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Amino Acids What are they and what do they do This potentially life-threatening condition branched-chain ketoaciduria often referred to as maple syrup urine disease because keto acids released into the urine cause it to smell like maple syrup Maple syrup urine disease Genetics Home Reference S National Library of Medicine Home What's New Browse Handbook Glossary Resources About Help Search Maple syrup urine disease Related Gene(s Quick links to this topic MedlinePlus Health Information Health Bureau of LaboratoriesGeneral Information It performs approximately180,000 tests each year for approximately 100 different diseases including HIV rabies blood lead hypothyroidism phenylketonuria tuberculosis and maple syrup urine disease

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