Hypothesis: proposals for the management of a neonate at risk of hyperammonaemia due to a urea cycle disorder

  1. Lookup NU author(s)
  2. Dr Martin Ward Platt
  3. Dr Andrew Morris
Author(s)Leonard JV, Ward Platt MP, Morris AA
Publication type Article
JournalEuropean Journal of Pediatrics
Year2008
Volume167
Issue3
Pages305-309
ISSN (print)0340-6199
ISSN (electronic)1432-1076
Full text for this publication is not currently held within this repository. Alternative links are provided below where available.
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 ?-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.
PublisherSpringer
URLhttp://dx.doi.org/10.1007/s00431-007-0486-z
DOI10.1007/s00431-007-0486-z
PubMed id17436013
Actions    Link to this publication
Share