Glycogen Storage Disease Type I

Known as: GSD Ia, HEPATORENAL GLYCOGENOSIS, disease glycogen i storage type 
An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose… (More)
National Institutes of Health

Topic mentions per year

Topic mentions per year

1935-2018
05101519352017

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2013
2013
BACKGROUND & AIMS Hepatocellular adenomas (HCA) are benign liver tumors mainly related to oral contraception and classified into… (More)
  • table 1
  • figure 1
  • table 2
  • table 3
  • figure 2
Is this relevant?
2009
2009
BACKGROUND AND OBJECTIVES Renal failure is a major complication in glycogen storage disease type I (GSD I). We studied the… (More)
  • figure 1
  • figure 2
  • figure 3
  • table 1
  • table 2
Is this relevant?
2005
2005
We present a series of 8 patients (6 males, 2 females) with hepatocellular carcinoma (HCC) and glycogen storage disease type Ia… (More)
  • table 2
  • table 2
Is this relevant?
2004
2004
OBJECTIVE To investigate brain morphology and function in patients with glycogen storage disease type I (GSDI). STUDY DESIGN… (More)
Is this relevant?
Highly Cited
2002
Highly Cited
2002
Glycogen storage disease type I (GSD I) is a relatively rare metabolic disease and therefore, no metabolic centre has experience… (More)
  • table 1
  • figure 1
  • figure 2
  • table 2
  • table 3
Is this relevant?
2002
2002
Glycogen storage disease type I (GSD I) (McKusick 232200) is caused by inherited defects of the glucose-6-phosphatase complex… (More)
  • table 1
Is this relevant?
2001
2001
Glycogen storage disease type I (GSD I) is characterized by impaired production of glucose from glycogenolysis and… (More)
  • figure 1
  • figure 2
  • table 1
  • table 2
  • figure 5
Is this relevant?
1996
1996
Glycogen storage disease type I (GSD I) is caused by a deficiency in one of the components of the glucose-6-phosphatase (G-6-Pase… (More)
  • table 1
Is this relevant?
1988
1988
With the advent of nocturnal intragastric feeding which protects against acute metabolic complications and promotes growth… (More)
  • table 1
  • table 2
  • table 3
  • figure 1
  • table 4
Is this relevant?
1983
1983
STUDIES of haptoglobin, 1-3 group-specific component,3,4 and numerous other products of hepatic synthesis59 have shown that liver… (More)
  • figure I
  • figure 2
Is this relevant?