The hepatic glycogen storage diseases ? problems beyond childhood

1995 ◽  
Vol 18 (4) ◽  
pp. 462-472 ◽  
Author(s):  
P. J. Lee ◽  
J. V. Leonard
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Storage Diseases ◽  
Glycogen Storage Diseases

scholarly journals Hepatic glycogen storage diseases are associated to microbial dysbiosis

PLoS ONE ◽  
2019 ◽  
Vol 14 (4) ◽  
pp. e0214582 ◽  
Author(s):  
Karina Colonetti ◽  
Bruna Bento dos Santos ◽  
Tatiéle Nalin ◽  
Carolina Fischinger Moura de Souza ◽  
Eric W. Triplett ◽  
...  
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Storage Diseases ◽  
Microbial Dysbiosis ◽  
Glycogen Storage Diseases

The prevalence of polycystic ovaries in the hepatic glycogen storage diseases: its association with hyperinsulinism

1995 ◽  
Vol 42 (6) ◽  
pp. 601-606 ◽  
Author(s):  
Philip J. Lee ◽  
Anita Patel ◽  
Peter C. Hindmarsh ◽  
Alex P. Mowat ◽  
James V. Leonard
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Polycystic Ovaries ◽  
Storage Diseases ◽  
Glycogen Storage Diseases

scholarly journals The multiple faces of urinary glucose tetrasaccharide as biomarker for patients with hepatic glycogen storage diseases

2020 ◽  
Vol 22 (11) ◽  
pp. 1915-1916 ◽  
Author(s):  
M. Rebecca Heiner-Fokkema ◽  
Jennifer van der Krogt ◽  
Foekje de Boer ◽  
Marieke J. Fokkert-Wilts ◽  
Ronald G. H. J. Maatman ◽  
...  
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Storage Diseases ◽  
Glycogen Storage Diseases ◽  
Urinary Glucose ◽  
Glucose Tetrasaccharide

scholarly journals Hepatic glycogen storage diseases: pathogenesis, clinical symptoms and therapeutic management

2019 ◽  
Author(s):  
Edyta Szymańska ◽  
Dominika A. Jóźwiak-Dzięcielewska ◽  
Joanna Gronek ◽  
Marta Niewczas ◽  
Wojciech Czarny ◽  
...  
Keyword(s):  
Clinical Symptoms ◽  
Glycogen Storage ◽  
Therapeutic Management ◽  
Hepatic Glycogen ◽  
Storage Diseases ◽  
Glycogen Storage Diseases

scholarly journals Correction: Hepatic glycogen storage diseases are associated to microbial dysbiosis

PLoS ONE ◽  
2019 ◽  
Vol 14 (6) ◽  
pp. e0218254
Author(s):  
Karina Colonetti ◽  
Bruna Bento dos Santos ◽  
Tatiéle Nalin ◽  
Carolina Fischinger Moura de Souza ◽  
Eric W. Triplett ◽  
...  
Keyword(s):  
Glycogen Storage ◽  
Hepatic Glycogen ◽  
Storage Diseases ◽  
Microbial Dysbiosis ◽  
Glycogen Storage Diseases

Prolonged Inhibition of Glycogen Phosphorylase in Livers of Zucker Diabetic Fatty Rats Models Human Glycogen Storage Diseases

2010 ◽  
Vol 38 (3) ◽  
pp. 393-401 ◽  
Author(s):  
Eike Floettmann ◽  
Laraine Gregory ◽  
Joanne Teague ◽  
John Myatt ◽  
Clare Hammond ◽  
...  
Keyword(s):  
Diabetes Mellitus ◽  
Glycogen Phosphorylase ◽  
Glycogen Storage ◽  
Prolonged Treatment ◽  
Hepatic Glycogen ◽  
Histopathological Analysis ◽  
Storage Diseases ◽  
Glycogen Storage Diseases ◽  
Zucker Diabetic Fatty Rats ◽  
Hepatic Glycogenosis

The preclinical efficacy and safety of GPi921, a glycogen phosphorylase inhibitor, was assessed following twenty-eight days of administration to Zucker Diabetic Fatty (ZDF) rats. The ZDF rat is an animal model of type 2 diabetes mellitus (TTDM) which develops severe hyperglycemia. Inhibition of glycogen phosphorylase throughout the duration of the study was demonstrated by reductions in twenty-four-hour glucose profiles and glycated hemoglobin levels. In addition, progression towards hyperglycemia was halted in treated but not control animals, which developed hyperglycemia over the twenty-eight days of the study. Biochemical and histopathological analysis revealed large increases in hepatic glycogen, which closely paralleled the development of hepatomegaly and ultimately resulted in increases in hepatic lipids. Furthermore, prolonged glycogen phosphorylase inhibition resulted in an increased incidence and severity of other adverse pathological findings in the liver, such as inflammation, fibrosis, hemorrhage, and necrosis. The observed biochemical and histopathological phenotype of the liver closely resembled that seen in severe cases of human glycogen storage diseases (GSD) and hepatic glycogenosis in poorly controlled diabetes mellitus. These findings revealed that although glycogen phosphorylase inhibitors are efficacious agents for the control of hyperglycemia, prolonged treatment might have the potential to cause significant clinical hepatic complications that resemble those seen in GSD and hepatic glycogenosis.

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