PMC5121296_01
PMC5121296_02
PMC5121296_03
PMC5121296_04
PMC5121296_05
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.
gr1.jpg
PMC5121296_06_gr1.jpg
f0005
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).
PMC5121296_06
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.
gr1.jpg
PMC5121296_06_gr1.jpg
f0005
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).
PMC5121296_07
PMC5121296_08
PMC5121296_09