Maple Syrup Urine Disease

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ketoacidemia maple syrup urine disease Hereditary disease due to deficiency of an enzyme involved in amino acid metabolism, characterised by urine that smells like maple syrup. Previous: MAP kinase

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or marked liver impairment, or leucine crystals in patients with severe liver disease or with maple syrup urine disease. Oxalate crystals in urine Triple phosphate crystals in urine Cystine

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Three of these are PKU; branched-chain ketoaciduria, or maple syrup urine disease (MSUD); and methylmalonic acidemia (MMA). Phenylketonuria: PKU is an inherited condition in which the amino acid

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