Related Experiment Video
Updated: Sep 10, 2025
An In vitro Model to Study Immune Responses of Human Peripheral Blood Mononuclear Cells to Human Respiratory Syncytial Virus Infection
Published on: December 10, 2013
Pediatric and Adult Inborn Errors of Immunity and COVID-19: A Comparative Study
Cansu Özdemiral1, Saliha Esenboga1, Ayşegul Akarsu1
1Department of Pediatrics, Division of Immunology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
Patients with inborn errors of immunity (IEI) face higher COVID-19 hospitalization and mortality risks, especially adults. Early interventions like immunoglobulin therapy and convalescent plasma may improve outcomes in these vulnerable populations.
Area of Science:
- Immunology
- Infectious Diseases
- Genetics
Background:
- Inborn errors of immunity (IEI) are associated with severe clinical outcomes in COVID-19 patients.
- Understanding the specific risks of hospitalization, ICU admission, and mortality in IEI patients is crucial for clinical management.
Purpose of the Study:
- To determine the risk factors for COVID-19-related hospitalization, ICU admission, and mortality in patients with IEI.
- To analyze clinical characteristics and outcomes of COVID-19 in a cohort of IEI patients.
Main Methods:
- Retrospective analysis of 62 COVID-19 patients (29 children, 33 adults) with diagnosed IEI.
- Data collection included demographics, IEI type, clinical presentation, laboratory markers (D-dimer, ferritin), comorbidities, and treatment received.
- Statistical analysis to identify risk factors for hospitalization and ICU admission.
Main Results:
- Hospitalization rates were higher in adults (21/33) than children (11/29).
- Cough, elevated D-dimer, and ferritin levels were associated with hospitalization.
- Immunoglobulin replacement therapy and cough were significant predictors of hospitalization risk.
- Higher ferritin levels correlated with ICU admission.
- Mortality was 6.5% overall, significantly higher than the general population, with no deaths in children.
- Agammaglobulinemia showed a high ICU admission rate, highlighting the role of mucosal IgA in COVID-19 defense.
Conclusions:
- IEI patients, particularly adults, exhibit increased risk for severe COVID-19 outcomes.
- Elevated D-dimer and ferritin levels are indicators of severe disease and hospitalization risk.
- Convalescent plasma therapy may shorten hospitalization duration in specific IEI types like agammaglobulinemia.
Abstract:
COVID-19 has worse clinical outcomes in inborn errors of immunity (IEI) patients. We aimed to determine COVID-19-related hospitalization/ICU admission/mortality risk in patients with IEI. We included 62 COVID-19 (29 children and 33 adults) in a referral center. F/M ratio was 0.94 with median age, 19 (8 months-64 years) years. 53.2% had primary antibody deficiency. Hospitalization rate was 11/29 in children, 21/33 in adults and 7/11 in patients with combined immunodeficiency diseases. Myalgia was more common in adults compared to children (p = 0.013). Inpatients have more cough compared to outpatients (p = 0.002). D-dimer and ferritin levels were higher in inpatients (p = 0.033 and p = 0.046, respectively). Cough (OR: 6.05; [95% CI: 1.76-20.74], p = 0.004) and immunoglobulin replacement therapy use in IEI (OR: 5.15; [95% CI: 1.46-18.11], p = 0.010) were related to hospitalization risk. Inpatients with intensive care unit (ICU) admission had higher ferritin levels (p = 0.02). 77.4% had at least one comorbidity like pulmonary (45.2%), autoimmune (38.7%), and gastrointestinal diseases (32.3%). ICU admission was high in agammaglobulinemia (40%) and immune dyseregulation (ID) (16.6%). An LRBA deficiency patient experienced MIS-C (Multisystem Inflammatory Syndrome in Children) and another died. Eight patients, four in the present center, received convalescent plasma (X-linked agammaglobulinemia [XLA] [n = 3], autosomal recessive agammaglobulinemia [n = 1], LRBA deficiency [n = 1], and CTLA4 deficiency [n = 1], CVID [n = 1], and STAT1 deficiency [n = 1]). Overall mortality was 6.5%, high in ID (16.6%), none in children. Higher D-dimer and ferritin levels is associated with a higher hospitalization ratio- twice in adults compared to children. Overall mortality (6.5%) was about six times the general population with no mortality in children. A high ICU ratio in agammaglobulinemia, suggesting the importance of mucosal IgA in COVID-19 defense. Convalescent plasma helps shorten hospitalization period in agammaglobulinemia.
More Related Videos
Related Concept Videos
01:26Factors Affecting the Risk of Infection
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...
01:22Development of Immunocompetence
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...
01:31Active versus Passive Immunity
Active Immunity
Active immunity refers to the resistance one develops...
01:25Immunodeficiency Diseases
There are three main causes of immunodeficiency...
01:21Introduction to Innate and Adaptive Immunity
Innate immunity is the body's natural, nonspecific defense system that acts quickly to protect against pathogens. It incorporates physical barriers like skin and mucous membranes and cellular elements such as phagocytes and natural killer cells. This part of our immune system provides an immediate,...
01:29Immune Response Against Viral Pathogens
NK Cells
NK cells are a crucial part of our innate immune system, acting as the first line of defense against viral infections. These cells can recognize and kill infected cells without prior exposure to the virus, effectively slowing down the spread of infection. Additionally, NK cells produce proinflammatory...