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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.

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 of 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 health care provider 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.

Alternative Names

Amino acids - urine; Urine amino acids

References

Hortin GL. In: Burtis CA ed. Amino Acids, Peptides, and Proteins. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 5th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 21.

Review Date 4/20/2015

Updated 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, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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