Zobrazeno 1 - 10 of 38 pro vyhledávání: '"C. Narcy"'
1
Liver transplantation in urea cycle disorders
Autor: J. Laurent, Pascale Delonlay, Philippe Jouvet, Pierre Kamoun, Guy Touati, Yann Revillon, C. Narcy, Daniel Rabier, Dominique Jan, J. M. Saudubray
Publikováno v: European Journal of Pediatrics. 158:S055-S059
We report here our experience in the long-term management of 28 patients with citrullinaemia, 13 patients with carbamoyl phosphate synthase deficiency and 15 patients with argininosuccinic aciduria. In addition, we report a national French survey of
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cd2a9f31242416f27ed818ff780ee846
https://doi.org/10.1007/pl00014323
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4
Liver transplantation in propionic acidaemia
Autor: Daniel Rabier, J. Laurent, Pascale Delonlay, C. Narcy, Yann Revillon, Pierre Kamoun, J. M. Saudubray, J. S. Schlenzig, Guy Touati, Philippe Jouvet, Dominique Jan
Publikováno v: European journal of pediatrics. 158
Despite the improvement in dietary therapy during the past 20 years, the overall outcome of severe forms of propionic acidaemia (PA) remains often disappointing. Good results can be obtained at a very high price in terms of medical attention, family
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::092dfc53f88b27ac16e20bfa957cf7f1
https://pubmed.ncbi.nlm.nih.gov/10603102
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6
[Mid-term outcome of 2 cases with maple syrup urine disease: role of liver transplantation in the treatment]
Autor: J C, Netter, G, Cossarizza, C, Narcy, P, Hubert, H, Ogier, Y, Revillon, D, Rabier, J M, Saudubray
Publikováno v: Archives de pediatrie : organe officiel de la Societe francaise de pediatrie. 1(8)
Dietary treatment of maple syrup urine disease remains difficult; chronic nutritional support in the child does not always avoid acute crises so that liver transplantation may represent an alternate choice in some cases.Two gypsy cousins were born by
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::e11301f2a826b99f9da9156d8b107692
https://pubmed.ncbi.nlm.nih.gov/7842107
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7
Contribution of odd-chain fatty acid oxidation to propionate production in disorders of propionate metabolism
Autor: J M Saudubray, A Mariotti, G N Thompson, F Poggi, D Sbaï, C Narcy, J L Bresson
Publikováno v: American Journal of Clinical Nutrition
American Journal of Clinical Nutrition, American Society for Nutrition, 1994, 59, pp.1332-1337
The American Journal of Clinical Nutrition
The American Journal of Clinical Nutrition, 1994, 59, pp.1332-1337
Whole-body propionate and protein kinetics and energy substrate metabolism were studied in five metronidazole-treated patients with propionic or methylmalonic acidemias by the use of a primed, 4-h constant infusion of [1-13C]propionate and L-[O-2H5]p
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::76f016da51e1a59a671d54fd60b03e86
https://hal.inrae.fr/hal-02701260
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9
Clinical approach to inherited metabolic disorders in neonates
Autor: J.M. Saudubray, C. Narcy, L. Lyonnet, J.P. Bonnefont, B.T. Poll The, A. Munnich
Publikováno v: Biology of the neonate, 58(Suppl. 1), 44-53. S. Karger AG
Europe PubMed Central
Most inborn errors of intermediary metabolism presenting in the neonatal period fall schematically into three clinical categories: (1) those which lead to a neurological distress ‘intoxication type’ with a symptom-free interval, vomiting, comas,
Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::bcdad415160069b2445c1475586b18a3
https://pubmed.ncbi.nlm.nih.gov/2265219
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