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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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
פורמט:	Article
שפה:	English
יצא לאור:	BMC 2018-01-01
סדרה:	Orphanet Journal of Rare Diseases
נושאים:	Fructose 1,6 bisphosphatase deficiency Dietary restrictions Fasting tolerance Uncooked cornstarch
גישה מקוונת:	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

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

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