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About 2,135 results

ALLMedicine™ Glycogen Storage Disease Center

Research & Reviews  1,059 results

A retrospective in-depth analysis of continuous glucose monitoring datasets for patient...
https://doi.org/10.1002/jimd.12383
Journal of Inherited Metabolic Disease; Peeks F, Hoogeveen IJ et. al.

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...

Type la glycogen storage disease complicated with diabetes mellitus: the role of flash ...
https://doi.org/10.1136/bcr-2020-240489
BMJ Case Reports; Marcalo J, Oliveira A et. al.

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...

Cardiac Phenotype Characterization at MRI in Patients with Danon Disease: A Retrospec...
https://doi.org/10.1148/radiol.2021203996
Radiology Wei X, Zhao L et. al.

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...

Impaired Very-Low-Density Lipoprotein catabolism links hypoglycemia to hypertriglycerid...
https://doi.org/10.1002/jimd.12380
Journal of Inherited Metabolic Disease; Hoogerland JA, Peeks F et. al.

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...

Clinical analysis and long-term treatment monitoring of 3 patients with glycogen storag...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7972195
BMC Medical Genomics; Du C, Li Z et. al.

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...

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Guidelines  4 results

Diagnosis and management of glycogen storage diseases type VI and IX
https://www.acmg.net/PDFLibrary/Diagnosis%20and%20Management%20of%20Glycogen%20Stored%20Diseases%20type%20VI%20and%20IX%20a%20practice%20resource%20of%20ACMG.pdf

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.

The Health Impact of Nighttime Eating: Old and New Perspectives
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4425165/
Nutrients Kinsey,A.,et al

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...

Diagnosis and management of glycogen storage disease type I: a practice guideline of th...
https://doi.org/10.1038/gim.2014.128
Genetics in Medicine : Official Journal of the American C... Kishnani PS, Austin SL et. al.

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 ...

Glycogen storage disease type III diagnosis and management guidelines.
https://doi.org/10.1097/GIM.0b013e3181e655b6
Genetics in Medicine : Official Journal of the American C... Kishnani PS, Austin SL et. al.

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...

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Clinicaltrials.gov  1,067 results

A retrospective in-depth analysis of continuous glucose monitoring datasets for patient...
https://doi.org/10.1002/jimd.12383
Journal of Inherited Metabolic Disease; Peeks F, Hoogeveen IJ et. al.

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...

Type la glycogen storage disease complicated with diabetes mellitus: the role of flash ...
https://doi.org/10.1136/bcr-2020-240489
BMJ Case Reports; Marcalo J, Oliveira A et. al.

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...

Cardiac Phenotype Characterization at MRI in Patients with Danon Disease: A Retrospec...
https://doi.org/10.1148/radiol.2021203996
Radiology Wei X, Zhao L et. al.

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...

Impaired Very-Low-Density Lipoprotein catabolism links hypoglycemia to hypertriglycerid...
https://doi.org/10.1002/jimd.12380
Journal of Inherited Metabolic Disease; Hoogerland JA, Peeks F et. al.

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...

Clinical analysis and long-term treatment monitoring of 3 patients with glycogen storag...
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7972195
BMC Medical Genomics; Du C, Li Z et. al.

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...

see more →

News  3 results

The Health Impact of Nighttime Eating: Old and New Perspectives
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4425165/
Nutrients Kinsey,A.,et al

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...

Update: Radiologic-Pathologic Correlation of Hepatocellular Adenoma
https://www.medscape.com/viewarticle/922023

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...

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Patient Education  2 results see all →