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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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The Extraction of Liver Glycogen Molecules for Glycogen Structure Determination
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Glycogenic Hepatopathy.

Bashar Sharma1, Marsha Antoine1, Mili Shah1

  • 1Department of Internal Medicine, State University of New York Upstate Medical University, Syracuse, NY.

ACG Case Reports Journal
|October 18, 2019
PubMed
Summary
This summary is machine-generated.

Glycogenic hepatopathy, a rare complication of type 1 diabetes, involves excessive liver glycogen. This case highlights its occurrence in a young woman with poorly controlled diabetes and diabetic ketoacidosis.

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Area of Science:

  • Hepatology
  • Endocrinology
  • Metabolic Disorders

Background:

  • Glycogenic hepatopathy is a rare condition characterized by excessive intrahepatic glycogen accumulation.
  • It is typically associated with long-standing, poorly controlled type 1 diabetes mellitus.
  • Diabetic ketoacidosis is a serious complication of diabetes requiring prompt management.

Observation:

  • A 19-year-old woman with a history of poorly controlled type 1 diabetes and recurrent diabetic ketoacidosis presented with abdominal pain, nausea, vomiting, and hepatomegaly.
  • Initial investigations revealed diabetic ketoacidosis with persistent hyperlactatemia unresponsive to insulin therapy.
  • These clinical signs suggested a potential complication beyond typical diabetic ketoacidosis management.

Findings:

  • Liver biopsy confirmed excessive intracytoplasmic glycogen accumulation, consistent with a diagnosis of glycogenic hepatopathy.
  • The patient's presentation and biopsy results established a direct link between her diabetes control and liver condition.
  • Elevated serum lactate levels, despite insulin treatment, were a key indicator of this metabolic derangement.

Implications:

  • This case underscores the importance of vigilant glycemic control in type 1 diabetes to prevent rare but serious complications like glycogenic hepatopathy.
  • Early recognition and diagnosis of glycogenic hepatopathy are crucial for appropriate management and preventing further liver damage.
  • Understanding the pathophysiology of glycogenic hepatopathy can inform future research into metabolic complications of diabetes.