This retrospective cohort study evaluated the diagnostic performance of the peak Clinical Respiratory Score (CRS) — a bedside tool — for identifying severe acute chest syndrome (ACS; NHLBI Clinical Severity Index [CSI] = 3) in 213 children and adolescents aged 3–21 years with sickle cell disease (SCD) admitted to a tertiary children's hospital between 2015 and 2021.
Peak CRS ≥ 4 detected severe ACS with **sensitivity 1.00** (95% CI 0.863–1.000), **specificity 0.888** (0.835–0.926), PPV 0.543, and **NPV 1.000** (0.978–1.000); no severe events occurred below this threshold (95% upper bound ~1.8%). In the lowest-risk subgroup (CRS 0–1; n = 74), zero patients had severe ACS, none needed high-flow O₂/NIPPV/intubation, 75.7% required no transfusion, and median LOS was 2.3 days (IQR 1.4–3.1).
- **Single-center, retrospective design** with chart-assigned CSI; prospective multicenter validation is lacking. - **Management/incorporation bias**: CRS-guided interventions (oxygen, NIPPV, transfusion) can both modify CSI category and be prompted by the score itself, inflating NPV and potentially lowering PPV. - **CRS not routinely captured in the ICU**, so the most severely ill patients may be underrepresented.
Use peak CRS ≥ 4 as a sensitive bedside flag to trigger escalation consideration (critical care consult, NIPPV, transfusion review) in pediatric SCD-ACS. Children with CRS 0–1 appear safe for streamlined monitoring or early discharge, but prospective validation before formal protocol adoption is warranted.
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