BACK TOTOP Browse A-ZSearchBrowse A-ZABCDEFGHIJKLMNOPQRSTUVWXYZ0-9 E-mail FormEmail ResultsName:Email address:Recipients Name:Recipients address:Message: Print-FriendlyBookmarksbookmarks-menuAminoaciduriaAmino acids - urine; Urine amino acidsAminoaciduria is an abnormally high amount of amino acids in the urine. Amino acids are the building blocks for proteins in the body. 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.Clean-catch urine sampleA clean catch is a method of collecting a urine sample to be tested. The clean-catch urine method is used to prevent germs from the penis or vagina ...ImageRead Article Now Book Mark Article 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 1337Arginine: 5 to 70Asparagine: 25 to 454Aspartic acid: 1 to 87Aspartic acidAspartic acid is a nonessential amino acid. Amino acids are building blocks of proteins. "Nonessential" means that our bodies produce it, even if we...Read Article Now Book Mark Article Citrulline: 1 to 27Cystine: 0 to 224Glutamic acid: 5 to 92Glutamine: 5 to 1756Glycine: 277 to 7997Histidine: 106 to 2534Isoleucine: 5 to 48Leucine: 5 to 129Lysine: 15 to 1021Methionine: 1 to 37Ornithine: 5 to 76Phenylalanine: 5 to 239Proline: 5 to 169Serine: 98 to 1053Taurine: 24 to 5336Threonine: 5 to 715Tyrosine: 5 to 389Valine: 5 to 147The 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 AlkaptonuriaAlkaptonuria is a rare condition in which a person's urine turns a dark brownish-black color when exposed to air. Alkaptonuria is part of a group of...Read Article Now Book Mark Article Canavan disease Canavan diseaseCanavan disease is a condition that affects how the body breaks down and uses aspartic acid.Read Article Now Book Mark Article Cystinosis Cystathioninuria Fructose intolerance Hereditary fructose intolerance is a disorder in which a person lacks the protein needed to break down fructose. Fructose is a fruit sugar that natu...Read Article Now Book Mark Article Fructose intoleranceHereditary fructose intolerance is a disorder in which a person lacks the protein needed to break down fructose. Fructose is a fruit sugar that natu...Read Article Now Book Mark Article Galactosemia GalactosemiaGalactosemia is a condition in which the body is unable to use (metabolize) the simple sugar galactose.ImageRead Article Now Book Mark Article Hartnup disease Hartnup diseaseHartnup disorder is a genetic condition in which there is a defect in the transport of certain amino acids (such as tryptophan and histidine) by the ...Read Article Now Book Mark Article Homocystinuria HomocystinuriaHomocystinuria is a genetic disorder that affects the metabolism of the amino acid methionine. Amino acids are the building blocks of all proteins i...ImageRead Article Now Book Mark Article Hyperammonemia Hyperparathyroidism HyperparathyroidismHyperparathyroidism is a condition in which 1 or more of the parathyroid glands in your neck produce too much parathyroid hormone (PTH).ImageRead Article Now Book Mark Article Maple syrup urine disease Maple syrup urine diseaseMaple syrup urine disease (MSUD) is a disorder in which the body cannot break down certain parts of proteins. The urine of people with this conditio...Read Article Now Book Mark Article Methylmalonic acidemia Methylmalonic acidemiaMethylmalonic acidemia is a disorder in which the body cannot break down certain proteins and fats. The result is a buildup of a substance called me...Read Article Now Book Mark Article Multiple myeloma Multiple myelomaMultiple myeloma is a blood cancer that starts from a type of white blood cell in the bone marrow called plasma cells. Bone marrow is the soft, spon...ImageRead Article Now Book Mark Article Ornithine transcarbamylase deficiency Osteomalacia OsteomalaciaOsteomalacia is softening of the bones. It most often occurs because of a problem that leads to vitamin D deficiency, which helps your body absorb c...ImageRead Article Now Book Mark Article Propionic acidemia Rickets RicketsRickets is a disorder that occurs in children before bone growth is complete. It is caused by a lack of vitamin D, calcium, or phosphate. It leads ...ImageRead Article Now Book Mark Article Tyrosinemia type 1 Tyrosinemia type 2 Viral hepatitis HepatitisHepatitis is swelling and inflammation of the liver.ImageRead Article Now Book Mark Article Wilson diseaseWilson diseaseWilson disease is an inherited disorder in which there is too much copper in the body's tissues. The excess copper damages the liver and nervous sys...ImageRead Article Now Book Mark Article 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.Open ReferencesReferencesDietzen 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.AllVideoImagesTogUrine sample - illustration 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.Urine sampleillustrationAminoaciduria urine test - illustration 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.Aminoaciduria urine testillustrationUrine sample - illustration 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.Urine sampleillustrationAminoaciduria urine test - illustration 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.Aminoaciduria urine testillustration Tests for Aminoaciduria AminoaciduriaRelated Information Metabolism(Special Topic)Enzyme(Special Topic)Chromatography(Special Topic)Plasma amino acids(Medical Test)Intellectual disability(Condition)Galactosemia(Condition)Hyperparathyroidism(Condition)Methylmalonic acidemia(Condition)Multiple myeloma(Condition)Osteomalacia(Condition)Hepatitis - InDepth(In-Depth) Review Date: 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. