Carglumic Acid Contributes to a Favorable Clinical Course in a Case of Severe Propionic Acidemia

Table 2

Blood acylcarnitine analysis before and after carglumic acid and carnitine treatment.

	At the onset time (before carglumic treatment)	3 months after carglumic treatment	Cutoff values (μmol/L)
C0	23.96	42.78	<10.00
C2	10.76	24.91
C3	73.85	9.78	≥3.50
C3/C2	6.86	0.39	≥0.25
C4	0.32	0.29	≥0.60
C5	0.21	0.2	≥1.00
C5 : 1	0.04	0.01	≥0.025
C5DC	0.08	0.06	≥0.25
OH-C5	0.19	0.24	≥0.60
C6	0.08	0.08
C8	0.09	0.06	≥0.30
C8/C10	1	0.79	≥1.00
C10	0.09	0.07	≥0.40
C12	0.09	0.11
C14	0.1	0.28
C14 : 1	0.07	0.05	≥0.40
C16	0.97	1.73	≥3.00
OH-C16	0.01	0.02	≥0.05
C0/(C16+C18)	18.75	17.25	≥100.00
C18	0.31	0.76
C18 : 1	0.47	1.02
OH-C18 : 1	0.01	0.01	≥.0.05

C0: free carnitine; C2: acetylcarnitine; C3: propionylcarnitine; C4: butyrylcarnitine; C5: isovalerylcarnitine; C5:1: tiglylcarnitine; C5-DC: glutarylcarnitine; OH-C5: 3-hydroxy isovalerylcarnitine; C6: hexanoylcarnitine; C8: octanoylcarnitine; C10: decanoylcarnitine; C12: lauroylcarnitine; C14: myristoylcarnitine; C14 : 1: myristoylcarnitine; C16: palmitoylcarnitine; OH-C16: 3-hydroxy palmitoylcarnitine; C18: octadecanoylcarnitine C18 : 1: octadecenoylcarnitine; OH-C18 : 1: 3-hydroxy octadecenoylcarnitine.