Aminoaciduria
Amino acids - urine; Urine amino acids
Aminoaciduria is an abnormally high amount of amino acids in the urine. Amino acids are the building blocks for proteins in the body.
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.
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.
How the Test is Performed
A clean-catch urine sample may be 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 breastfeeding, 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 µmol/g creatinine. The values below represent normal ranges in 24 hours urine for adults. In children, normal values vary with age. In addition, normal values may vary between different laboratories. Talk to your provider about your specific test results.
Alanine: 78 to 1337
Arginine: 5 to 70
Asparagine: 25 to 454
Aspartic acid: 1 to 87
Citrulline: 1 to 27
Cystine: 0 to 224
Glutamic acid: 5 to 92
Glutamine: 5 to 1756
Glycine: 277 to 7997
Histidine: 106 to 2534
Isoleucine: 5 to 48
Leucine: 5 to 129
Lysine: 15 to 1021
Methionine: 1 to 37
Ornithine: 5 to 76
Phenylalanine: 5 to 239
Proline: 5 to 169
Serine: 98 to 1053
Taurine: 24 to 5336
Threonine: 5 to 715
Tyrosine: 5 to 389
Valine: 5 to 147
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
Dietzen DJ, Willrich MAV. Amino acids, peptides, and proteins. In: Rifai N, Chiu RWK, Young I, Burnham Carey-Ann D, Wittwer CT, eds. Tietz Textbook of Laboratory Medicine. 7th ed. St Louis, MO: Elsevier; 2023:chap 31.
Kliegman RM, St. Geme JW, Blum NJ, et al. Defects in metabolism of amino acids. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics. 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 105.
Merritt JL, Gallagher RC. Inborn errors of carbohydrate, ammonia, amino acid, and organic acid metabolism. In: Gleason CA, Sawyer T, eds. Avery's Diseases of the Newborn. 11th ed. Philadelphia, PA: Elsevier; 2024:chap 29.
Riley RS, McPherson RA. Basic examination of urine. In: McPherson RA, Pincus MR, eds. Henry's Clinical Diagnosis and Management by Laboratory Methods. 24th ed. Philadelphia, PA: Elsevier; 2022:chap 29.
Version Info
Last reviewed on: 4/8/2025
Reviewed by: Anna C. Edens Hurst, MD, MS, Associate Professor in Medical Genetics, The University of Alabama at Birmingham, Birmingham, AL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
