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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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Cytomegalovirus Disease01:27

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Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
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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.
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Special Features of Adaptive Immunity01:20

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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.
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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
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Common Variable Immunodeficiency.

Biman Saikia1, Sudhir Gupta2

  • 1Department of Immunopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India. bimansaikia@gmail.com.

Indian Journal of Pediatrics
|February 13, 2016
PubMed
Summary

Common variable immunodeficiency (CVID) is a primary immunodeficiency affecting all ages, often under-diagnosed in South Asia. Early diagnosis and immunoglobulin therapy are crucial for managing infections, autoimmune diseases, and malignancies associated with CVID.

Keywords:
AutoimmunityClinical manifestationsCommon variable immunodeficiencyImmunological changes

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency, impacting children, adolescents, and adults.
  • CVID is significantly under-diagnosed in India and Southeast Asian countries.
  • While often sporadic, CVID can be inherited in autosomal recessive or dominant patterns.

Purpose of the Study:

  • To highlight the under-diagnosis of CVID in India and Southeast Asia.
  • To emphasize the diverse clinical manifestations of CVID, including infections, autoimmune diseases, and malignancies.
  • To underscore the importance of early diagnosis and awareness among clinicians.

Main Methods:

  • Diagnosis relies on reduced immunoglobulin levels (IgG with IgA and/or IgM) and impaired antibody responses.
  • Flow cytometry reveals disturbed B cell homeostasis (reduced memory B cells, increased CD21(low) and transitional B cells).
  • Genetic mutations are identified in less than 20% of CVID cases; T cell defects are observed in about one-third of patients.

Main Results:

  • Patients experience recurrent sino-pulmonary infections, autoimmune conditions, and cancers like lymphoma and leukemia.
  • Characteristic findings include interstitial lung disease and nodular lymphoid hyperplasia of the gut.
  • Despite diagnostic criteria, many patients remain undiagnosed, delaying treatment.

Conclusions:

  • Increased awareness of CVID among immunologists and clinicians in India and Southeast Asia is essential for timely diagnosis.
  • Immunoglobulin therapy is the primary treatment for CVID.
  • Early and accurate diagnosis can improve patient outcomes by managing associated complications.