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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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 disorders...
Development of Immunocompetence01:22

Development of Immunocompetence

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...
Primary Lymphoid Organs01:16

Primary Lymphoid Organs

Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

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,...
Antigens Involved in Adaptive Immunity01:26

Antigens Involved in Adaptive Immunity

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 reactivity.
Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

Overview

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Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR
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Simultaneous Quantification of T-Cell Receptor Excision Circles (TRECs) and K-Deleting Recombination Excision Circles (KRECs) by Real-time PCR

Published on: December 6, 2014

Revisiting human primary immunodeficiencies.

Jean-Laurent Casanova1, Claire Fieschi, Shen-Ying Zhang

  • 1Laboratory of Human Genetics of Infectious Diseases, Institut National de la Santé et de la Recherche Médicale, Paris, France. casanova@necker.fr

Journal of Internal Medicine
|June 12, 2008
PubMed
Summary
This summary is machine-generated.

Primary immunodeficiencies (PIDs) are more diverse than previously thought. These complex immune disorders can be common, sporadic, and present in adults with varied symptoms beyond infections.

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Primary immunodeficiencies (PIDs) are traditionally viewed as rare, inherited disorders affecting immune cells and causing severe infections in infants.
  • This narrow definition overlooks a significant and growing number of PID cases.

Purpose of the Study:

  • To broaden the understanding of primary immunodeficiencies.
  • To highlight the expanding spectrum of PID presentations and underlying genetic causes.

Main Methods:

  • Review of current literature and clinical case studies.
  • Analysis of emerging patterns in PID diagnosis and presentation.

Main Results:

  • PIDs encompass a wider range of conditions than previously recognized, including common and sporadic forms.
  • PID pathogenesis can involve non-hematopoietic cells and present as dominant or polygenic traits.
  • Adults can present with single or multiple morbid phenotypes, not exclusively infections.

Conclusions:

  • The clinical and genetic spectrum of human PIDs is significantly broader than conventionally accepted.
  • Increased medical awareness is crucial to recognize diverse PID presentations in various clinical settings.
  • PIDs should be considered in a wider array of patient presentations, including adults with non-infectious conditions.