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Table 2 Amino-acids values at diagnosis

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

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