Neonatal intrahepatic cholestasis caused by citrin deficiency: prevalence and SLC25A13mutations among thai infants

Table 3 Plasma amino acids (PAA) of NICCD and non-NICCD infants with jaundice at enrollment

PAA	NICCD (n=4) median (range)	Non-NICCD (n=18) median (range)	Normal value	P-value^b
Citrulline (umol/L)	60 (36-152)	17 (8-42)	3-35	0.004
Methionine (umol/L)	141 (32-243)	37 (11-702)	9-42	0.23
Phenylalanine (umol/L)	38 (26-40)	45 (11-291)	31-75	0.19
Threonine (umol/L)	181 (139-326)	140 (42-339)	24-174	0.11
Arginine (umol/L)	63 (50-135)	41 (12-92)	12-133	0.16
Tyrosine (umol/L)	121 (33-166)	74 (18-557)	22-108	0.49
Threonine/serine ratio^a	1.63 (1.37-2.10)	1.16 (0.56-2.30)	1.10 (0.88-1.19)	0.03
Fisher ratio (BCAA/AAA)	1.12 (0.67-2.47)	2.23 (0.01-3.42)	3.42±0.33	0.13

BCAA branched chain amino acids (valine + isoleucine + leucine), AAA aromatic amino acids (phenylalanine + tyrosine).
^a median and 25-75% range of threonine/serine ratio in NICCD 2.29 (1.69-2.81) were as described by Kobayashi K and Saheki T (GeneReviews) [8].
^b Only definite NICCD and non-NICCC groups were compared, and statistical significance if P-value <0.05.

ISSN: 1471-230X