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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

919
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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Complementation Tests00:49

Complementation Tests

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A complementation test is a simple cross to identify whether the two mutations are located on the same gene or different genes. It was first performed by Edward Lewis in the 1940s while working on fruit flies. He developed the test to identify the location and arrangement of different mutations on chromosomes.
Organisms heterozygous for different mutations are crossed pairwise in all combinations. If present on different genes, the mutations can complement each other by providing the missing...
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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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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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Factors Affecting the Risk of Infection01:26

Factors Affecting the Risk of Infection

11.5K
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.
The integrity and count of the white blood cells help the body resist pathogens and fight infection. When impaired, it reduces the body's resistance to pathogens. The acidic pH levels of the gastrointestinal, genitourinary tracts, and skin...
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Updated: Jun 12, 2025

Depletion of Specific Cell Populations by Complement Depletion
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Depletion of Specific Cell Populations by Complement Depletion

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Immunodeficiency: Complement disorders.

Jeremy C McMurray1, Brandon J Schornack1, Andrew L Weskamp1

  • 1From the Allergy and Immunology Service, Walter Reed National Military Medical Center, Bethesda, Maryland; and.

Allergy and Asthma Proceedings
|September 19, 2024
PubMed
Summary
This summary is machine-generated.

Complement system defects impact immunity, increasing infection and autoimmune risks. Diagnosis involves functional assays and immunoassay, with management focusing on vaccination and surveillance.

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Area of Science:

  • Immunology
  • Innate and adaptive immunity
  • Complement system biology

Background:

  • The complement system comprises three pathways: classic, alternative, and lectin, crucial for immune responses.
  • Deficiencies or overactivation of its 50+ proteins can lead to infections, autoimmunity, angioedema, or thrombosis.
  • Specific pathway defects correlate with distinct clinical manifestations and associated diseases.

Purpose of the Study:

  • To outline the clinical significance of complement system deficiencies.
  • To detail the spectrum of diseases associated with defects in each complement pathway.
  • To describe diagnostic approaches and management strategies for complement deficiencies.

Main Methods:

  • Review of complement system pathways and associated protein functions.
  • Correlation of complement protein deficiencies with clinical presentations.
  • Description of diagnostic assays including functional tests (CH50, AH50, ELISA) and immunoassays.
  • Outline of management principles including vaccination, antibiotics, and comorbid condition treatment.

Main Results:

  • Classic pathway defects are linked to lupus erythematosus and encapsulated organism infections.
  • Alternative pathway defects increase susceptibility to Neisseria infections.
  • Lectin pathway defects may cause pyogenic infections or autoimmunity.
  • C3 deficiency leads to severe infections and glomerulonephritis.
  • Terminal pathway defects result in disseminated Neisseria infections.

Conclusions:

  • Complement system deficiencies present a wide range of clinical outcomes, from asymptomatic to severe infections and autoimmune diseases.
  • Accurate diagnosis through functional and specific factor assays is essential.
  • Comprehensive management involves tailored strategies including prophylaxis and surveillance.