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Related to argininosuccinic acid: Argininosuccinic aciduria
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In the first study, in which a dilute urine was used, mean recoveries were 87-122%, with the exceptions of argininosuccinic acid (132%) and aminobutyric acid (81%).
BUPHENYL(R) is indicated as adjunctive therapy in the chronic management of patients with urea cycle disorders involving deficiencies of carbamylphosphate synthetase (CPS), ornithine transcarbamylase (OTC), or argininosuccinic acid synthetase (AS).
IS, internal standard; NA, not applied; Ile, isoleucine; TYR, tyrosinemia; MSUD, maple syrup urine disease; HCYS, homocystinuria; NKG, nonketotic hyperglycinemia; ASD, argininosuccinic acid synthetase deficiency; ALD, argininosuccinase deficiency; PYG/PIP, pyroglutamic/pipecolic acidemia; PRO, hyperprolinemia.