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Immunodeficiency Diseases

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...

Humoral Immune Responses

Humoral Immune Responses

Development of Immunocompetence

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...

Transcytosis of IgG

Transcytosis of IgG

Transcytosis is the process in which molecules are internalized by endocytosis, transported across the cell, and released through exocytosis from the opposite end of the cell. Molecules such as insulin, immunoglobulins, and certain nutrients are transferred through the recycling endosomes by recycling and transcytosis.
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...

Antibody Structure

Antibody Structure

Overview
Antibodies, also known as immunoglobulins (Ig), are essential players of the adaptive immune system. These antigen-binding proteins are produced by B cells and make up 20 percent of the total blood plasma by weight. In mammals, antibodies fall into five different classes, which each elicits a different biological response upon antigen binding.
The Y-Shaped Structure of Antibodies Consists of Four Polypeptide Chains
Antibodies consist of four polypeptide chains: two identical heavy...

Special Features of Adaptive Immunity

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,...

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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

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Primary Immunodeficiency: Specific antibody deficiency with normal IgG.

Vishaka R Hatcher, Veronica C Alix, Tasha S Hellu

Allergy and Asthma Proceedings

|September 19, 2024

Summary

Specific antibody deficiency (SAD) is a common immune disorder in children and adults with recurrent infections. Diagnosis involves poor response to polysaccharide vaccines, with management tailored to clinical severity.

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Separation of Immune Cell Subpopulations in Peripheral Blood Samples from Children with Infectious Mononucleosis

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Published on: September 7, 2022

Area of Science:

Immunology
Clinical Medicine

Background:

Specific antibody deficiency (SAD) is a frequent primary immunodeficiency.
It presents as recurrent or severe sinopulmonary infections in older children and adults.
Diagnosis relies on inadequate antibody response to polysaccharide vaccines despite normal protein antigen responses.

Purpose of the Study:

To outline the diagnostic criteria and management strategies for Specific Antibody Deficiency.
To highlight the importance of age-appropriate immune maturity for diagnosis.
To emphasize the need for standardized diagnostic approaches and ongoing patient monitoring.

Main Methods:

Diagnosis is based on serotype testing before and after polysaccharide vaccination (e.g., pneumococcal).
Assessment includes evaluating antibody response to polysaccharide antigens versus protein antigens.
Serum immunoglobulin levels and IgG subclasses are typically normal.

Main Results:

Patients show inadequate antibody response specifically to polysaccharide antigens.
Immune maturity, particularly polysaccharide responsiveness, develops with age, with diagnosis typically established after age 2.
Management strategies include prophylactic antibiotics and immunoglobulin replacement therapy based on clinical severity.

Conclusions:

SAD diagnosis requires careful consideration of age-specific immune responses.
Management is individualized, with potential for resolution in children and progression to more severe immunodeficiency in some patients.
Close follow-up and monitoring are crucial for patients with SAD, with possible discontinuation of immunoglobulin replacement.