
Glycogen Storage Disease Type I - GeneReviews® - NCBI Bookshelf
Apr 19, 2006 · Glycogen storage disease type I (GSD I) is characterized by accumulation of glycogen and fat in the liver and kidneys resulting in hepatomegaly and nephromegaly. …
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Dietary Management of the Glycogen Storage Diseases: Evolution of ...
We examine historic and current approaches for preventing hypoglycemia associated with GSDs. There is a lack of consensus on the optimal dietary management of GSDs despite decades of …
Glycogen Storage Disease Type III - GeneReviews® - NCBI Bookshelf
Mar 9, 2010 · Glycogen storage disease type III (GSD III) is characterized by variable liver, cardiac muscle, and skeletal muscle involvement. GSD IIIa is the most common subtype, …
Glycogen Storage Disease - StatPearls - NCBI Bookshelf
Jan 21, 2025 · Glycogen storage diseases (GSDs) are inherited inborn errors of carbohydrate metabolism that result in abnormal glycogen storage. The onset can range from neonatal life …
Hepatic glycogen storage diseases: pathogenesis, clinical …
Glycogen storage diseases (GSDs) are genetically determined metabolic diseases that cause disorders of glycogen metabolism in the body. Due to the enzymatic defect at some stage of …
Diagnosis and management of glycogen storage diseases type VI ... - PubMed
Purpose: Glycogen storage disease (GSD) types VI and IX are rare diseases of variable clinical severity affecting primarily the liver. GSD VI is caused by deficient activity of hepatic glycogen …
Glycogen storage disease type III: diagnosis, genotype ... - PubMed
Glycogen storage disease type III (GSDIII) is a rare disorder of glycogenolysis due to AGL gene mutations, causing glycogen debranching enzyme deficiency and storage of limited dextrin. …
Usefulness of a Digitally Assisted Person-Centered Care ... - PubMed
May 18, 2023 · The intervention was adapted into a digital version called digitally assisted guided self-determination (DA-GSD) hosted by the Sundhed DK website that can be delivered face-to …
Von Gierke: fatos, causas e tratamento - liverfoundation.org
A doença de armazenamento de glicogênio tipo I (GSD I), também conhecida como doença de von Gierke, é a forma mais comum de doença de armazenamento de glicogênio, sendo …