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 urine culture is a lab test to check for bacteria or other germs in a urine sample. It can be used to check for a urinary tract infection in adults...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 mmol/mol creatinine. The values below represent normal ranges in 24 hours urine for adults.Alanine: 9 to 98Arginine: 0 to 8Asparagine: 10 to 65Aspartic acid: 5 to 50Aspartic 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 22Cystine: 2 to 12Glutamic acid: 0 to 21Glutamine: 11 to 42Glycine: 17 to 146Histidine: 49 to 413Isoleucine: 30 to 186Leucine: 1 to 9Lysine: 2 to 16Methionine: 2 to 53Ornithine: 1 to 5Phenylalanine: 1 to 5PhenylalanineSerum phenylalanine screening is a blood test to look for signs of the disease phenylketonuria (PKU). The test detects abnormally high levels of an ...Read Article Now Book Mark Article Proline: 3 to 13Serine: 0 to 9Taurine: 18 to 89Threonine: 13 to 587Tyrosine: 3 to 14Valine: 3 to 36Normal 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. What Abnormal Results Mean Increased total urine amino acids may be due to:Alkaptonuria Canavan disease Cystinosis Cystathioninuria Fructose intolerance 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 Homocystinuria Hyperammonemia Hyperparathyroidism HyperparathyroidismHyperparathyroidism is a disorder 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 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 in the plasma cells in the bone marrow. Bone marrow is the soft, spongy tissue found inside most bone...ImageRead Article Now Book Mark Article Ornithine transcarbamylase deficiency Osteomalacia OsteomalaciaOsteomalacia is softening of the bones. It most often occurs because of a problem with vitamin D, which helps your body absorb calcium. Your body n...ImageRead Article Now Book Mark Article Propionic acidemia Rickets RicketsRickets is a disorder caused by a lack of vitamin D, calcium, or phosphate. It leads to softening and weakening of the bones.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, 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.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: 4/24/2023 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. 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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 urine culture is a lab test to check for bacteria or other germs in a urine sample. It can be used to check for a urinary tract infection in adults...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 mmol/mol creatinine. The values below represent normal ranges in 24 hours urine for adults.Alanine: 9 to 98Arginine: 0 to 8Asparagine: 10 to 65Aspartic acid: 5 to 50Aspartic 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 22Cystine: 2 to 12Glutamic acid: 0 to 21Glutamine: 11 to 42Glycine: 17 to 146Histidine: 49 to 413Isoleucine: 30 to 186Leucine: 1 to 9Lysine: 2 to 16Methionine: 2 to 53Ornithine: 1 to 5Phenylalanine: 1 to 5PhenylalanineSerum phenylalanine screening is a blood test to look for signs of the disease phenylketonuria (PKU). The test detects abnormally high levels of an ...Read Article Now Book Mark Article Proline: 3 to 13Serine: 0 to 9Taurine: 18 to 89Threonine: 13 to 587Tyrosine: 3 to 14Valine: 3 to 36Normal 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. What Abnormal Results Mean Increased total urine amino acids may be due to:Alkaptonuria Canavan disease Cystinosis Cystathioninuria Fructose intolerance 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 Homocystinuria Hyperammonemia Hyperparathyroidism HyperparathyroidismHyperparathyroidism is a disorder 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 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 in the plasma cells in the bone marrow. Bone marrow is the soft, spongy tissue found inside most bone...ImageRead Article Now Book Mark Article Ornithine transcarbamylase deficiency Osteomalacia OsteomalaciaOsteomalacia is softening of the bones. It most often occurs because of a problem with vitamin D, which helps your body absorb calcium. Your body n...ImageRead Article Now Book Mark Article Propionic acidemia Rickets RicketsRickets is a disorder caused by a lack of vitamin D, calcium, or phosphate. It leads to softening and weakening of the bones.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, 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.