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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

3.1K
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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Humoral Immune Responses01:36

Humoral Immune Responses

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Overview
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Primary Lymphoid Organs01:16

Primary Lymphoid Organs

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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...
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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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Pleiotropy01:33

Pleiotropy

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Pleiotropy is the phenomenon in which a single gene impacts multiple, seemingly unrelated phenotypic traits. For example, defects in the SOX10 gene cause Waardenburg Syndrome Type 4, or WS4, which can cause defects in pigmentation, hearing impairments, and an absence of intestinal contractions necessary for elimination. This diversity of phenotypes results from the expression pattern of SOX10 in early embryonic and fetal development. SOX10 is found in neural crest cells that form melanocytes,...
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Intellectual Disability01:29

Intellectual Disability

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Intellectual disability (ID) is a neurodevelopmental condition characterized by deficits in intellectual and adaptive functioning that manifest during the developmental period. This condition encompasses challenges in reasoning, memory, problem-solving, and learning, accompanied by impairments in everyday life skills, such as communication, self-care, and social interactions. Intellectual disability affects approximately 1% of the population in the United States, impacting an estimated 5...
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Updated: Apr 1, 2026

Characterization of Thymus-dependent and Thymus-independent Immunoglobulin Isotype Responses in Mice Using Enzyme-linked Immunosorbent Assay
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The 2015 IUIS Phenotypic Classification for Primary Immunodeficiencies.

Aziz Bousfiha1, Leïla Jeddane2, Waleed Al-Herz3,4

  • 1Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco. profbousfiha@gmail.com.

Journal of Clinical Immunology
|October 9, 2015
PubMed
Summary
This summary is machine-generated.

Nearly 300 genetic immune disorders cause diverse conditions like infections and autoimmunity. This study presents the revised 2015 International Union of Immunological Societies classification for Primary Immunodeficiency Diseases (PID).

Keywords:
IUIS PID expert committeePrimary immunodeficienciesclassification

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Over 300 single-gene inborn errors of immunity are known, leading to varied phenotypes including infection, malignancy, allergy, autoimmunity, and auto-inflammation.
  • Primary Immunodeficiency Diseases (PID) represent a rapidly expanding field, with increasing numbers of phenotypes linked to these genetic disorders.

Purpose of the Study:

  • To present the revised figures based on the 2015 International Union of Immunological Societies (IUIS) expert committee (EC) classification of Primary Immunodeficiency Diseases (PID).
  • To provide an updated classification of PIDs, building upon previous IUIS classifications.

Main Methods:

  • The study is based on the accompanying 2015 IUIS PID EC classification.
  • Utilizes a classification system defined by shared pathogenesis and/or clinical consequences.
  • Incorporates a user-friendly, phenotypic classification approach based on key clinical phenotypes.

Main Results:

  • Presents revised figures reflecting the 2015 IUIS PID EC classification.
  • Details a classification of nearly 300 single-gene inborn errors of immunity.
  • Organizes disorders based on pathogenesis and clinical consequences.

Conclusions:

  • The 2015 IUIS PID EC classification offers an updated framework for understanding and categorizing Primary Immunodeficiency Diseases.
  • This revised classification aids in the diagnosis and management of a wide spectrum of immune system disorders.