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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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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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Development of Immunocompetence01:22

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The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
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The hosts' susceptibility to infection depends on several factors. The integrity of the skin and mucous membranes helps protect the body against microbial attacks. When the skin is altered, the chance of infection, limb loss, and even death increases.
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Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
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Immune Response Against Viral Pathogens01:29

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The immune system's response to viral infections is a complex and coordinated process involving natural killer (NK) cells, T cell-mediated responses, and antibody-mediated responses.
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Keeping up with recent developments in immunodeficiency.

Michael W Tsoulis1, Kelli W Williams2

  • 1Section of Allergy and Clinical Immunology, Dartmouth-Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Hanover, New Hampshire.

Annals of Allergy, Asthma & Immunology : Official Publication of the American College of Allergy, Asthma, & Immunology
|December 24, 2024
PubMed
Summary
This summary is machine-generated.

Inborn errors of immunity (IEIs) are genetic immune system disorders. Recent advancements have identified over 485 IEIs, aiding diagnosis and treatment strategies.

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Inborn errors of immunity (IEIs) represent a growing category of monogenetic immune system disorders.
  • Recent breakthroughs in genetic testing and functional studies have significantly accelerated the discovery of IEI genes and disease mechanisms.
  • The International Union of Immunological Societies (IUIS) has updated IEI classifications, with the latest in 2022, now encompassing over 485 disorders across 10 main groups.

Purpose of the Study:

  • To review recent clinical advancements in the field of IEIs.
  • To provide an in-depth look at newly identified IEI disorders.
  • To offer guidance on the diagnosis, management, and treatment of IEIs.

Main Methods:

  • Literature review of recent clinical developments in IEIs.
  • Analysis of the 2022 IUIS IEI classification.
  • Highlighting specific IEI disorders with phenotypes including severe atopy, immune dysregulation, lymphoproliferation, autoinflammation, and innate immune defects.

Main Results:

  • The number of described IEIs has surpassed 485, categorized into 10 main groups.
  • Recent discoveries have expanded the understanding of IEI genetics and disease mechanisms.
  • Specific IEI phenotypes such as severe atopy, immune dysregulation, and autoinflammation are increasingly recognized.

Conclusions:

  • Clinicians can utilize an updated diagnostic approach for suspected IEIs.
  • Management and treatment strategies for IEIs are evolving with new discoveries.
  • Continued research and classification updates are crucial for addressing the expanding landscape of IEIs.