ArticleEuropean journal of pediatrics2008
Hypothesis: proposals for the management of a neonate at risk of hyperammonaemia due to a urea cycle disorder.
Article in European journal of pediatrics, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
8 citing papers in PubMed, 20 citations in OpenAlex.
- The circulatory arrest recovery ammonia problem (CARAP) hypothesis: A proton magnetic resonance spectroscopy (The Journal of thoracic and cardiovascular surgery · 2025Article
- Fifteen years of urea cycle disorders brain research: Looking back, looking forward.Analytical biochemistry · 2022Review
- Urea cycle disorders-update.Journal of human genetics · 2019Review
- Efficacy and safety of i.v. sodium benzoate in urea cycle disorders: a multicentre retrospective study.Orphanet journal of rare diseases · 2016Article
- High glucose intake and glycaemic level in critically ill neonates with inherited metabolic disorders of intoxication.European journal of pediatrics · 2016Article
- Ammonia toxicity to the brain.Journal of inherited metabolic disease · 2013Review
- Suggested guidelines for the diagnosis and management of urea cycle disorders.Orphanet journal of rare diseases · 2012Review
- New developments in the treatment of hyperammonemia: emerging use of carglumic acid.International journal of general medicine · 2011Article
Corrections and comments
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Authors and funding
3 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
It is difficult to prevent hyperammonaemia in patients with urea cycle disorders that present in the newborn period. This is true, even if treatment is started prospectively because of an affected relative. We propose several additional measures that could be used in conjunction with conventional therapy to improve the metabolic control. Catabolism could be reduced by delivering the babies by elective caesarean section, by starting intravenous glucose immediately after delivery and, possibly, by using beta-blockers or octreotide and insulin. The effectiveness of sodium benzoate and sodium phenylbutyrate might be increased by giving phenobarbital to the mother before delivery and subsequently to the baby to induce the enzymes for conjugation. We would expect the proposed measures to reduce the risk of hyperammonaemia and to improve the outcome for these patients. They have not, however, previously been used in this context, so families would need to be counselled carefully and controlled studies should be undertaken.
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Registered trials
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