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Related Concept Videos

Autoimmune Disorders01:29

Autoimmune Disorders

313
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
Crohn's disease is a chronic, systemic inflammatory bowel disease (IBD) that predominantly affects the gastrointestinal tract. It is marked by...
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What is the Immune System?01:38

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Drugs Used in Lower Respiratory Disorders: Overview01:17

Drugs Used in Lower Respiratory Disorders: Overview

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Lower respiratory tract disorders present challenges that often require skilled and nuanced approaches for effective management. Common ailments, such as asthma and chronic obstructive pulmonary disease (COPD), have prompted the development of intricate treatment strategies involving bronchodilators and anti-inflammatory drugs, each tailored to ease breathing and revitalize the lungs.
Bronchodilators, the first step of respiration enhancement, come in various forms, each with its own mechanism...
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Hypersensitivities01:30

Hypersensitivities

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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...
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Pediatric Systemic Autoinflammatory Disorders: An Overview.

Julisa M Patel1

  • 1Allergy-Immunology and Pediatric Rheumatology Division, Department of Pediatrics, Children's Hospital of Georgia, Augusta University Medical Center, 1120 15th Street, BG 102, Augusta, GA, 30912, USA. jupatel1@augusta.edu.

Current Allergy and Asthma Reports
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Summary
This summary is machine-generated.

Systemic autoinflammatory disorders (SAIDs) involve innate immunity dysregulation causing recurrent inflammation. Early diagnosis and prompt treatment of these hereditary conditions are crucial for improving patient outcomes.

Keywords:
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Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Systemic autoinflammatory disorders (SAIDs) are characterized by unprovoked inflammatory attacks due to innate immunity dysregulation.
  • The study of SAIDs has advanced significantly since the discovery of the familial Mediterranean fever (FMF) gene mutation 28 years ago.
  • Key pathways involved include inflammasomes, nuclear factor-κB, and interferon signaling.

Purpose of the Study:

  • To provide an overview of pediatric systemic autoinflammatory disorders.
  • To discuss their presentation, diagnostic workup, complications, and therapeutic management.
  • To highlight the importance of early recognition and intervention.

Main Methods:

  • Review of current literature on systemic autoinflammatory disorders.
  • Analysis of advances in genetic and biomolecular techniques.
  • Synthesis of information on pathogenesis, clinical features, and treatment.

Main Results:

  • Genetic analysis has accelerated the identification of causative mutations for many SAIDs.
  • Biomolecular advances have identified key players like inflammasomes, leading to improved treatments.
  • High clinical suspicion is essential in children with unexplained fever and inflammation.

Conclusions:

  • Timely diagnosis and prompt treatment of SAIDs significantly improve patient morbidity and mortality.
  • Understanding the pathogenesis and clinical presentation is key for effective management.
  • Advances in genetics and molecular biology offer promising therapeutic options for SAIDs.