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Background: Deferred central venous catheter (CVC) replacement places children with intestinal failure (IF) at risk of complications. We hypothesized that early CVC replacement after uncomplicated candidemia is safe and beneficial., Methods: We performed a retrospective review of children with IF. Patients were divided into early (<7 days after their first negative culture), and late (≥7 days after their first negative culture) CVC replacement following uncomplicated candidemia. We calculated the median time to CVC removal, clearance of infection, CVC replacement or exchange, and duration of the initial hospitalization. The proportion of patients readmitted within 30 days was also calculated, taking note of the number of candida reinfections., Results: Early replacement occurred in 18 encounters and late replacement in 21 encounters. The median time in both groups to CVC removal was 3 days ( P = 0.949), and clearance of infection was 4 days ( P = 0.466). The median time to CVC replacement or exchange in the early group was 4 days, compared to 10 days in the late group ( P < 0.001). The median duration of the hospitalization in the early group was 12 days compared to 21 days in the late group ( P = 0.011). In total 39% of patients from the early group were readmitted within 30 days compared to 57% from the late group ( P = 0.359). None of the patients were reinfected with candida within 30 days., Conclusion: Early CVC replacement after uncomplicated candidemia in children with IF decreases hospital stay without increased risk of readmission or reinfection., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.)

Background: Autoantibodies against type I IFNs occur in approximately 10% of adults with life-threatening coronavirus disease 2019 (COVID-19). The frequency of anti-IFN autoantibodies in children with severe sequelae of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is unknown., Objective: We quantified anti-type I IFN autoantibodies in a multicenter cohort of children with severe COVID-19, multisystem inflammatory syndrome in children (MIS-C), and mild SARS-CoV-2 infections., Methods: Circulating anti-IFN-α2 antibodies were measured by a radioligand binding assay. Whole-exome sequencing, RNA sequencing, and functional studies of peripheral blood mononuclear cells were used to study any patients with levels of anti-IFN-α2 autoantibodies exceeding the assay's positive control., Results: Among 168 patients with severe COVID-19, 199 with MIS-C, and 45 with mild SARS-CoV-2 infections, only 1 had high levels of anti-IFN-α2 antibodies. Anti-IFN-α2 autoantibodies were not detected in patients treated with intravenous immunoglobulin before sample collection. Whole-exome sequencing identified a missense variant in the ankyrin domain of NFKB2, encoding the p100 subunit of nuclear factor kappa-light-chain enhancer of activated B cells, aka NF-κB, essential for noncanonical NF-κB signaling. The patient's peripheral blood mononuclear cells exhibited impaired cleavage of p100 characteristic of NFKB2 haploinsufficiency, an inborn error of immunity with a high prevalence of autoimmunity., Conclusions: High levels of anti-IFN-α2 autoantibodies in children and adolescents with MIS-C, severe COVID-19, and mild SARS-CoV-2 infections are rare but can occur in patients with inborn errors of immunity., (Copyright © 2023. Published by Elsevier Inc.)