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Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia

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References

  1. Gebhardt B, Vlaho S, Fischer D et al (2003) N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria. Mol Genet Metab 79:303–4

    Article  CAS  PubMed  Google Scholar 

  2. Gebhardt B, Dittrich S, Parbel S et al (2005) N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia. J Inherit Metab Dis 28:241–4

    Article  CAS  PubMed  Google Scholar 

  3. Jones S, Reed CA, Vijay S et al (2008) N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia. J Inherit Metab Dis 31:464. doi:10.1007/s10545-008-0777-1

    Google Scholar 

  4. Levrat V, Forest I, Fouilhoux A et al (2008) Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia? Orphanet J Rare Dis 3:2. doi:10.1186/1750-1172-3-2

    Article  PubMed  Google Scholar 

  5. Tuchman M, Caldovic L, Daikhin Y et al (2008) N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers. Pediatr Res 64:213–7

    Article  CAS  PubMed  Google Scholar 

  6. Gessler P, Buchal P, Schwenk HU, Wermuth B (2009) Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency. Eur J Pediat Jun 17 [epub ahead of print]

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Authors and Affiliations

  1. Department of Metabolic Medicine, Royal Hospital for Sick Children, Dalnair Street, Glasgow, G3 8SJ, UK

    Bernd C. Schwahn & Peter H. Robinson

  2. Department of Biochemistry, Royal Hospital for Sick Children, Glasgow, UK

    Peter G. Galloway

  3. Paediatric Department, Victoria Hospital, Kirkcaldy, UK

    Louisa Pieterse

  4. Royal Aberdeen Children’s Hospital, Aberdeen, UK

    W. Michael Bisset

Authors
  1. Bernd C. Schwahn
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  2. Louisa Pieterse
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  3. W. Michael Bisset
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  4. Peter G. Galloway
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  5. Peter H. Robinson
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Correspondence to Bernd C. Schwahn.

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Schwahn, B.C., Pieterse, L., Bisset, W.M. et al. Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia. Eur J Pediatr 169, 133–134 (2010). https://doi.org/10.1007/s00431-009-1036-7

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  • DOI: https://doi.org/10.1007/s00431-009-1036-7

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