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Two individuals with PWS were found to have an abnormal ester-to-free carnitine ratio in addition to a low free carnitine level, plus elevations on acylcarnitine profiles, suggesting that there may be a small subset of the PWS population (approx 5%) who have a true secondary carnitine deficiency
These include action potential production in excitatory tissues used for cell adhesion and interactions, neurotransmitter utilisation in cell signalling such as dopamine, choline and GABA and the calcium uptake and retention regulation by synaptosomes
As peroxides build up, cell membrane integrity and flexibility is reducedhemolysis
Dose adjustment, protocol modification, or reevaluation of injury severity and type may be appropriate
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