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 times of illness, there is uncertainty about the need for other dietary management strategies such as sucrose or fructose restriction. We assessed international differences in the dietary management of FBPase deficiency. METHODS:A cross-sectional questionnaire (13 questions) was emailed to all members of the Society for the Study of Inborn Errors of Metabolism (SSIEM) and a wide database of inherited metabolic disorder dietitians. RESULTS:Thirty-six centres reported the dietary prescriptions of 126 pati

SUBMITTER: Pinto A 

PROVIDER: S-EPMC5785792 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature