ABSTRACT
Acute chest syndrome (ACS) is a leading cause of morbimortality in sickle cell disease (SCD). In this prospective observational study, we investigated sputum interleukin-6 (IL-6) level as an ACS severity marker during 30 ACS episodes in 26 SCD children. Sputum IL-6 levels measured within the first 72 h of hospitalisation for ACS were significantly higher in patients with oxygen requirement ≥2 L/min, ventilation (invasive and/or non-invasive) length ≥5 days, bilateral and/or extensive opacities on chest X-ray or erythrocytapheresis requirement. Sputum IL-6 could serve as an ACS severity marker to help identify patients requiring targeted anti-inflammatory treatments such as tocilizumab.
Subject(s)
Acute Chest Syndrome , Anemia, Sickle Cell , Biomarkers , Interleukin-6 , Severity of Illness Index , Sputum , Humans , Anemia, Sickle Cell/complications , Acute Chest Syndrome/etiology , Child , Interleukin-6/analysis , Interleukin-6/blood , Male , Female , Adolescent , Sputum/metabolism , Prospective Studies , Child, PreschoolABSTRACT
A 13-year-old boy was admitted with severe meningococcal meningitis. Immunologic workup revealed a properdin deficiency, and genetic sequencing of CFP identified a novel, private and predicted pathogenic variant in exon 8. The patient received broad immunizations and penicillin prophylaxis. Children with invasive meningococcal disease should be tested for complement deficiency.
