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Proteoglycans01:05

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Glycans, a class of complex heterogeneous molecules, can be covalently attached to proteins to form glycosylated proteins that regulate various physiological and pathological processes. Glycosylated proteins or glycoproteins comprise N-linked and O-linked oligosaccharides. O-glycosylation is the most common type of protein glycosylation. Here, glycans attach to the oxygen atom of the hydroxyl groups of Serine or Threonine residues. O-linked glycosylation occurs later in protein processing,...
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Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
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Carbohydrates consumed through foods are converted into glucose, a crucial energy source for the body. In the prandial state, high blood glucose levels stimulate the secretion of insulin from the pancreas. Insulin inhibits hepatic glucose production and stimulates glucose uptake and metabolism by muscle and adipose tissue. The excess glucose is converted into glycogen and stored in the liver and muscles.
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Carbohydrate metabolism is a fundamental biochemical process that ensures a constant supply of energy to living cells. The most important carbohydrate is glucose, which can be broken down via glycolysis to enter into the Krebs cycle and eventually lead to the production of ATP through oxidative phosphorylation.
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Diabetes mellitus is a chronic metabolic disorder characterized by hyperglycemia. The four categories of diabetes are type 1 diabetes, type 2 diabetes, other specific types of diabetes, and gestational diabetes.
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Glucose transporters facilitate the transport of glucose across the cell membrane. In addition to glucose, some glucose transporters can also aid the movement of other hexoses such as fructose, mannose, and galactose.
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Related Experiment Video

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The Extraction of Liver Glycogen Molecules for Glycogen Structure Determination
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Glycogenic Hepatopathy: Thinking Outside the Box.

Nishant Parmar1, Muslim Atiq2, Lee Austin2

  • 1Department of Internal Medicine, Sanford USD School of Medicine, Sioux Falls, S. Dak., USA.

Case Reports in Gastroenterology
|August 14, 2015
PubMed
Summary

Glycogenic hepatopathy (GH) is often misdiagnosed as non-alcoholic fatty liver disease in adults. This case highlights that even minor improvements in glycemic control can significantly alleviate GH symptoms.

Keywords:
Celiac diseaseDual-echo magnetic resonance imagingElevated liver enzymesGlycogenic hepatopathyHepatomegalyNon-alcoholic fatty liver disease

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Area of Science:

  • Hepatology
  • Endocrinology
  • Internal Medicine

Background:

  • Glycogenic hepatopathy (GH) is underrecognized in adults, frequently misdiagnosed as non-alcoholic fatty liver disease due to similar presentations and indistinguishable ultrasound findings.
  • Clinical presentation can include abdominal pain, hepatomegaly, lactic acidosis, and marked elevation of liver enzymes, potentially exacerbated by steroid administration.

Observation:

  • A young adult female presented with abdominal pain, hepatomegaly, lactic acidosis, and significantly elevated liver enzymes.
  • Her condition worsened after high-dose steroid administration, and transaminitis persisted despite fluid resuscitation.

Findings:

  • Liver biopsy confirmed a diagnosis of glycogenic hepatopathy.
  • Biochemical drivers include high circulating glucose and insulin or high-dose steroid administration.
  • Symptomatic relief was achieved with a modest 0.6% improvement in glycated hemoglobin, suggesting other factors contribute to GH.

Implications:

  • Accurate diagnosis of GH is crucial to avoid mismanagement, particularly differentiating it from NAFLD.
  • While glycemic control is key, the role of other contributing factors in GH warrants further investigation.
  • This case underscores the importance of considering GH in adults presenting with unexplained liver enzyme elevations, especially those with metabolic derangements or steroid exposure.