Description

A patient with propionic or methylmalonic acidemia may show exercise intolerance that is linked to mitochondrial energy failure.


Patient selection: propionic or methylmalonic acidemia

 

Features:

(1) muscular hypotonia

(2) exercise intolerance with impaired stamina

(3) impairment in daily activities requiring exertion

 

The exercise intolerance tends to be worse during:

(1) acute metabolic decompensation (associated with fever, infection, trauma, stress, etc)

(2) chronic metabolic instability


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