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Aminoaciduria
     
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Aminoaciduria

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.

How the Test is Performed

 

A clean-catch urine sample is needed. This is often done at your health care provider'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 provider knows all of the medicines you recently used. If this test is being done on an infant who is breast-feeding, make sure the 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 mmol/mol creatinine. The values below represent normal ranges in 24 hours urine for adults.

Alanine: 9 to 98

Arginine: 0 to 8

Asparagine: 10 to 65

Aspartic acid: 5 to 50

Citrulline: 1 to 22

Cystine: 2 to 12

Glutamic acid: 0 to 21

Glutamine: 11 to 42

Glycine: 17 to 146

Histidine: 49 to 413

Isoleucine: 30 to 186

Leucine: 1 to 9

Lysine: 2 to 16

Methionine: 2 to 53

Ornithine: 1 to 5

Phenylalanine: 1 to 5

Proline: 3 to 13

Serine: 0 to 9

Taurine: 18 to 89

Threonine: 13 to 587

Tyrosine: 3 to 14

Valine: 3 to 36

Normal value ranges may vary slightly among different laboratories. Talk to your 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:

  • 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 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.

 

 

References

Camargo SMR, Makrides V, Kleta R, Verrey F. Kidney transport of amino acids and oligopeptides, and aminoacidurias. In: Alpern RJ, Moe OW, Caplan M, eds. Seldin and Giebisch’s The Kidney. 5th ed. Waltham, MA: Elsevier Academic Press; 2013:chap 71.

Dietzen DJ. Amino acids, peptides, and proteins. In: Rifai N, ed. Tietz Textbook of Clinical Chemistry and Molecular Diagnostics. 6th ed. St Louis, MO: Elsevier; 2018:chap 28.

Kliegman RM, Stanton BF, St. Geme JW, Schor NF. Defects in metabolism of amino acids. In: Kliegman RM,  Stanton BF, Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 85.

Riley RS, McPherson RA. Basic examination of urine. In: McPherson RA, Pincus MR, eds. Henry's Clinical Diagnosis and Management by Laboratory Methods. 23rd ed. St Louis, MO: Elsevier; 2017:chap 28.

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  • Urine sample

    Urine sample

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  • Aminoaciduria urine test

    Aminoaciduria urine test

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    • Urine sample

      Urine sample

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    • Aminoaciduria urine test

      Aminoaciduria urine test

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    Tests for Aminoaciduria

     
     

    Review Date: 5/1/2017

    Reviewed By: Anna C. Edens Hurst, MD, MS, Assistant Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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