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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.
There are three main causes of immunodeficiency...
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Development of Immunocompetence01:22

Development of Immunocompetence

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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.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
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Disorders of Leukocytes01:27

Disorders of Leukocytes

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Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune...
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Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

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The adaptive immune system, a crucial component of the overall immune response, offers a highly specialized defense against pathogens. It involves specific cell types and features, enabling it to combat infections effectively and efficiently.
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
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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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Antigens Involved in Adaptive Immunity01:26

Antigens Involved in Adaptive Immunity

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An antigen is any substance the immune system identifies as foreign and potentially harmful to the body, prompting an immune response. Antigens have two functional properties: immunogenicity and reactivity. Immunogenicity is the ability of an antigen to stimulate a specific immune response. At the same time, reactivity describes the antigen's ability to react with the cells and antibodies produced in response to it.
Complete Antigens
Complete antigens possess both immunogenicity and...
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Guideline for the Diagnosis and Management of Heritable IFNAR1 Deficiency in Oceania.

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Defining Common Variable Immunodeficiency Disorders in 2020.

Rohan Ameratunga1, Caroline Allan2, See-Tarn Woon3

  • 1Department of Virology and Immunology, Auckland City Hospital, Auckland, New Zealand; Auckland Healthcare Services, Park Road, Grafton, Auckland 1010, New Zealand; Clinical Immunology, Auckland City Hospital, Auckland, New Zealand; Department of Molecular Medicine and Pathology, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.

Immunology and Allergy Clinics of North America
|July 14, 2020
PubMed
Summary
This summary is machine-generated.

Common variable immunodeficiency disorders (CVID) are frequent in adults but lack a definitive cause or diagnostic test. Recent studies explore uncertainties, aiming to clarify the evolving understanding of these complex immune deficiencies.

Keywords:
CVIDCommon variable immunodeficiencyDiagnostic criteriaHGUSHypogammaglobulinemiaIVIGSCIGTHA

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Common variable immunodeficiency disorders (CVID) represent the most prevalent symptomatic primary immune deficiencies in adults.
  • The etiology of CVID remains largely unknown, complicating diagnosis due to the absence of a specific diagnostic marker.
  • Genetic mutations can explain some CVID-like disorders, classifying them as specific primary immunodeficiencies or inborn errors of immunity.

Purpose of the Study:

  • To review recent advancements in understanding Common Variable Immunodeficiency Disorders (CVID).
  • To address the persistent uncertainties surrounding the diagnosis and underlying causes of CVID.
  • To provide an updated perspective on the evolving field of primary immunodeficiencies.

Main Methods:

  • Literature review of recent studies on CVID.
  • Analysis of research addressing diagnostic uncertainties in primary immunodeficiencies.
  • Synthesis of current knowledge on inborn errors of immunity.

Main Results:

  • Identification of causative mutations in a subset of patients leads to classification as CVID-like disorders.
  • A significant proportion of CVID cases lack a clear genetic explanation, highlighting knowledge gaps.
  • Ongoing research continues to uncover potential genetic and molecular underpinnings of CVID.

Conclusions:

  • The understanding of CVID is dynamic, with ongoing research progressively clarifying its complexities.
  • Distinguishing idiopathic CVID from genetically defined disorders remains a clinical challenge.
  • Further investigation into the genetic and immunological basis of CVID is crucial for improved diagnosis and management.