Bonnefont JP - Search Results

1

In vivo propionate oxidation as a prognostic indicator in disorders of propionate metabolism.

Thompson GN, Walter JH, Bresson JL, Bonnefont JP, Saudubray JM, Leonard JV, Halliday D. Thompson GN, et al. Among authors: bonnefont jp. Eur J Pediatr. 1990 Mar;149(6):408-11. doi: 10.1007/BF02009661. Eur J Pediatr. 1990. PMID: 2332010

2

A simple isotopic technique for assessing vitamin responsiveness in vivo in propionic acidaemia.

Thompson GN, Bresson JL, Bonnefont JP, Walter JH, Read MA, Saudubray JM, Leonard JV, Halliday D. Thompson GN, et al. Among authors: bonnefont jp. J Inherit Metab Dis. 1990;13(3):349-51. doi: 10.1007/BF01799392. J Inherit Metab Dis. 1990. PMID: 2122099 No abstract available.

3

Substrate disposal in metabolic disease: a comparison between rates of in vivo propionate oxidation and urinary metabolite excretion in children with methylmalonic acidemia.

Thompson GN, Walter JH, Bresson JL, Ford GC, Bonnefont JP, Chalmers RA, Saudubray JM, Leonard JV, Halliday D. Thompson GN, et al. Among authors: bonnefont jp. J Pediatr. 1989 Nov;115(5 Pt 1):735-9. doi: 10.1016/s0022-3476(89)80651-1. J Pediatr. 1989. PMID: 2809905

4

Protein and leucine metabolism in maple syrup urine disease.

Thompson GN, Bresson JL, Pacy PJ, Bonnefont JP, Walter JH, Leonard JV, Saudubray JM, Halliday D. Thompson GN, et al. Among authors: bonnefont jp. Am J Physiol. 1990 Apr;258(4 Pt 1):E654-60. doi: 10.1152/ajpendo.1990.258.4.E654. Am J Physiol. 1990. PMID: 2185648

5

The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states.

Bonnefont JP, Specola NB, Vassault A, Lombes A, Ogier H, de Klerk JB, Munnich A, Coude M, Paturneau-Jouas M, Saudubray JM. Bonnefont JP, et al. Eur J Pediatr. 1990 Dec;150(2):80-5. doi: 10.1007/BF02072043. Eur J Pediatr. 1990. PMID: 2279514

6

Clinical outcome and long-term management of 17 patients with propionic acidaemia.

van der Meer SB, Poggi F, Spada M, Bonnefont JP, Ogier H, Hubert P, Depondt E, Rapoport D, Rabier D, Charpentier C, Parvy P, Bardet J, Kamoun P, Saudubray JM. van der Meer SB, et al. Among authors: bonnefont jp. Eur J Pediatr. 1996 Mar;155(3):205-10. doi: 10.1007/BF01953939. Eur J Pediatr. 1996. PMID: 8929729

7

Human trifunctional protein deficiency: a new disorder of mitochondrial fatty acid beta-oxidation.

Wanders RJ, IJlst L, Poggi F, Bonnefont JP, Munnich A, Brivet M, Rabier D, Saudubray JM. Wanders RJ, et al. Among authors: bonnefont jp. Biochem Biophys Res Commun. 1992 Nov 16;188(3):1139-45. doi: 10.1016/0006-291x(92)91350-y. Biochem Biophys Res Commun. 1992. PMID: 1445348

8

Aqueous humour, a possible material for postmortem methylmalonic acidaemia diagnosis.

Coude M, Bonnefont JP, Charpentier C, Chadefaux B, Saudubray JM, Kamoun P. Coude M, et al. Among authors: bonnefont jp. J Inherit Metab Dis. 1989;12(1):95-6. doi: 10.1007/BF01805538. J Inherit Metab Dis. 1989. PMID: 2501591 No abstract available.

9

Mitochondrial DNA rearrangements with onset as chronic diarrhea with villous atrophy.

Cormier-Daire V, Bonnefont JP, Rustin P, Maurage C, Ogler H, Schmitz J, Ricour C, Saudubray JM, Munnich A, Rötig A. Cormier-Daire V, et al. Among authors: bonnefont jp. J Pediatr. 1994 Jan;124(1):63-70. doi: 10.1016/s0022-3476(94)70255-1. J Pediatr. 1994. PMID: 8283377

10

Approach to the patient with a fatty acid oxidation disorder.

Saudubray JM, Mitchell G, Bonnefont JP, Schwartz G, Nuttin C, Munnich A, Brivet M, Vassault A, Demaugre F, Rabier D, et al. Saudubray JM, et al. Among authors: bonnefont jp. Prog Clin Biol Res. 1992;375:271-88. Prog Clin Biol Res. 1992. PMID: 1438373 Review. No abstract available.

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