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

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 times o...

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發表在:Orphanet J Rare Dis
Main Authors: Pinto, A., Alfadhel, M., Akroyd, R., Atik Altınok, Y., Bernabei, S. M., Bernstein, L., Bruni, G., Caine, G., Cameron, E., Carruthers, R., Cochrane, B., Daly, A., de Boer, F., Delaunay, S., Dianin, A., Dixon, M., Drogari, E., Dubois, S., Evans, S., Gribben, J., Gugelmo, G., Heidenborg, C., Hunjan, I., Kok, I. L., Kumru, B., Liguori, A., Mayr, D., Megdad, E., Meyer, U., Oliveira, R. B., Pal, A., Pozzoli, A., Pretese, R., Rocha, J. C., Rosenbaum-Fabian, S., Serrano-Nieto, J., Sjoqvist, E., Timmer, C., White, L., van den Hurk, T., van Rijn, M., Zweers, H., Ziadlou, M., MacDonald, A.
格式: Artigo
語言:Inglês
出版: BioMed Central 2018
主題:
Research
在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC5785792/
https://ncbi.nlm.nih.gov/pubmed/29370874
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/s13023-018-0760-3
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