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citrullinemia/kuhara

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Neonatal citrllinemia: treatment with keto-analogues of essential amino acids.

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Ingia / Ingia
A patient with neonatal onset citrullinemia survived to 8 months of age when treated with a mixture of essential amino acids and their keto-analogues. The initial plasma citrulline concentration was 2.7 mM; the blood ammonia concentration was greater than 500 muM. During the first week of therapy,
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