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notesduchennes

Duchenne’s muscular dystrophy


Duchenne’s muscular dystrophy:

• DMD is the most common childhood muscular dystrophy (1/3500 male births).
• Patients suffer from progressive muscular degeneration (skeletal, cardiac, smooth) starting at age 3 to 5.
• Patients usually have failure to walk by adolescence and death before the third decade.
• Dilated cardiomyopathy occurs in over 50% by age 15.
• Perioperative adverse events are not common.
• Acute rhabdomyolysis (R) can be triggered by succinylcholine.
• Inhalational agents an also cause R and also metabolic reactions that resemble malignant hyperthermia.
• The lack of evidence to support an association between DMD and MH has led to an alternative diagnosis termed ‘anesthesia-related rhabdomyolysis’ (AIR).
• Although only a small proportion of DMD patients develop AIR when receiving inhalational agents, it is recommended that they be avoided and TIVA be used instead.


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