J. Bardet
Necker-Enfants Malades Hospital
27 Papers
205 Citations
J. Bardet is an academic researcher from Necker-Enfants Malades Hospital. The author has contributed to research in topics: Arginine & Citrulline. The author has an hindex of 11, co-authored 25 publications.
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Papers
Early alterations of plasma free amino acids in chronic renal failure.
TL;DR: Changes in the plasma concentration of several non essential amino acids are already present in the early stage of renal failure in patients with no sign of protein malnutrition: these may result from altered metabolic pathways of amino acids related to uremia and/or nephron loss per se.
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Age-related reference values for free amino acids in first morning urine specimens.
TL;DR: Age-related reference values for urinary free amino acids in first morning urine specimens from 360 control subjects were determined and the use of these results to detect aminoacidopathies and tubulopathies is discussed.
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Arginine remains an essential amino acid after liver transplantation in urea cycle enzyme deficiencies.
TL;DR: The experience of two patients whose livers were replaced because of OTC deficiency and argininosuccinate synthetase (ASS) deficiency is reported, suggesting the importance of the extrahepatic parts of the urea cycle.
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Persistent hypocitrullinaemia as a marker for mtDNA NARP T 8993 G mutation
Daniel Rabier,C. Diry,Agnès Rötig,P. Rustin,Bénédicte Héron,J. Bardet,P. Parvy,G. Ponsot,Cécile Marsac,J. M. Saudubray,Arnold Munnich,P. Kamoun +11 more
TL;DR: A new situation in which hypocitrullinaemia was observed in 5 patients with mt ATP6 NARP T 8993 G mutation, which impairs the function of the subunit a or ATPase 6 of the Fo segment of the complex V.
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Therapeutic use of carbamylglutamate in the case of carbamoyl-phosphate synthetase deficiency.
G. Kuchler,Daniel Rabier,F. Poggi-Travert,D. Meyer-Gast,J. Bardet,V. Drouin,M. Cadoudal,J. M. Saudubray +7 more
TL;DR: Treatment with CG in a kinetic variant or a partial deficit of CPS has never been clinically proven until now, and the first use has been in N-acetylglutamate synthetase deficiency.
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