Learn About Aminoaciduria


A clean-catch urine sample may be needed. This is often done at your health care provider's office or health clinic.

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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.


The test involves only normal urination.


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.


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.


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
What is the definition of Aminoaciduria?

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

What are the alternative names for Aminoaciduria?

Amino acids - urine; Urine amino acids

What is some background information about Aminoaciduria?

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.

Urine sample
Aminoaciduria urine test
Who are the top Aminoaciduria Local Doctors?
Highly rated in

University Of Leuven

Leuven, VLG, BE 3000

Elena Levtchenko is in Leuven, Belgium. Levtchenko is rated as an Elite expert by MediFind in the treatment of Aminoaciduria. She is also highly rated in 31 other conditions, according to our data. Her top areas of expertise are Cystinosis, Fanconi Syndrome, Fanconi Bickel Syndrome, and Aminoaciduria.

Highly rated in

Hôpital Des Enfants

Toulouse, FR 

Maithe Tauber is in Toulouse, France. Tauber is rated as an Elite expert by MediFind in the treatment of Aminoaciduria. She is also highly rated in 16 other conditions, according to our data. Her top areas of expertise are Prader-Willi Syndrome, Aminoaciduria, Obesity, and Hypotonia.

Learn about our expert tiers
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Highly rated in
Medical Genetics

The University of Kansas Health System

The University Of Kansas Hospital

4000 Cambridge St 
Kansas City, KS 66160

Merlin Butler is a Pediatrics specialist and a Medical Genetics doctor in Kansas City, Kansas. Dr. Butler has been practicing medicine for over 44 years and is rated as an Elite doctor by MediFind in the treatment of Aminoaciduria. He is also highly rated in 11 other conditions, according to our data. His top areas of expertise are Angelman Syndrome, Prader-Willi Syndrome, Aminoaciduria, and 15q11.2 Microdeletion. He is licensed to treat patients in Missouri. Dr. Butler is currently accepting new patients.

What are the latest Aminoaciduria Clinical Trials?
A Phase II Clinical Trial to Evaluate the Efficacy of the Infusion of Autologous CD34+ Cells Transduced With a Lentiviral Vector Carrying the FANCA Gene (Orphan Drug) in Patients With Fanconi Anemia Subtype A
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Dysregulation of Hematopoiesis in Fanconi Anemia
What are the Latest Advances for Aminoaciduria?
Cystinosis in Pediatric Renal Transplant Recipients: A Case-Control Study From Kuwait.
The Efficacy, Safety, and Pharmacology of a Ghrelin O-Acyltransferase Inhibitor for the Treatment of Prader-Willi Syndrome.
Tired of the same old research?
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Acquired growth hormone deficiency in Fanconi-Bickel syndrome.
Who are the sources who wrote this article ?

Published Date: May 02, 2021
Published 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 Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

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, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM. Defects in metabolism of amino acids. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 103.

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. Elsevier; 2022:chap 29.