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Table 2 Amino acid levels in both infants

From: Neonatal hyperinsulinism in transient and classical forms of tyrosinemia

Amino acid levels μM (normal values) Patient I Patient II
Day 19 of life Day 22 of life Approximately 2 months of life Day 7–8 of life Day 12 of life 3 days after starting Nitisinone 17 months of life
Leucine 47–155 164.1 166.7 78.3 126.1 157.7 103.1
Alanine 131–710 534.8 526.6 352.7 841.3 628.6 302.3
Isoleucine 31–86 92.9 102 51.0 50.9 81.1 43.9
Valine 86–190 258.3 234 147.2 265.7 290.5 267.6
Lysine 52–196 200.9 242.7 127.4 395.9 345.9 69
Phenylalanine 31–75 96.5 78.3 41.3 53.6 12 72.6
Tyrosine 22–108 904.3 660.5 211.8
79 (6 months of life)
506 26.8 426.4
  1. Values with upward arrow and in italic indicate high levels of amino acids