Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

974
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...
974
Humoral Immune Responses01:36

Humoral Immune Responses

73.0K
Overview
73.0K
Primary Lymphoid Organs01:16

Primary Lymphoid Organs

1.6K
Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
1.6K
Secondary Lymphoid Organs01:15

Secondary Lymphoid Organs

1.6K
Secondary organs, including lymph nodes, the spleen, and mucosa-associated lymphoid tissue (MALT), work harmoniously to protect us from disease and infection.
The spleen is a vital organ in the lymphatic system, nestled in the upper left side of the abdomen. It is composed of two primary regions: the red pulp and the white pulp, each having distinct functions. The red pulp performs a significant role in blood filtration. It efficiently purges the blood of old or damaged red blood cells and...
1.6K
What is the Immune System?01:38

What is the Immune System?

111.8K
Overview
111.8K
Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

874
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,...
874

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Remdesivir and Nitazoxanide for Chronic Norovirus Type 1 Infection in a Child Post-cardiac Transplantation.

The Pediatric infectious disease journal·2026
Same author

Treosulfan-fludarabine conditioning in infants with severe combined immunodeficiencies: Extended study of the UK paediatric treosulfan study.

British journal of haematology·2026
Same author

Clinical presentation and management of 239 children and adolescents with hereditary angioedema.

The journal of allergy and clinical immunology. In practice·2025
Same author

Oral berotralstat for hereditary angioedema prophylaxis in patients aged 2 to <12 years: APeX-P interim results.

Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology·2025
Same author

Hybrid B- and T-Cell Immunity Associates With Protection Against Breakthrough Infection After Severe Acute Respiratory Syndrome Coronavirus 2 Vaccination in Avon Longitudinal Study of Parents and Children (ALSPAC) Participants.

The Journal of infectious diseases·2025
Same author

Effectiveness of biomarker-guided duration of antibiotic treatment in children hospitalised with confirmed or suspected bacterial infection: the BATCH RCT.

Health technology assessment (Winchester, England)·2025

Related Experiment Video

Updated: Jul 19, 2025

Detection of Polyfunctional T Cells in Children Vaccinated with Japanese Encephalitis Vaccine via the Flow Cytometry Technique
09:37

Detection of Polyfunctional T Cells in Children Vaccinated with Japanese Encephalitis Vaccine via the Flow Cytometry Technique

Published on: September 23, 2022

1.9K

The Multifaceted Syndromic Primary Immunodeficiencies in Children.

Khuen Foong Ng1, Anu Goenka1,2, Florence Manyika1

  • 1Department of Paediatric Immunology and Infectious Diseases, Bristol Royal Hospital for Children, Bristol BS1 3NU, UK.

Journal of Clinical Medicine
|August 12, 2023
PubMed
Summary

Syndromic primary immunodeficiencies (sPIDs) often involve multiple organ systems and are frequently linked to genetic disorders. Early clinical assessment for immune disorders is crucial in children with syndromic features and recurrent infections.

Keywords:
genetic syndromesinborn errors of immunitymultisystem disorderprimary immunodeficiency disordersyndromic children

More Related Videos

Simultaneous Quantification of T-Cell Receptor Excision Circles TRECs and K-Deleting Recombination Excision Circles KRECs by Real-time PCR
14:14

Simultaneous Quantification of T-Cell Receptor Excision Circles TRECs and K-Deleting Recombination Excision Circles KRECs by Real-time PCR

Published on: December 6, 2014

16.7K
A High-Throughput Multiplexed Screening for Type 1 Diabetes, Celiac Diseases, and COVID-19
06:46

A High-Throughput Multiplexed Screening for Type 1 Diabetes, Celiac Diseases, and COVID-19

Published on: July 5, 2022

2.8K

Related Experiment Videos

Last Updated: Jul 19, 2025

Detection of Polyfunctional T Cells in Children Vaccinated with Japanese Encephalitis Vaccine via the Flow Cytometry Technique
09:37

Detection of Polyfunctional T Cells in Children Vaccinated with Japanese Encephalitis Vaccine via the Flow Cytometry Technique

Published on: September 23, 2022

1.9K
Simultaneous Quantification of T-Cell Receptor Excision Circles TRECs and K-Deleting Recombination Excision Circles KRECs by Real-time PCR
14:14

Simultaneous Quantification of T-Cell Receptor Excision Circles TRECs and K-Deleting Recombination Excision Circles KRECs by Real-time PCR

Published on: December 6, 2014

16.7K
A High-Throughput Multiplexed Screening for Type 1 Diabetes, Celiac Diseases, and COVID-19
06:46

A High-Throughput Multiplexed Screening for Type 1 Diabetes, Celiac Diseases, and COVID-19

Published on: July 5, 2022

2.8K

Area of Science:

  • Pediatric Immunology
  • Rare Genetic Diseases
  • Clinical Genetics

Background:

  • Immune disorders are often overlooked in rare genetic multisystemic conditions.
  • Syndromic primary immunodeficiencies (sPIDs) represent a complex intersection of genetic and immune system dysfunction.

Purpose of the Study:

  • To characterize the syndromic features and immunological defects in children diagnosed with sPIDs.
  • To improve the recognition and diagnosis of immune deficiencies in syndromic conditions.

Main Methods:

  • Retrospective descriptive study of pediatric patients (0-18 years) with sPIDs.
  • Data collected from a specialized pediatric immunology service between January 2006 and September 2021.

Main Results:

  • 36 patients with sPIDs were identified.
  • 19% of patients had genetic diagnoses not typically associated with primary immunodeficiencies (PIDs), including Trisomy 22 and Noonan syndrome.
  • Recurrent/severe infections (92%) were common, with half exhibiting combined or antibody deficiency. Multiorgan involvement was frequent, affecting nervous, musculoskeletal, and gastrointestinal systems.

Conclusions:

  • sPIDs frequently present with multiorgan involvement, extending beyond immunological manifestations.
  • A low threshold for immune assessment is recommended for children with syndromic features, particularly those with recurrent infections or signs of immune dysregulation.