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

Pinto, A.; Alfadhel, M.; Akroyd, R.; Atik Altınok, Y.; Bernabei, S.; Bernstein, L.; Bruni, G.; Caine, G.; Cameron, E.; Carruthers, R.; Cochrane, B.

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 of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose...

Date: 2018   |   Origin: Repositório Científico da Unidade Local de Saúde de Santo António (ULSSA)
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