ALLMedicine™ Glycogen Storage Disease Center

Apr 9th, 2021 - Continuous glucose monitoring (CGM) systems have great potential for real-time assessment of glycaemic variation in patients with hepatic glycogen storage disease (GSD). However, detailed descriptions and in-depth analysis of CGM data from hepatic...

Mar 26th, 2021 - A 22-year-old woman with type Ia glycogen storage disease was referred to the endocrinology department with new-onset diabetes mellitus-glycated haemoglobin (HbA1c) of 8.2%. She had suffered from repeated bouts of hypoglycaemia since the first day...

Mar 23rd, 2021 - Danon disease (DD) is a rare X-chromosome-linked dominant lysosomal glycogen storage disease. Its features have seldom been reported by using cardiac MRI. This case series aimed to evaluate cardiac features of DD on the basis of MRI observations f...

Mar 19th, 2021 - Prevention of hypertriglyceridemia is one of the biomedical targets in Glycogen Storage Disease type Ia (GSD Ia) patients, yet it is unclear how hypoglycemia links to plasma triglyceride (TG) levels. We analyzed whole-body TG metabolism in normogl...

Mar 18th, 2021 - To investigate the clinical and genetic characteristics of patients with glycogen storage disease type Ib (GSD Ib). This report retrospectively analyzed the clinical data of 3 patients with GSD Ib admitted into our hospital, and summarized their o...

Jan 18th, 2019 - 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 phosphorylase, an enzyme encoded by the PYGL gene.

Apr 6th, 2015 - Nighttime eating, particularly before bed, has received considerable attention. Limiting and/or avoiding food before nighttime sleep has been proposed as both a weight loss strategy and approach to improve health and body composition. Indeed, nega...

Oct 30th, 2014 - Glycogen storage disease type I (GSD I) is a rare disease of variable clinical severity that primarily affects the liver and kidney. It is caused by deficient activity of the glucose 6-phosphatase enzyme (GSD Ia) or a deficiency in the microsomal ...

Jul 15th, 2010 - Glycogen storage disease type III is a rare disease of variable clinical severity affecting primarily the liver, heart, and skeletal muscle. It is caused by deficient activity of glycogen debranching enzyme, which is a key enzyme in glycogen degra...

Apr 9th, 2021 - Continuous glucose monitoring (CGM) systems have great potential for real-time assessment of glycaemic variation in patients with hepatic glycogen storage disease (GSD). However, detailed descriptions and in-depth analysis of CGM data from hepatic...

Mar 26th, 2021 - A 22-year-old woman with type Ia glycogen storage disease was referred to the endocrinology department with new-onset diabetes mellitus-glycated haemoglobin (HbA1c) of 8.2%. She had suffered from repeated bouts of hypoglycaemia since the first day...

Mar 23rd, 2021 - Danon disease (DD) is a rare X-chromosome-linked dominant lysosomal glycogen storage disease. Its features have seldom been reported by using cardiac MRI. This case series aimed to evaluate cardiac features of DD on the basis of MRI observations f...

Mar 19th, 2021 - Prevention of hypertriglyceridemia is one of the biomedical targets in Glycogen Storage Disease type Ia (GSD Ia) patients, yet it is unclear how hypoglycemia links to plasma triglyceride (TG) levels. We analyzed whole-body TG metabolism in normogl...

Mar 18th, 2021 - To investigate the clinical and genetic characteristics of patients with glycogen storage disease type Ib (GSD Ib). This report retrospectively analyzed the clinical data of 3 patients with GSD Ib admitted into our hospital, and summarized their o...

Apr 6th, 2015 - Nighttime eating, particularly before bed, has received considerable attention. Limiting and/or avoiding food before nighttime sleep has been proposed as both a weight loss strategy and approach to improve health and body composition. Indeed, nega...

May 22nd, 2014 - VANCOUVER, B. C.

Abstract and Introduction Introduction Hepatocellular adenoma (HA) is a relatively uncommon benign liver neoplasm that is typically seen in obese women of childbearing age who are on long-term oral contraceptives.[1] It is also reported to occur i...