. 2020 Aug 28;25:100641. doi: 10.1016/j.ymgmr.2020.100641

Table 1.

Clinical and laboratory findings for the case.

	0 days	1 month	6 months	1 year	2 years	3 years	4 years
	0 days	NAC-MTZ	NAC-MTZ	NAC-MTZ CoQ10-B2	NAC-MTZ CoQ10-B2	NAC-MTZ CoQ10-B2	NAC-MTZ CoQ10-B2 operation
Clinic
Spastic paraparesis	++	++	++	++	+++	+++	+
Diarrhoea (times/day)	15-20	15	10	4	4	3	2

Laboratory
ethylmalonic acid (U)†	46	36	10	23	31	28	19
n.v. <8.4^⁎	46	36	10	23	31	28	19
isovaleryl glycine (U)	19	16	16	3	1.5	2.4	–
n.v. 0^⁎	19	16	16	3	1.5	2.4	–
isobutyryl glycine (U)	10	8.4	6.6	4.1	2	3.5	0.12
n.v.<1.6^⁎	10	8.4	6.6	4.1	2	3.5	0.12
methylsuccinic acid (U)	1.6	0.4	–	0.8	1	0.16	–
n.v. <4.4^⁎	1.6	0.4	–	0.8	1	0.16	–
2-methylbutyrylglycine (U)	0.8	–	–	–	0.4	0.21	–
n.v. 0^⁎	0.8	–	–	–	0.4	0.21	–
C4-Carnitine (P)⁋	1.26	1.08	1.15	1.2	1.2	1.25	1
n.v. 0-1.3^⁎⁎	1.26	1.08	1.15	1.2	1.2	1.25	1
C5-Carnitine (P)	0.39	0.32	0.29	0.27	0.3	0.31	0.25
n.v. 0.31- 0.01^⁎⁎	0.39	0.32	0.29	0.27	0.3	0.31	0.25
Lactate (P)	14	15	12	13	15	17	11
n.v. 4.5-19.8^⁎⁎⁎	14	15	12	13	15	17	11

^⁎mmol/mol creatinine; ^⁎⁎μmol/L; ^⁎⁎⁎mg/dl; ⁋ plasma; † urine