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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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Detalles Bibliográficos
Autores principales: 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.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5785792/
https://www.ncbi.nlm.nih.gov/pubmed/29370874
http://dx.doi.org/10.1186/s13023-018-0760-3