case_images: PMC5121296_01
case_images: PMC5121296_02
case_images: PMC5121296_03
case_images: PMC5121296_04
case_images: PMC5121296_05
case_images: Urine organic acids profiles (Key: 3-OH PA: 3-hydroxypropionic acid, IC: internal standard). Of note our biochemistry laboratory did not quantitate 3-hydroxypropionate at the time the initial spectrum was obtained. A. Marked excretion of 3-hydroxypropionate with normal excretion of methylcitrate while patient is on ketogenic diet. B. Undetectable 3-hydroxypropionic acid after initiation of therapy for PA.
case_images: gr1.jpg
case_images: PMC5121296_06_gr1.jpg
case_images: f0005
case_images: The patient was treated with natural protein restriction, carnitine, biotin, and thiamine with resolution of the metabolic acidosis and of the propionate metabolites in the urine organic acids profile (Fig. 1B).
case_images: PMC5121296_06
case_images: Urine organic acids profiles (Key: 3-OH PA: 3-hydroxypropionic acid, IC: internal standard). Of note our biochemistry laboratory did not quantitate 3-hydroxypropionate at the time the initial spectrum was obtained. A. Marked excretion of 3-hydroxypropionate with normal excretion of methylcitrate while patient is on ketogenic diet. B. Undetectable 3-hydroxypropionic acid after initiation of therapy for PA.
case_images: gr1.jpg
case_images: PMC5121296_06_gr1.jpg
case_images: f0005
case_images: The patient was treated with natural protein restriction, carnitine, biotin, and thiamine with resolution of the metabolic acidosis and of the propionate metabolites in the urine organic acids profile (Fig. 1B).
case_images: PMC5121296_07
case_images: PMC5121296_08
case_images: PMC5121296_09