Glycogen Storage Disease Type Ia: Current Management Options, Burden and Unmet Needs

Glycogen storage disease type Ia (GSDIa) is caused by defective glucose-6-phosphatase, a key enzyme in carbohydrate metabolism. Affected individuals cannot release glucose during fasting and accumulate excess glycogen and fat in the liver and kidney, putting them at risk of severe hypoglycaemia and...

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Main Authors:	Terry G. J. Derks, David F. Rodriguez-Buritica, Ayesha Ahmad, Foekje de Boer, María L. Couce, Sarah C. Grünert, Philippe Labrune, Nerea López Maldonado, Carolina Fischinger Moura de Souza, Rebecca Riba-Wolman, Alessandro Rossi, Heather Saavedra, Rupal Naik Gupta, Vassili Valayannopoulos, John Mitchell
Format:	article
Language:	EN
Published:	MDPI AG 2021
Subjects:	glycogen storage disease type Ia dietary treatment uncooked cornstarch burden of disease unmet need long-term complications Nutrition. Foods and food supply TX341-641
Online Access:	https://doaj.org/article/49373e0a313c4a1bbb2b3940a73fdda6
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Glycogen Storage Disease Type Ia: Current Management Options, Burden and Unmet Needs

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