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Aminoaciduria

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

Alternative Names

Amino acids - urine; Urine amino acids

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:

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.

Updated: 6/4/2013

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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