Biochemical efficacy of N-carbamylglutamate in neonatal severe hyperammonaemia due to propionic acidaemia-Reference-Cited by-同舟云学术

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

Published:2009-08-14 Issue:1 Volume:169 Page:133-134
ISSN:0340-6199
Container-title:European Journal of Pediatrics
language:en
Short-container-title:Eur J Pediatr

Author:

Schwahn Bernd C.,Pieterse Louisa,Bisset W. Michael,Galloway Peter G.,Robinson Peter H.

Publisher

Springer Science and Business Media LLC

Subject

Pediatrics, Perinatology and Child Health

Link

http://link.springer.com/content/pdf/10.1007/s00431-009-1036-7.pdf

Reference6 articles.

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

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

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

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

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

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