Amino acids - urine

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Amino acids - urine Article

Amino acids - urine

plasma-amino-acids urine-culture-clean-catch serum-phenylalanine-screening

Definition

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

This article discusses the test used to look for increased amounts of amino acids in the urine.

Alternative Names

Amino acids - urine; Urine amino acids

Why the Amino acids - urine 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, but increased levels of individual amino acids can be a sign of an inborn error of metabolism.

How the Amino acids - urine 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 Amino acids - urine

No special preparations are necessary. Make sure your doctor knows about all the medicines you or your child are taking or have 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 Amino acids - urine Will Feel

The test involves only normal urination.

Considerations

Failure to have the urine sample promptly evaluated in the laboratory alters the results of the test.

This test is ineffective if the baby is under 6 weeks old and has not been fed dietary protein in the last 48 hours.

Urine chromatography is necessary to accurately measure increased levels of specific amino acids.

Screening infants for increased levels of amino acids can lead to early diagnosis of an inborn error of metabolism. If the condition is promptly treated, complications such as severe mental retardation may be prevented.

Normal Results

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

Note: All values are in micro mole per deciliter (micro mol/dL)

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's disease

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