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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