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Graf, C., Elmassry, M., Chu, V

Fatal hyperammonaemia due to late-onset ornithine transcarbamylase deficiency

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Primary outcomes included exercise capacity (e.g., maximal oxygen uptake (VO 2 max), maximal work rate (W max), peak oxygen uptake (Peak VO 2 ), six-minute walk distance (6MWD)), muscle function [e.g., one-repetition maximum (1RM), muscle cross-sectional area], mitochondrial function [e.g., citrate synthase (CS) activity, ETC complex activity], and metabolic or inflammatory markers

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