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The pancreatic islets comprising only 1%-2% of the volume are highly vascularized and innervated mini-organs. They contain five endocrine cell types, including β cells that secrete insulin, which is synthesized as a single polypeptide chain, preproinsulin, processed to proinsulin, and finally to insulin and C-peptide. This process is complex and regulated, involving the Golgi complex, the endoplasmic reticulum, and the secretory granules of the β cell.
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Insulin secretory vesicles release insulin to stimulate blood glucose uptake and regulate carbohydrate metabolism. When the blood glucose levels increase, glucose enters the pancreatic β-islet cells through glucose transporters. Once inside, glucose is metabolized through glycolysis, the citric acid cycle, and the electron transport chain, producing ATP. This increase in ATP concentration closes ATP-sensitive potassium channels, leading to depolarization of the membrane and the opening of...
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Insulin action is mediated through a receptor tyrosine kinase, akin to the IGF-1 receptor. The number of receptors per cell varies significantly, from 40 on erythrocytes to 300,000 on adipocytes and hepatocytes. The insulin receptor consists of linked α/β subunit dimers, forming a heterotetramer glycoprotein with two extracellular α subunits and two β subunits spanning the membrane. The α subunits inhibit the inherent tyrosine kinase activity of the β subunits, but...
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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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Diabetes mellitus is a chronic metabolic disorder characterized by high blood glucose levels due to inadequate insulin production, insulin resistance, or both. The condition affects millions worldwide and can significantly impact their health and quality of life.
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Insulin is released by beta cells of the pancreas when blood glucose levels are high. It facilitates glucose absorption and utilization in insulin-dependent cells with insulin receptors on their plasma membranes. Insulin promotes glucose uptake by increasing the number of glucose transport proteins in the cell membrane, allowing glucose to enter the cell. As a result, glucose utilization and ATP production are enhanced.
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Double Trouble: When Focal and Diffuse Hyperinsulinism Occur Simultaneously.

Victoria R Sanders1,2, Katherine Lord1,2,3, Winnie Sigal1,2,3

  • 1Division of Endocrinology and Diabetes, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

Hormone Research in Paediatrics
|March 8, 2026
PubMed
Summary
This summary is machine-generated.

Congenital hyperinsulinism (HI) can present as both focal and diffuse forms in the same patient. Surgical removal of focal lesions may require diazoxide for managing residual diffuse disease.

Area of Science:

  • Endocrinology
  • Pediatric Surgery
  • Medical Genetics

Background:

  • Congenital hyperinsulinism (HI) is typically classified as diazoxide-responsive or unresponsive.
Keywords:
ABCC8Beta cellsDiffuse hyperinsulinismFocal hyperinsulinismHypoglycemiaInsulinKCNJ11Pancreas

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  • Diazoxide-unresponsive HI is further categorized into diffuse or focal forms, each with distinct management approaches.
  • While usually presenting as a single form, co-occurrence of focal and diffuse HI is possible.