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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Главные авторы: 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
Формат: Статья
Язык:English
Опубликовано: BMC 2018-01-01
Серии:Orphanet Journal of Rare Diseases
Предметы:
Fructose 1,6 bisphosphatase deficiency
Dietary restrictions
Fasting tolerance
Uncooked cornstarch
Online-ссылка:http://link.springer.com/article/10.1186/s13023-018-0760-3

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

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