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

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

PAA NICCD (n=4) median (range) Non-NICCD (n=18) median (range) Normal value P-valueb
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 ratioa 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
  1. BCAA branched chain amino acids (valine + isoleucine + leucine), AAA aromatic amino acids (phenylalanine + tyrosine).
  2. 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].
  3. b Only definite NICCD and non-NICCC groups were compared, and statistical significance if P-value <0.05.