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Particularly, we found that mean plasma levels of Valine (U:140.9 µmol/L, K:127.8 µmol/L), Lysine (U:109.9 µmol/L, K:95.7 µmol/L), Threonine (U:67.5 µmol/L, K:57.9 µmol/L) and Tryptophan (U:2.9 µmol/L, K:2.3 µmol/L), fell below the expected ranges for healthy adolescents (UCSF Clinical Core Accepted Reference Ranges) for both those affected with konzo and their unaffected siblings