Update on Lysinuric Protein Intolerance, a Multi-faceted Disease Retrospective cohort analysis from birth to adulthood

Table 2 Amino-acids values at diagnosis

	Age (years)	Patient values at diagnosis						Normal range adapted with age
		Plasma (μmol/l)			Urine (μmol/mmol creatinine)			Plasma (μmol/l)			Urine (μmol/mmol creatinine)
		Lys	Arg	Orn	Lys	Arg	Orn	Lys	Arg	Orn	Lys	Arg	Orn
1 *	1.5	116	82	47	3664	305	230	89-241	34-106	24-92	16-69	<8	<8
2 *	0.8	78	82	47	NA	NA	NA	96-262	41-117	22-114	13-79	<11	<8
3 **	16.2	34	14	9	32	7	2	120-268	40-124	25-125	7-58	<5	<5
5 ***	2.2	52	13	15	822	36	16	85-241	38-110	23-91	10-46	<9	<7
6	1.3	35	9	5	1372	107	31	89-241	34-106	24-92	16-69	<8	<8
8 ****	0.7	113	20	16	2990	195	67	96-262	41-117	22-114	13-79	<11	<8
9	2.2	74	17	8	1849	188	125	85-241	38-110	23-91	10-46	<9	<7
10	1.3	121	28	16	1721	231	135	89-241	34-106	24-92	16-69	<8	<8
11	12.6	33	7	10	33	7	10	141-224	35-115	20-132	10-56	<6	<6
12	3.4	77	27	11	886	155	38	85-241	38-110	23-91	10-46	<9	<7
13 ****	10.3	38	16	21	747	398	95	141-224	35-115	20-132	10-56	<6	<6
14 *	0.1	206	23	11	1504	121	58	106-246	35-91	22-154	22-171	<14	<19
15 *	0.005	177	33	69	673	129	75	106-246	35-91	22-154	22-171	<14	<19
16 ***	0.008	137	19	13	927	68	32	106-246	35-91	22-154	22-171	<14	<19

*,**,*** and **** refer to 4 different families. Cases without asterisk are from different families

ISSN: 1750-1172