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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

929
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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Factors Affecting the Risk of Infection01:26

Factors Affecting the Risk of Infection

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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...
11.5K
Chronic Bowel Disorders: Introduction01:17

Chronic Bowel Disorders: Introduction

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Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
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Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

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Overview
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Inflammatory Bowel Disease I: Ulcerative Colitis01:27

Inflammatory Bowel Disease I: Ulcerative Colitis

160
Introduction
Inflammatory bowel disease, or IBD, encompasses a group of disorders characterized by chronic inflammation or ulceration of the gastrointestinal tract.
Risk Factors
The exact cause of IBD remains unclear, although it is believed to be due to a mix of genetic, environmental, microbial, and immune factors. Genetic factors are significant in determining susceptibility to IBD, with family history being a critical risk factor. Individuals with a first-degree relative who has IBD are at...
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Pulmonary Tuberculosis I01:29

Pulmonary Tuberculosis I

228
Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
Causative Organism
The primary infectious agent causing tuberculosis is Mycobacterium tuberculosis, a slow-growing, acid-fast, aerobic rod that exhibits sensitivity to heat and ultraviolet light. Instances of Mycobacterium bovis and Mycobacterium avium contributing to the development of TB infection are rare.
Mode of...
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Separation of Immune Cell Subpopulations in Peripheral Blood Samples from Children with Infectious Mononucleosis
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Noninfectious Complications in B-Lymphopenic Common Variable Immunodeficiency.

S Pashangzadeh1, S Delavari1,2, T Moeini Shad1

  • 1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.

Journal of Investigational Allergology & Clinical Immunology
|July 30, 2024
PubMed
Summary
This summary is machine-generated.

Common variable immunodeficiency (CVID) patients frequently experience noninfectious complications, especially those with B-cell lymphopenia. Early monitoring and treatment are crucial for managing CVID complications and improving patient outcomes.

Keywords:
AutoimmunityCommon variable immunodeficiencyImmune dysregulationInborn errors of immunityMalignancyPrimary immunodeficiency

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Area of Science:

  • Immunology
  • Hematology
  • Genetics

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by significant symptomatic manifestations.
  • Noninfectious complications pose a major challenge for CVID patients, alongside frequent infectious issues.

Purpose of the Study:

  • To investigate the prevalence and characteristics of noninfectious complications in CVID patients.
  • To determine the association between B-cell lymphopenia and specific noninfectious manifestations in CVID.

Main Methods:

  • Retrospective cohort study of CVID patients from a national database.
  • Patients categorized into groups based on the presence or absence of B-cell lymphopenia.
  • Evaluation of demographic data, laboratory results, organ involvement, autoimmunity, and lymphoproliferative diseases.

Main Results:

  • Noninfectious complications were diagnosed in 66.4% of 387 CVID patients.
  • Enteropathy, autoimmunity, and lymphoproliferative disorders were common, affecting 35.1%, 24.3%, and 21.4% respectively.
  • B-cell lymphopenia was associated with increased frequency of autoimmunity, specific organ involvement (dermatologic, endocrine, musculoskeletal), and certain autoimmune manifestations (rheumatologic, hematologic, gastrointestinal).

Conclusions:

  • Noninfectious complications, particularly those linked to B-cell lymphopenia, necessitate regular patient monitoring.
  • Comprehensive management including medication beyond immunoglobulin replacement therapy is recommended.
  • Proactive follow-up aims to prevent sequelae and enhance the quality of life for CVID patients.