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Aminoaciduria
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Aminoaciduria

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Amino acids - urine; Urine amino acids

Aminoaciduria is an abnormal amount of amino acids in the urine. Amino acids are the building blocks for proteins in the body.

I Would Like to Learn About:

  • How the Test is Performed

    A clean-catch urine sample is needed. This is often done at your doctor's office or health clinic.

  • How to Prepare for the Test

    Most of the time you do not need to take special steps before this test. Make sure your doctor knows all of the medicines you recently used. If this test is being done on an infant who is breast-feeding, make sure the health care provider knows what medicines the nursing mother is taking.

  • How the Test will Feel

    The test involves only normal urination.

  • Why the Test is Performed

    This test is done to measure amino acid levels in the urine. There are many different types of amino acids. It is common for some of each kind to be found in the urine. Increased levels of individual amino acids can be a sign a problem with metabolism.

  • Normal Results

    The specific value is measured in micromoles per deciliter (micromol/dL).

    • 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

    Normal value ranges may vary slightly among different laboratories. Talk to your doctor about the meaning of your specific test results.

    The examples above show the common measurements for results for these tests. Some laboratories use different measurements or may test different specimens.

  • What Abnormal Results Mean

    Increased total urine amino acids may be due to:

    • 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
  • Considerations

    Screening infants for increased levels of amino acids can help detect problems with metabolism. Early treatment for these conditions may prevent complications in the future.

Related Information

  MetabolismEnzymeChromatographyPlasma amino acids...Intellectual disab...GalactosemiaHyperparathyroidis...Methylmalonic acid...Multiple myeloma...Osteomalacia    

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Review Date: 6/4/2013  

Reviewed By: Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.

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