Glycemic control and complications in glycogen storage disease type I: results from the Swiss registry

N Kaiser, M Gautschi, L Bosanska, F Meienberg… - Molecular genetics and …, 2019 - Elsevier
Background Regular carbohydrate intake to avoid hypoglycemia is the mainstay of dietary
treatment in glycogen storage disease type I (GSDI). The aim of this study was to evaluate
the quality of dietary treatment and glycemic control in a cohort of GSDI patients, in relation
to the presence of typical long-term complications. Methods Data of 25 patients (22 GSD
subtype Ia and 3 GSDIb, median age 20y) from the Swiss hepatic glycogen storage disease
registry was analyzed cross-sectionally. Frequency and type of hypoglycemia symptoms …