Aminoaciduria

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Aminoaciduria urine test

Urine sample

Amino acids - urine; Urine amino acids

• Alanine
  • Children: 65 to 190
  • Adults: 160 to 690
• Alpha-aminoadipic acid
  • Children: 25 to 78
  • Adults: 0 to 165
• Alpha-amino-N-butyric acid
  • Children: 7 to 25
  • Adults: 0 to 28
• Arginine
  • Children: 10 to 25
  • Adults: 13 to 64
• Asparagine
  • Children: 15 to 40
  • Adults: 34 to 100
• Aspartic acid
  • Children: 10 to 26
  • Adults: 14 to 89
• Beta-alanine
  • Children: 0 to 42
  • Adults: 0 to 93
• Beta-amino-isobutyric acid
  • Children: 25 to 96
  • Adults: 10 to 235
• Carnosine
  • Children: 34 to 220
  • Adults: 16 to 125
• Citrulline
  • Children: 0 to 13
  • Adults: 0 to 11
• Cystine
  • Children: 11 to 53
  • Adults: 28 to 115
• Glutamic acid
  • Children: 13 to 22
  • Adults: 27 to 105
• Glutamine
  • Children: 150 to 400
  • Adults: 300 to 1,040
• Glycine
  • Children: 195 to 855
  • Adults: 750 to 2,400
• Histidine
  • Children: 46 to 725
  • Adults: 500 to 1,500
• Hydroxyproline
  • Children: not measured
  • Adults: not measured
• Isoleucine
  • Children: 3 to 15
  • Adults: 4 to 23
• Leucine
  • Children: 9 to 23
  • Adults: 20 to 77
• Lysine
  • Children: 19 to 140
  • Adults: 32 to 290
• Methionine
  • Children: 7 to 20
  • Adults: 5 to 30
• 1-methylhistidine
  • Children: 41 to 300
  • Adults: 68 to 855
• 3-methylhistidine
  • Children: 42 to 135
  • Adults: 64 to 320
• Ornithine
  • Children: 3 to 16
  • Adults: 5 to 70
• Phenylalanine
  • Children: 20 to 61
  • Adults: 36 to 90
• Phosphoserine
  • Children: 16 to 34
  • Adults: 28 to 95
• Phosphoethanolamine
  • Children: 24 to 66
  • Adults: 17 to 95
• Proline
  • Children: not measured
  • Adults: not measured
• Serine
  • Children: 93 to 210
  • Adults: 200 to 695
• Taurine
  • Children: 62 to 970
  • Adults: 267 to 1,290
• Threonine
  • Children: 25 to 100
  • Adults: 80 to 320
• Tyrosine
  • Children: 30 to 83
  • Adults: 38 to 145
• Valine
  • Children: 17 to 37
  • Adults: 19 to 74

Comparison is necessary with the plasma amino acids to develop a complete picture of the aminoacidurias.

Note: all measurements in micro mole per deciliter (micro mol/dl)

Increased total urine amino acids may indicate any of the following:

• Alkaptonuria
• Canavan disease
• Cystinosis
• Cystathioninuria
• Fructose intolerance
• Galactosemia
• Hartnup disease
• Homocystinuria
• Hyperammonemia
• Hyperparathyroidism
• Maple syrup urine disease
• Methylmalonic acidemia
• Multiple myeloma
• Ornithine transcarbamylase deficiency
• Osteomalacia
• Propionic acidemia
• Rickets
• Tyrosinemia type 1
• Tyrosinemia type 2
• Viral hepatitis
• Wilson's disease

Review Date: 04/29/2005
Reviewed By: Frank A. Greco, M.D., Ph.D., Director, Biophysical Laborartory, The Lahey Clinic, Burlington, MA. Review provided by VeriMed Healthcare Network.