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Archivist: Survival after treatment for hyperammonaemic crises of urea cycle disorders

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Treatment of urea cycle disorders includes a low-protein diet, adequate calorie intake, alternative-pathway treatment for hyperammonaemic crises, and dialysis if needed. Alternative-pathway treatment consists of giving phenylacetate and benzoate so that waste nitrogen is excreted as phenylacetylglutamine and N-benzoylglycine rather than producing ammonia. It was first suggested by Brusilow and colleagues in 1979 and now a multicentre US study extending over 25 years has been reported by researchers including Dr Brusilow …

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