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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.

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Urine sample
Aminoaciduria urine test

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 of a problem with metabolism.

Normal Results

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

Alanine

Alpha-aminoadipic acid

Alpha-amino-N-butyric acid

Arginine

Asparagine

Aspartic acid

Beta-alanine

Beta-amino-isobutyric acid

Carnosine

Citrulline

Cystine

Glutamic acid

Glutamine

Glycine

Histidine

Hydroxyproline

Isoleucine

Leucine

Lysine

Methionine

1-methylhistidine

3-methylhistidine

Ornithine

Phenylalanine

Phosphoserine

Phosphoethanolamine

Proline

Serine

Taurine

Threonine

Tyrosine

Valine

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.

Related Information

Metabolism
Enzyme
Chromatography
Plasma amino acids
Intellectual disability
Galactosemia
Hyperparathyroidism
Methylmalonic acidemia
Multiple myeloma
Osteomalacia
Rickets
Hepatitis
Wilson disease

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.

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Review Date: 4/20/2015  

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

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