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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:

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.

    • Urine sample

      Urine sample - illustration

      A clean-catch urine sample is performed by collecting the sample of urine in midstream. Men or boys should wipe clean the head of the penis. Women or girls need to wash the area between the lips of the vagina with soapy water and rinse well. A small amount of urine should initially fall into the toilet bowl before it is collected (this clears the urethra of contaminants). Then, in a clean container, catch about 1 to 2 ounces of urine and remove the container from the urine stream. The container is then given to the health care provider.

      Urine sample

      illustration

    • Aminoaciduria urine test

      Aminoaciduria urine test - illustration

      Aminoaciduria is a test that screens for increased levels of amino acid excretion in the urine which may indicate inborn errors of metabolism caused by a specific enzyme deficiency.

      Aminoaciduria urine test

      illustration

      • Urine sample

        Urine sample - illustration

        A clean-catch urine sample is performed by collecting the sample of urine in midstream. Men or boys should wipe clean the head of the penis. Women or girls need to wash the area between the lips of the vagina with soapy water and rinse well. A small amount of urine should initially fall into the toilet bowl before it is collected (this clears the urethra of contaminants). Then, in a clean container, catch about 1 to 2 ounces of urine and remove the container from the urine stream. The container is then given to the health care provider.

        Urine sample

        illustration

      • Aminoaciduria urine test

        Aminoaciduria urine test - illustration

        Aminoaciduria is a test that screens for increased levels of amino acid excretion in the urine which may indicate inborn errors of metabolism caused by a specific enzyme deficiency.

        Aminoaciduria urine test

        illustration

      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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