International practices in the dietary management of fructose 1-6 biphosphatase deficiency

International practices in the dietary management of fructose 1-6 biphosphatase deficiency

Abstract Background In fructose 1,6 bisphosphatase (FBPase) deficiency, management aims to prevent hypoglycaemia and lactic acidosis by avoiding prolonged fasting, particularly during febrile illness. Although the need for an emergency regimen to avoid metabolic decompensation is well established at...

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Detalles Bibliográficos
Main Authors: A. Pinto, M. Alfadhel, R. Akroyd, Y. Atik Altınok, S. M. Bernabei, L. Bernstein, G. Bruni, G. Caine, E. Cameron, R. Carruthers, B. Cochrane, A. Daly, F. de Boer, S. Delaunay, A. Dianin, M. Dixon, E. Drogari, S. Dubois, S. Evans, J. Gribben, G. Gugelmo, C. Heidenborg, I. Hunjan, I. L. Kok, B. Kumru, A. Liguori, D. Mayr, E. Megdad, U. Meyer, R. B. Oliveira, A. Pal, A. Pozzoli, R. Pretese, J. C. Rocha, S. Rosenbaum-Fabian, J. Serrano-Nieto, E. Sjoqvist, C. Timmer, L. White, T. van den Hurk, M. van Rijn, H. Zweers, M. Ziadlou, A. MacDonald
Formato: Artigo
Idioma:English
Publicado: BMC 2018-01-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Fructose 1,6 bisphosphatase deficiency
Dietary restrictions
Fasting tolerance
Uncooked cornstarch
Acceso en liña:http://link.springer.com/article/10.1186/s13023-018-0760-3

Internet

http://link.springer.com/article/10.1186/s13023-018-0760-3

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