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Related Concept Videos

Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum sickness, a systemic...
Hypersensitivities01:30

Hypersensitivities

Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
Hypersensitivity Reactions: Delayed Hypersensitivity Reactions01:29

Hypersensitivity Reactions: Delayed Hypersensitivity Reactions

Delayed-Type Hypersensitivity (DTH), or Type IV hypersensitivity, is a cell-mediated immune response. It occurs when T cells, rather than antibodies, mediate a reaction to specific antigens. It is characterized by a delayed onset (1-2 days) and involves the recruitment of macrophages to the inflammation site.The initiation of a DTH response begins with the sensitization of T cells. During this phase, which lasts at least 1-2 weeks, antigen-specific T cells are activated, clonally expanded, and...
Antiasthma Drugs: Mast Cell Stabilizers and Anti-IgE Drugs01:25

Antiasthma Drugs: Mast Cell Stabilizers and Anti-IgE Drugs

Asthma is a chronic respiratory condition for which new therapeutic avenues, including anti-inflammatory drugs like mast cell stabilizers and anti-IgE treatments, continue to be developed.
Mast cell stabilizers, such as cromolyn (also known as sodium cromoglycate) and nedocromil (Tilade), are effective drugs in asthma management. These stabilizers hinder histamine release by skillfully obstructing the activation of mast cells and other cellular entities. Notably, they navigate this task without...
Irritable Bowel Syndrome01:23

Irritable Bowel Syndrome

DefinitionIrritable bowel syndrome (IBS) is a functional gastrointestinal disorder characterized by recurrent combinations of abdominal pain, bloating, diarrhea, or constipation.Pathophysiology of irritable bowel syndromeIts pathophysiology is multifactorial, involving disturbances in motility, sensory processing, microbial balance, barrier integrity, and gut–brain communication. These mechanisms interact to produce symptoms that vary across IBS subtypes.Altered Motility PatternsDisordered...
Gastritis II: Pathophysiology01:26

Gastritis II: Pathophysiology

The pathophysiology of gastritis begins with the colonization of the stomach lining by Helicobacter pylori (H. pylori). This bacterium spreads mainly via the oral-oral route through saliva or shared utensils, and can also be transmitted in overcrowded or unhygienic environments through contaminated water, despite its brief survival outside the body.ColonizationOnce ingested, H. pylori enters the stomach and begins colonization by navigating through the mucus layer lining the stomach wall. It...

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Related Experiment Video

Updated: May 25, 2026

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
10:27

Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

Hyper-IgE syndrome update.

Kathryn J Sowerwine1, Steven M Holland, Alexandra F Freeman

  • 1Laboratory of Clinical Infectious Diseases, NIAID, NIH, Bethesda, Maryland, USA.

Annals of the New York Academy of Sciences
|January 25, 2012
PubMed
Summary
This summary is machine-generated.

Autosomal dominant hyper-IgE syndrome (AD-HIES), a primary immunodeficiency, presents diverse symptoms due to STAT3 mutations. Recent research expands the known clinical features and deepens understanding of its disease mechanisms.

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job's syndrome, is a primary immunodeficiency.
  • It is characterized by a broad spectrum of clinical manifestations.
  • The condition arises from dominant-negative mutations in the STAT3 gene.

Purpose of the Study:

  • To update the understanding of AD-HIES.
  • To highlight the expanded clinical phenotype, including newly recognized features.
  • To detail advancements in understanding the disease's pathogenesis.

Main Methods:

  • Review of recent clinical and genetic studies on AD-HIES.
  • Analysis of case reports and literature concerning STAT3 mutations.
  • Comparative analysis of established and newly identified clinical features.

Main Results:

  • The clinical spectrum of AD-HIES is broader than previously recognized.
  • Arterial aneurysms are a newly identified feature of the syndrome.
  • Significant progress has been made in elucidating the molecular pathogenesis of AD-HIES.

Conclusions:

  • AD-HIES is a complex primary immunodeficiency with evolving clinical and pathogenetic understanding.
  • STAT3 mutations are central to AD-HIES, leading to diverse systemic effects.
  • Continued research is crucial for comprehensive diagnosis and management of AD-HIES.