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These findings corroborate evidence of decreased Complex II protein levels and OXPHOS activity, which is a marker for mitochondrial FAD in muscle of patients with severe neuromuscular phenotype and novel variants in SLC25A32 [30,31]

Patients with copper sensitivity may experience more pronounced injection site reactions, and this is the primary clinical indicator that leads to protocol adjustment or discontinuation

Anyway, a state of relative insuficiency may persist for several months after the discontinuation of the therapy, so any stress that occurs in this period requires reinstituting corticosteorid therapy or dose increase