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Introduction
Type I Iterferon Defects in COVID-19
Infectious Complications of New Inborn Errors of Immunity
New Diagnostics for Inborn Errors of Immunity
SEVERE BACTERIAL INFECTIONS IN PREVIOUSLY HEALTHY CHILDREN AS A FIRST MANIFESTATION OF PRIMARY IMMUNODEFICIENCY: A 7-YEAR SINGLE-CENTER RETROSPECTIVE ANALYSIS OF ROUTINE IMMUNOLOGICAL TESTING
Abstract
Backgrounds:
Severe bacterial infections (SBI) in otherwise healthy children are rare, and they might represent a first clinical manifestation of primary immunodeficiency (PID). However, it is unclear which children should be assessed at all and how. We aimed to support clinical decision-making by characterizing the frequency and features of PID in otherwise healthy children after an episode of SBI.
Methods
We retrospectively analyzed epidemiological, microbiological and immunological data from hospital records at the University Children’s Hospital Zurich, Switzerland. We included children who were previously healthy, aged 3d-18y at SBI diagnosis, and were diagnosed and/or immunologically followed-up between 01/01/2013 and 31/03/2020. Children with proven or clinically diagnosed SBI with pleuropneumonia, meningitis, and/or sepsis who were routinely followed up and screened for PID were included.
Results:
We identified 432 children with SBI, and after excluding those without general consent, a final SBI cohort of 360 children remained (median age 3.4 years). Outpatient follow-up data were available for 265 (74%) children. Immunological testing was performed in 244 children (92%), whereas 21 patients (8%) were assessed clinically only. Laboratory abnormalities were detected in 51 of 244 patients (21%), revealing 38 definite PID cases (16%). Selective deficiencies in IgA and/or IgM (n=24) and hypogammaglobulinemia (IgG deficiency, n=8) were the most common, but PID diagnoses also included two cases of specific antibody deficiency and one case each of C2 deficiency, C7 deficiency, properdin deficiency and autoimmune neutropenia.
Conclusions/Learning Points:
Routine testing for PID after an episode of SBI in children revealed abnormal immunological laboratory tests in 21% of children. Although the clinical significance of some of these findings remains unclear, their identification allows optimal counseling of families and optimization of preventive measures such as additional vaccinations to avoid future SBI episodes.