Table 1 Weighted arithmetic mean values of plasma BCAAs and L-arginine in the study sample.

From: Long-term effects of medical management on growth and weight in individuals with urea cycle disorders

 

L-valine

L-leucine

L-isoleucine

L-arginine

Mean, SD; n

Mean, SD; n

Mean, SD; n

Mean, SD; n

Reference range

133–273

64–164

31–83

38–98

Conservative management

Protein restricted diet

145, 54; 23

68, 25; 23

38, 16; 23

73, 33, 23

No protein restricted diet

166, 72; 22

92, 45; 22

45, 23; 22

74, 32, 22

p value

0.25

0.06

0.24

0.78

Liver transplantation

Prior to LTx

144, 58; 18

86, 63; 18

40, 23; 18

n/a

After LTx

224, 70; 15

123, 32; 15

67, 21; 16

n/a

p value

0.001

0.048

0.001

n/a

  1. Reference ranges were defined according to (20) for children in the age range of 2–10 years and are shown in µmol/l. P values were calculated using a two-sided t-test, p values < 0.05 were considered significant, n refers to number of patients included in each group. Of note, 65% (n = 15/23) of individuals with a protein restricted diet and 59% (n = 13/22) of individuals without a protein restricted diet received (at least temporarily) supplementation with L-arginine within the observation period. BCAAs, branched-chain amino acid(s); LTx, liver transplantation; n/a, not available.
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