Literature DB >> 19680687

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

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

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Year:  2009        PMID： 19680687     DOI： 10.1007/s00431-009-1036-7

Source DB:  PubMed          Journal:  Eur J Pediatr        ISSN： 0340-6199            Impact factor:   3.183


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  6 in total

1.  N-carbamylglutamate protects patients with decompensated propionic aciduria from hyperammonaemia.

Authors:  B Gebhardt; S Dittrich; S Parbel; S Vlaho; O Matsika; H Bohles
Journal:  J Inherit Metab Dis       Date:  2005       Impact factor: 4.982

2.  Favourable long-term outcome after immediate treatment of neonatal hyperammonemia due to N-acetylglutamate synthase deficiency.

Authors:  Peter Gessler; Peter Buchal; Hans U Schwenk; Bendicht Wermuth
Journal:  Eur J Pediatr       Date:  2009-06-17       Impact factor: 3.183

3.  Carglumic acid: an additional therapy in the treatment of organic acidurias with hyperammonemia?

Authors:  Virginie Levrat; Isabelle Forest; Alain Fouilhoux; Cécile Acquaviva; Christine Vianey-Saban; Nathalie Guffon
Journal:  Orphanet J Rare Dis       Date:  2008-01-30       Impact factor: 4.123

4.  N-carbamylglutamate enhances ammonia detoxification in a patient with decompensated methylmalonic aciduria.

Authors:  Boris Gebhardt; Stefan Vlaho; Doris Fischer; Adrian Sewell; Hansjosef Böhles
Journal:  Mol Genet Metab       Date:  2003-08       Impact factor: 4.797

5.  N-carbamylglutamate for neonatal hyperammonaemia in propionic acidaemia.

Authors:  S Jones; C A B Reed; S Vijay; J H Walter; A A M Morris
Journal:  J Inherit Metab Dis       Date:  2008-02-21       Impact factor: 4.982

6.  N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers.

Authors:  Mendel Tuchman; Ljubica Caldovic; Yevgeny Daikhin; Oksana Horyn; Ilana Nissim; Itzhak Nissim; Mark Korson; Barbara Burton; Marc Yudkoff
Journal:  Pediatr Res       Date:  2008-08       Impact factor: 3.756
  6 in total
  22 in total

Review 1.  Acute management of propionic acidemia.

Authors:  Kimberly A Chapman; Andrea Gropman; Erin MacLeod; Kathy Stagni; Marshall L Summar; Keiko Ueda; Nicholas Ah Mew; Jill Franks; Eddie Island; Dietrich Matern; Loren Pena; Brittany Smith; V Reid Sutton; Tiina Urv; Charles Venditti; Anupam Chakrapani
Journal:  Mol Genet Metab       Date:  2011-09-24       Impact factor: 4.797

2.  Nutritional and Pharmacological Management during Chemotherapy in a Patient with Propionic Acidaemia and Rhabdomyosarcoma Botryoides.

Authors:  E Martín-Hernández; P Quijada-Fraile; L Oliveros-Leal; Mt García-Silva; C Pérez-Cerdá; M Baro-Fernández; V Pérez-Alonso; Jl Vivanco
Journal:  JIMD Rep       Date:  2012-03-21

3.  Short-term outcome of propionic aciduria treated at presentation with N-carbamylglutamate: a retrospective review of four patients.

Authors:  Sébastien Lévesque; Marie Lambert; Aspasia Karalis; Serge Melancon; Laura Russell; Nancy Braverman
Journal:  JIMD Rep       Date:  2011-09-06

4.  Stable isotopes in the diagnosis and treatment of inherited hyperammonemia.

Authors:  Nicholas Ah Mew; Marc Yudkoff; Mendel Tuchman
Journal:  J Pediatr Biochem       Date:  2014-01-01

5.  N-carbamylglutamate treatment for acute neonatal hyperammonemia in isovaleric acidemia.

Authors:  Cigdem Seher Kasapkara; Fatih Suheyl Ezgu; Ilyas Okur; Leyla Tumer; Gursel Biberoglu; Alev Hasanoglu
Journal:  Eur J Pediatr       Date:  2011-01-05       Impact factor: 3.183

6.  Precision medicine in rare disease: Mechanisms of disparate effects of N-carbamyl-l-glutamate on mutant CPS1 enzymes.

Authors:  Dashuang Shi; Gengxiang Zhao; Nicholas Ah Mew; Mendel Tuchman
Journal:  Mol Genet Metab       Date:  2016-12-08       Impact factor: 4.797

7.  N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia.

Authors:  Nicholas Ah Mew; Robert McCarter; Yevgeny Daikhin; Itzhak Nissim; Marc Yudkoff; Mendel Tuchman
Journal:  Pediatrics       Date:  2010-06-21       Impact factor: 7.124

8.  Cobalamin C defect presenting as severe neonatal hyperammonemia.

Authors:  Diego Martinelli; Andrea Dotta; Laura Massella; Stefano Picca; Alessandra Di Pede; Sara Boenzi; Chiara Aiello; Carlo Dionisi-Vici
Journal:  Eur J Pediatr       Date:  2010-12-10       Impact factor: 3.183

Review 9.  Methylmalonic and propionic acidemias: clinical management update.

Authors:  Jamie L Fraser; Charles P Venditti
Journal:  Curr Opin Pediatr       Date:  2016-12       Impact factor: 2.856

Review 10.  Liver transplantation in propionic and methylmalonic acidemia: A single center study with literature review.

Authors:  Nishitha R Pillai; Bridget M Stroup; Anna Poliner; Linda Rossetti; Brandy Rawls; Brian J Shayota; Claudia Soler-Alfonso; Hari Priya Tunuguntala; John Goss; William Craigen; Fernando Scaglia; V Reid Sutton; Ryan Wallace Himes; Lindsay C Burrage
Journal:  Mol Genet Metab       Date:  2019-11-07       Impact factor: 4.797
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