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. © 1997- All rights reserved. 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AminoaciduriaAmino acids - urine; Urine amino acidsAminoaciduria is an abnormally high amount of amino acids in the urine. Amino acids are the building blocks for proteins in the body. 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.Clean-catch urine sampleA clean catch is a method of collecting a urine sample to be tested. The clean-catch urine method is used to prevent germs from the penis or vagina ...ImageRead Article Now Book Mark Article 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 1337Arginine: 5 to 70Asparagine: 25 to 454Aspartic acid: 1 to 87Aspartic acidAspartic acid is a nonessential amino acid. Amino acids are building blocks of proteins. "Nonessential" means that our bodies produce it, even if we...Read Article Now Book Mark Article Citrulline: 1 to 27Cystine: 0 to 224Glutamic acid: 5 to 92Glutamine: 5 to 1756Glycine: 277 to 7997Histidine: 106 to 2534Isoleucine: 5 to 48Leucine: 5 to 129Lysine: 15 to 1021Methionine: 1 to 37Ornithine: 5 to 76Phenylalanine: 5 to 239Proline: 5 to 169Serine: 98 to 1053Taurine: 24 to 5336Threonine: 5 to 715Tyrosine: 5 to 389Valine: 5 to 147The 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 AlkaptonuriaAlkaptonuria is a rare condition in which a person's urine turns a dark brownish-black color when exposed to air. Alkaptonuria is part of a group of...Read Article Now Book Mark Article Canavan disease Canavan diseaseCanavan disease is a condition that affects how the body breaks down and uses aspartic acid.Read Article Now Book Mark Article Cystinosis Cystathioninuria Fructose intolerance Hereditary fructose intolerance is a disorder in which a person lacks the protein needed to break down fructose. Fructose is a fruit sugar that natu...Read Article Now Book Mark Article Fructose intoleranceHereditary fructose intolerance is a disorder in which a person lacks the protein needed to break down fructose. Fructose is a fruit sugar that natu...Read Article Now Book Mark Article Galactosemia GalactosemiaGalactosemia is a condition in which the body is unable to use (metabolize) the simple sugar galactose.ImageRead Article Now Book Mark Article Hartnup disease Hartnup diseaseHartnup disorder is a genetic condition in which there is a defect in the transport of certain amino acids (such as tryptophan and histidine) by the ...Read Article Now Book Mark Article Homocystinuria HomocystinuriaHomocystinuria is a genetic disorder that affects the metabolism of the amino acid methionine. Amino acids are the building blocks of all proteins i...ImageRead Article Now Book Mark Article Hyperammonemia Hyperparathyroidism HyperparathyroidismHyperparathyroidism is a condition in which 1 or more of the parathyroid glands in your neck produce too much parathyroid hormone (PTH).ImageRead Article Now Book Mark Article Maple syrup urine disease Maple syrup urine diseaseMaple syrup urine disease (MSUD) is a disorder in which the body cannot break down certain parts of proteins. The urine of people with this conditio...Read Article Now Book Mark Article Methylmalonic acidemia Methylmalonic acidemiaMethylmalonic acidemia is a disorder in which the body cannot break down certain proteins and fats. The result is a buildup of a substance called me...Read Article Now Book Mark Article Multiple myeloma Multiple myelomaMultiple myeloma is a blood cancer that starts from a type of white blood cell in the bone marrow called plasma cells. Bone marrow is the soft, spon...ImageRead Article Now Book Mark Article Ornithine transcarbamylase deficiency Osteomalacia OsteomalaciaOsteomalacia is softening of the bones. It most often occurs because of a problem that leads to vitamin D deficiency, which helps your body absorb c...ImageRead Article Now Book Mark Article Propionic acidemia Rickets RicketsRickets is a disorder that occurs in children before bone growth is complete. It is caused by a lack of vitamin D, calcium, or phosphate. It leads ...ImageRead Article Now Book Mark Article Tyrosinemia type 1 Tyrosinemia type 2 Viral hepatitis HepatitisHepatitis is swelling and inflammation of the liver.ImageRead Article Now Book Mark Article Wilson diseaseWilson diseaseWilson disease is an inherited disorder in which there is too much copper in the body's tissues. The excess copper damages the liver and nervous sys...ImageRead Article Now Book Mark Article 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.Open ReferencesReferencesDietzen 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.
