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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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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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Humoral Immune Responses01:36

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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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Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

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Author Spotlight: Advancing Immune Monitoring in Critical Care Patients Using Whole Blood Assays
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Combined immunodeficiencies.

Carolina Sanchez Aranda1, Rafaela Rola Guimarães1, Mariana de Gouveia-Pereira Pimentel1

  • 1Universidade Federal de São Paulo, Departamento de Pediatria, São Paulo, SP, Brazil.

Jornal De Pediatria
|December 19, 2020
PubMed
Summary
This summary is machine-generated.

Combined immunodeficiencies (CIDs), a type of Inborn Error of Immunity (IEI), involve T-cell defects. Early diagnosis through neonatal screening significantly improves outcomes for patients with these complex genetic disorders.

Keywords:
Gene therapyHematopoietic stem cell transplantationImmunosuppressionMolecular biologyPrimary immunodeficiencyVaccination

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Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Inborn Errors of Immunity (IEI), or primary immunodeficiencies, are congenital diseases affecting immune response.
  • Clinical features include susceptibility to infections, autoimmunity, inflammation, allergies, and malignancies.

Purpose of the Study:

  • Review literature on combined immunodeficiencies (CIDs).
  • Focus on diagnosis, treatment, and clinical management of CID patients.

Main Methods:

  • Critical integrative literature review.
  • Searched PubMed and SciELO databases for publications from the last twenty years.

Main Results:

  • CIDs characterized by T-cell development, signaling, or function defects.
  • Diverse clinical manifestations range from bacterial infections to opportunistic infections.
  • Neonatal screening aids in early disease detection.

Conclusions:

  • Combined immunodeficiencies (CIDs) are genetic disorders involving T-cells.
  • Neonatal screening improves prognosis, particularly for severe cases like SCIDs.