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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গ্রন্থ-পঞ্জীর বিবরন
প্রধান লেখক: 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
বিন্যাস: প্রবন্ধ
ভাষা:English
প্রকাশিত: BMC 2018-01-01
মালা:Orphanet Journal of Rare Diseases
বিষয়গুলি:
Fructose 1,6 bisphosphatase deficiency
Dietary restrictions
Fasting tolerance
Uncooked cornstarch
অনলাইন ব্যবহার করুন:http://link.springer.com/article/10.1186/s13023-018-0760-3

আন্তর্জাল

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

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