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:Comment

Excretion of certain amino acids is increased in several specific aminoacidurias, such as phenylketonuria and maple syrup urine disease. Dibasic amino acids (lysine, ornithine, arginine, Cystine) are increased in cystinuria. Aminoacidopathies, with ninhydrin – positive urinary amino acids, include phenylketouria, cystinuria, nonketotic hyperglycinemia and homocystinuria. Their investigation includes urine organic acids and amino acids, blood NH3+, biotinidase, and carni-tine. Aminoaciduria may also be seen in a variety of other disorders, including viral hepatitis, multiple myeloma, rickets, hyperparathyrodisim, and chronic renal failure. A positive test should be followed up with quantitation on a 24- hour collection

Sample: Random urine, 24 hr urine