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Rare metabolic disorder characterized by an idiopathic myoglobinuria. The two clinical entities of this disease are type I, which is associated with onset after exertion, and type II, which occurs after infection.

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Idiopathic Paroxysmal Myoglobinuria.

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

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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.

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

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

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

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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.

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

Savage DCL: Idiopathic rhabdomyolysis. Arch Dis Child 46:594, 1971.  [PubMed: 4107384]

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