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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

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Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune...
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Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

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Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
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Inflammatory Bowel Disease II: Crohn's Disease01:30

Inflammatory Bowel Disease II: Crohn's Disease

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Introduction
Inflammatory bowel disease, commonly known as IBD, refers to a collection of disorders that lead to persistent inflammation of the gastrointestinal tract. The two types of IBD are ulcerative colitis, which impacts the colon, and Crohn's disease, which can involve any part of the gastrointestinal segment.
Crohn's disease
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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF01:24

Drugs for Treatment of Crohn's Disease in IBD Using Biologic Agents: Anti-TNF

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Tumor Necrosis Factor (TNF), a proinflammatory cytokine, contributes significantly to the inflammation seen in Crohn's disease. It exists as soluble TNF and membrane-bound TNF, with actions mediated through TNF receptors (TNFR). TNFR activation leads to the release of proinflammatory cytokines, T-cell activation, collagen production, and leukocyte migration, all contributing to inflammation in Crohn's disease. Anti-TNF monoclonal antibodies, namely infliximab (Remicade), adalimumab...
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Inflammatory Bowel Disease I: Ulcerative Colitis01:27

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Related Experiment Video

Updated: Sep 1, 2025

Single-cell Analysis of Immunophenotype and Cytokine Production in Peripheral Whole Blood via Mass Cytometry
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Systemic autoinflammatory diseases in pediatric population.

Maria Luís Marques1, Inês Machado Cunha2, Sérgio Alves3

  • 1Allergy and Clinical Immunology Department, Hospital da Senhora da Oliveira, Guimarães,Portugal.

Asia Pacific Allergy
|August 15, 2022
PubMed
Summary
This summary is machine-generated.

Systemic autoinflammatory diseases (SAID) are inherited immune disorders. This study characterized pediatric SAID patients, finding clinical features similar to other studies, emphasizing early recognition for better quality of life.

Keywords:
Behçet diseaseChildClinical symptomsGenetic testingSystemic Juvenile idiopathic arthritisTreatment

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Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Systemic autoinflammatory diseases (SAID) are inherited disorders affecting innate immune system regulation.
  • These conditions can be monogenic or polygenic.

Purpose of the Study:

  • To characterize the clinical and genetic features of pediatric patients diagnosed with SAID.
  • To raise awareness among the medical community for earlier diagnosis and treatment of SAID.

Main Methods:

  • Retrospective chart review of pediatric patients with SAID.
  • Data collected from 1998 to 2020.

Main Results:

  • Fifty-four pediatric patients were evaluated.
  • Common diagnoses included periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA), Behçet disease, and systemic juvenile idiopathic arthritis (sJIA).
  • Clinical features observed were consistent with findings in other international studies.

Conclusions:

  • The clinical presentation of pediatric SAID patients in this cohort aligns with existing literature.
  • Early recognition and treatment of SAID are crucial for improving the quality of life in affected children.