria. The two clinical entities of this disease are type I, associated with an onset after physical exertion, and type II, that occurs after infection. Clinically, the symptoms include a classical triad consisting of muscle pain, generalized weakness, and discolored brown urine as a result of the excretion of myoglobin.

Synonyms

Idiopathic Paroxysmal Myoglobinuria; Primary Myoglobinuria; Idiopathic paroxysmal rhabdomyolysis.

History

This medical condition was first described in the German literature in the 19th century (c.1895). The full description of the disease was first presented in 1910 by Hans Meyer and Vladimir Betz, German physicians.

Incidence

The exact incidence remains unknown in the general population. However, screening of 337 military recruits for myoglobin serum levels showed rhabdomyolysis in 40%. The presence of myoglobinuria is more common in men, particularly well-educated professionals, who arrange work schedules to allow for daily exercising. This has led to rename this condition the “white collar rhabdomyolysis.”

Pathophysiology

Focal necrosis of skeletal muscle leads to rhabdomyolysis and myoglobinuria. Death may occur secondary to immediate hyperkalemia or subsequent renal tubular necrosis.

Diagnosis

No specific test for this disorder. Diagnosis is based on clinical and laboratory findings, specifically hyperkalemia, myoglobinuria, renal failure, and muscle weakness associated with either exertion or febrile illness.

Clinical aspects

Hyperkalemia, myoglobinuria, renal failure, muscle weakness that may lead to respiratory failure, association with exertion or febrile illness.

N.B.: This disease mimics disorders of lipid metabolism, in which similar signs and symptoms occur with hypoglycemia.

Precautions before anesthesia

Obtain baseline serum electrolytes, glucose, blood urea nitrogen, and creatinine. Check temperature. Avoid prolonged fasting; start dextrose infusion while administering nothing by mouth.

Anesthetic considerations

Anticipate need to treat intraoperative hyperkalemia and renal failure. Frequently check glucose and supplement if needed. Prevent hyperthermia and cool aggressively if it occurs. Although few data on the use of succinylcholine in Meyer-Betz disease are available, prudence suggests substitution with nondepolarizing neuromuscular blocking agents in reduced doses in the presence of muscle weakness.

Pharmacological implications

Avoid succinylcholine. Titrate nondepolarizing neuromuscular blocking agents with a nerve stimulator.

References

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Denson  HB: Primary myoglobinuria (Meyer-Betz disease). Am J Med 29:534–538, 1960.  [PubMed: 13815966]
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Kasap  B, Soylu  A, Türkmen  M, Kavukcu  S: An adolescent girl with Meyer-Betz syndrome. Clin Rheumatol 25(6):904–906, 2006.  [PubMed: 16328090]
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Meyer-Betz  F: Beobachtugen an einem eigenartigen mit muskellahmungen verbundenen fall von hamoglobinurie. Dtsc Arch Klin Med 101:85–127, 1910 (Original descriptive article).
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Savage  DCL: Idiopathic rhabdomyolysis. Arch Dis Child 46:594, 1971. ...

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