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

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 th... Full description

Journal Title: European journal of pediatrics 2007, Vol.167 (3), p.305-309
Main Author: Leonard, James Vivian
Other Authors: Ward Platt, Martin Peter , Morris, Andrew Alan Myles
Format: Electronic Article Electronic Article
Language: English
Subjects:
Quelle: Alma/SFX Local Collection
Publisher: Berlin/Heidelberg: Springer-Verlag
ID: ISSN: 0340-6199
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title: Hypothesis: proposals for the management of a neonate at risk of hyperammonaemia due to a urea cycle disorder
format: Article
creator:
  • Leonard, James Vivian
  • Ward Platt, Martin Peter
  • Morris, Andrew Alan Myles
subjects:
  • Adrenergic beta-Antagonists - therapeutic use
  • Aminoacid disorders
  • Biological and medical sciences
  • Cesarean Section
  • Disease susceptibility
  • Errors of metabolism
  • General aspects
  • Glucose - therapeutic use
  • Humans
  • Hyperammonemia - etiology
  • Hyperammonemia - prevention & control
  • Infant, Newborn
  • Infants (Newborn)
  • Insulin - therapeutic use
  • Medical sciences
  • Medicine
  • Medicine & Public Health
  • Metabolic diseases
  • Metabolism, Inborn Errors - complications
  • Octreotide - therapeutic use
  • Original Paper
  • Pediatrics
  • Risk Factors
  • Sodium benzoate
  • Urea
  • Urea - metabolism
ispartof: European journal of pediatrics, 2007, Vol.167 (3), p.305-309
description: 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 β-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.
language: eng
source: Alma/SFX Local Collection
identifier: ISSN: 0340-6199
fulltext: fulltext
issn:
  • 0340-6199
  • 1432-1076
url: Link


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descriptionIt 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 β-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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subjectAdrenergic beta-Antagonists - therapeutic use ; Aminoacid disorders ; Biological and medical sciences ; Cesarean Section ; Disease susceptibility ; Errors of metabolism ; General aspects ; Glucose - therapeutic use ; Humans ; Hyperammonemia - etiology ; Hyperammonemia - prevention & control ; Infant, Newborn ; Infants (Newborn) ; Insulin - therapeutic use ; Medical sciences ; Medicine ; Medicine & Public Health ; Metabolic diseases ; Metabolism, Inborn Errors - complications ; Octreotide - therapeutic use ; Original Paper ; Pediatrics ; Risk Factors ; Sodium benzoate ; Urea ; Urea - metabolism
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descriptionIt 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 β-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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abstractIt 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 β-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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