{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T15:25:19Z","timestamp":1786116319675,"version":"build-2736575974"},"reference-count":20,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2026,7,15]],"date-time":"2026-07-15T00:00:00Z","timestamp":1784073600000},"content-version":"vor","delay-in-days":14,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,7,1]],"date-time":"2026-07-01T00:00:00Z","timestamp":1782864000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100000019","name":"National Hemophilia Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000019","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K23\u2010HL148548"],"award-info":[{"award-number":["K23\u2010HL148548"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Acute chest syndrome (ACS) is a leading cause of morbidity in pediatric sickle cell disease (SCD). The Clinical Respiratory Score (CRS) is used prospectively at the bedside for ACS, whereas the NHLBI Clinical Severity Index (CSI) classifies ACS severity retrospectively. We evaluated the diagnostic performance of peak CRS to identify severe ACS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a retrospective cohort study of patients with ACS (one admission each) aged 3\u201321 years at a tertiary children's hospital (2015\u20132021). Peak CRS was abstracted from bedside documentation; CSI (1\u2009=\u2009mild, 2\u2009=\u2009moderate, 3\u2009=\u2009severe) was assigned from the chart. The primary analysis estimated sensitivity, specificity, positive\/negative predictive values (PPV\/NPV), and accuracy of peak CRS\u2009\u2265\u20094 for identifying CSI\u2009=\u20093, with exact 95% confidence intervals. We prespecified a CRS 0\u20131 subgroup to assess NPV and de\u2010escalation outcomes [respiratory support, transfusion, length of stay (LOS)].<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Among 213 patients, peak CRS\u2009\u2265\u20094 predicted CSI\u2009=\u20093 with sensitivity 1.00 (95% CI 0.863\u20131.000), specificity 0.888 (0.835\u20130.926), PPV 0.543 (0.402\u20130.678), NPV 1.000 (0.978\u20131.000), and accuracy 0.901 (0.854\u20130.935). No severe events occurred with CRS\u2009&lt;\u20094 (95% upper bound ~1.8%). In CRS 0\u20131 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200974), none had severe ACS (95% upper bound ~4.1%) nor required significant respiratory support; 75.7% were not transfused; and median LOS was 2.3 days (IQR 1.4\u20133.1).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Peak CRS\u2009\u2265\u20094 identifies a high\u2010risk cohort for severe ACS; CRS 0\u20131 identifies a low\u2010risk cohort requiring minimal intervention. CRS offers a bidirectional bedside tool to guide escalation and de\u2010escalation of care; prospective validation is warranted.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ppul.71736","type":"journal-article","created":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T06:30:17Z","timestamp":1784183417000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Clinical Respiratory Score: Diagnostic Performance for Acute Chest Syndrome in Sickle Cell Disease"],"prefix":"10.1002","volume":"61","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3402-9624","authenticated-orcid":false,"given":"Shaniqua J.","family":"Anum","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Hematology\u2010Oncology University of Alabama at Birmingham Birmingham Alabama USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andy C.","family":"Yu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1330-6618","authenticated-orcid":false,"given":"Jonathan M.","family":"Flanagan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine Houston Texas USA"},{"name":"Texas Children's Cancer and Hematology Center Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3721-614X","authenticated-orcid":false,"given":"Danielle","family":"Guffey","sequence":"additional","affiliation":[{"name":"Institute for Clinical and Translational Research Baylor College of Medicine Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0388-7693","authenticated-orcid":false,"given":"Nicholas A.","family":"Ettinger","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Critical Care, Emory University School of Medicine Children's Healthcare of Atlanta Atlanta Georgia USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9266-9304","authenticated-orcid":false,"given":"Saleh","family":"Bhar","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine Houston Texas USA"},{"name":"Texas Children's Cancer and Hematology Center Houston Texas USA"},{"name":"Division of Pediatric Critical Care Texas Children's Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-0868-252X","authenticated-orcid":false,"given":"Julie","family":"Katkin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine Houston Texas USA"},{"name":"Division of Pediatric Pulmonary Medicine Texas Children's Hospital Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6832-2389","authenticated-orcid":false,"given":"Shruti","family":"Chaturvedi","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Medicine Johns Hopkins Hospital Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gladstone","family":"Airewele","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine Houston Texas USA"},{"name":"Texas Children's Cancer and Hematology Center Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5973-8507","authenticated-orcid":false,"given":"Ven\u00e9e N.","family":"Tubman","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Baylor College of Medicine Houston Texas USA"},{"name":"Texas Children's Cancer and Hematology Center Houston Texas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,7,15]]},"reference":[{"key":"e_1_2_9_2_1","unstructured":"\u201cData & Statistics on Sickle Cell Disease | CDC.\u201d U.S. Department of Health & Human Services Preprint posted online2019 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