Background: Inborn errors of immunity (IEIs) are considered significant challenges for children with IEIs, their families, and their medical providers. Infections are the most common complication of IEIs and children can acquire coronavirus disease 2019 (COVID-19) even when protective measures are taken. Objectives: To estimate the incidence of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in children with IEIs and analyse the demographic parameters, clinical characteristics and treatment outcomes in children with IEIs with COVID-19 illness. Methods: For this systematic review, we searched ProQuest, Medline, Embase, PubMed, CINAHL, Wiley online library, Scopus and Nature through the Preferred Reporting Items for Systematic Reviews and Meta Analyses (PRISMA) guideline for studies on the development of COVID-19 in children with IEIs, published from December 1, 2019 to February 28, 2023, with English language restriction. Results: Of the 1095 papers that were identified, 116 articles were included in the systematic review (73 case report, 38 cohort 4 case-series and 1 case–control studies). Studies involving 710 children with IEIs with confirmed COVID-19 were analyzed. Among all 710 IEIs pediatric cases who acquired SARS-CoV-2, some children were documented to be admitted to the intensive care unit (ICU) (n = 119, 16.8%), intubated and placed on mechanical ventilation (n = 87, 12.2%), suffered acute respiratory distress syndrome (n = 98, 13.8%) or died (n = 60, 8.4%). Overall, COVID-19 in children with different IEIs patents resulted in no or low severity of disease in more than 76% of all included cases (COVID-19 severity: asymptomatic = 105, mild = 351, or moderate = 88). The majority of children with IEIs received treatment for COVID-19 (n = 579, 81.5%). Multisystem inflammatory syndrome in children (MIS-C) due to COVID-19 in children with IEIs occurred in 103 (14.5%). Fatality in children with IEIs with COVID-19 was reported in any of the included IEIs categories for cellular and humoral immunodeficiencies (n = 19, 18.6%), immune dysregulatory diseases (n = 17, 17.9%), innate immunodeficiencies (n = 5, 10%), bone marrow failure (n = 1, 14.3%), complement deficiencies (n = 1, 9.1%), combined immunodeficiencies with associated or syndromic features (n = 7, 5.5%), phagocytic diseases (n = 3, 5.5%), autoinflammatory diseases (n = 2, 3%) and predominantly antibody deficiencies (n = 5, 2.5%). Mortality was COVID-19-related in a considerable number of children with IEIs (29/60, 48.3%). The highest ICU admission and fatality rates were observed in cases belonging to cellular and humoral immunodeficiencies (26.5% and 18.6%) and immune dysregulatory diseases (35.8% and 17.9%) groups, especially in children infected with SARS-CoV-2 who suffered severe combined immunodeficiency (28.6% and 23.8%), combined immunodeficiency (25% and 15%), familial hemophagocytic lymphohistiocytosis (40% and 20%), X-linked lymphoproliferative diseases-1 (75% and 75%) and X-linked lymphoproliferative diseases-2 (50% and 50%) compared to the other IEIs cases. Conclusion: Children with IEIs infected with SARS-CoV-2 may experience higher rates of ICU admission and mortality in comparison with the immunocompetent pediatric populations. Underlying immune defects does seem to be independent risk factors for severe SARS-CoV-2 infection in children with IEIs, a number of children with SCID and CID were reported to have prolonged infections–though the number of patients is small–but especially immune dysregulation diseases (XLP1 and XLP2) and innate immunodeficiencies impairing type I interferon signalling (IFNAR1, IFNAR2 and TBK1).

Introduction: Acute otitis media (AOM) is a common early childhood infection, often emerging as a complication of upper respiratory tract infections. With an estimated 60%–70% of children experiencing AOM during childhood, it presents a significant health concern, especially in the paediatric population. The prevalence of AOM underscores its substantial burden on healthcare systems. However, there is a noticeable gap in research regarding the risk factors and complications of AOM, specifically in the child population of Saudi Arabia. This study aims to determine the prevalence of AOM among Saudi Arabian children, identify associated risk factors and investigate complications arising from AOM. Methods: This cross-sectional study, conducted from July to October 2023, targeted parents or guardians of Saudi Arabian children aged 0–12 years diagnosed with AOM. Non-Saudi children and those with chronic otitis media were excluded. Data were collected through a structured questionnaire in both English and Arabic, focusing on demographics, risk factors, day-care attendance and breastfeeding duration. The sample size was calculated using Raosoft software, requiring a minimum of 377 participants. Statistical analysis was performed using IBM SPSS Statistics version 28.0. Results: The study involved 952 participants. Key findings included a high prevalence of AOM symptoms in the past year, with 33.8% of children diagnosed by healthcare professionals. A significant correlation was found between AOM prevalence and day-care attendance (P < 0.001), with children attending day care for <10 h per week showing a higher incidence of AOM. The study also found a significant relationship between extended breastfeeding duration and decreased AOM prevalence and episodes. However, a substantial number of parents reported a lack of information regarding the AOM prevention benefits of breastfeeding. Conclusion: The study reveals that AOM is a significant health concern in Saudi Arabian children, with day-care attendance and breastfeeding practices showing varying associations with AOM incidence. These findings contribute to our understanding of AOM in this demographic and highlight the need for increased awareness and preventive measures, especially in day-care settings. They also underscore the potential role of breastfeeding in reducing AOM episodes and emphasise the importance of healthcare provider guidance in this context. Future research should focus on longitudinal studies to establish causal relationships and explore the long-term impacts of AOM.