Aminoaciduria

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

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…

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…

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.

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, Shah SS, Tasker RC, Wilson KM. Defects in metabolism of amino…