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 |