{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T21:57:52Z","timestamp":1788645472801,"version":"build-2803163510"},"reference-count":101,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2026,5,12]],"date-time":"2026-05-12T00:00:00Z","timestamp":1778544000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>Acute chest syndrome (ACS) is a common pulmonary complication in children with sickle cell disease, defined by a new pulmonary infiltrate on imaging accompanied by fever and\/or respiratory symptoms. ACS pathophysiology is multifactorial and incompletely understood, involving vaso-occlusion, pulmonary infarction, inflammation, hypoventilation, and infection\u2014the latter being a frequent trigger in children. While most pediatric cases are mild, ACS can be life-threatening and requires prompt diagnosis and management to prevent progression into respiratory failure. Mild cases are managed with pain control, IV hydration, empiric antibiotics, incentive spirometry, and supplemental oxygen when needed. More severe cases may require simple or exchange transfusion to reduce hemoglobin S levels and limit further vaso-occlusion. ACS is associated with neurologic events and long-term pulmonary complications, making prevention a clinical priority. Disease-modifying therapies include hydroxyurea and chronic transfusion. This review summarizes current evidence on the pathophysiology, risk factors, clinical presentation, diagnosis, acute management, and preventative therapies for ACS in children.<\/jats:p>","DOI":"10.3390\/children13050670","type":"journal-article","created":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T16:46:18Z","timestamp":1779381978000},"page":"670","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Acute Chest Syndrome in Children with Sickle Cell Disease: A Narrative Review"],"prefix":"10.3390","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-5004-1596","authenticated-orcid":false,"given":"Veronica","family":"Ramirez","sequence":"first","affiliation":[{"name":"Faculty of Medicine, Universit\u00e9 de Montr\u00e9al, Montreal, QC H3T 1J4, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-5002-7719","authenticated-orcid":false,"given":"Jules","family":"Mercier-Ross","sequence":"additional","affiliation":[{"name":"Centre Hospitalier de l\u2019Universit\u00e9 de Montr\u00e9al, Montreal, QC H2X 0C1, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,5,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1561","DOI":"10.1056\/NEJMra1510865","article-title":"Sickle Cell Disease","volume":"376","author":"Piel","year":"2017","journal-title":"N. Engl. J. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3276","DOI":"10.1182\/bloodadvances.2022009202","article-title":"Long-term survival with sickle cell disease: A nationwide cohort study of Medicare and Medicaid beneficiaries","volume":"7","author":"Jiao","year":"2023","journal-title":"Blood Adv."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2018","DOI":"10.1016\/S0140-6736(10)61029-X","article-title":"Sickle-cell disease","volume":"376","author":"Rees","year":"2010","journal-title":"Lancet"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1033","DOI":"10.1001\/jama.2014.10517","article-title":"Management of sickle cell disease: Summary of the 2014 evidence-based report by expert panel members","volume":"312","author":"Yawn","year":"2014","journal-title":"JAMA"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1002\/ppul.20066","article-title":"Asthma and acute chest in sickle-cell disease","volume":"38","author":"Boyd","year":"2004","journal-title":"Pediatr. Pulmonol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"1787","DOI":"10.1182\/blood.V89.5.1787","article-title":"Acute chest syndrome in sickle cell disease: Clinical presentation and course. Cooperative Study of Sickle Cell Disease","volume":"89","author":"Vichinsky","year":"1997","journal-title":"Blood"},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Serjeant, G.R., Chin, N., Asnani, M.R., Serjeant, B.E., Mason, K.P., Hambleton, I.R., and Knight-Madden, J.M. (2018). Causes of death and early life determinants of survival in homozygous sickle cell disease: The Jamaican cohort study from birth. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0192710"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1089\/ped.2017.0814","article-title":"Acute Chest Syndrome in Children with Sickle Cell Disease","volume":"30","author":"Jain","year":"2017","journal-title":"Pediatr. Allergy Immunol. Pulmonol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1097\/MPH.0000000000001012","article-title":"Recurrent Acute Chest Syndrome in Pediatric Sickle Cell Disease: Clinical Features and Risk Factors","volume":"40","author":"Patterson","year":"2018","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1164\/rccm.200710-1606OC","article-title":"Pulmonary hypertension and cor pulmonale during severe acute chest syndrome in sickle cell disease","volume":"177","author":"Leon","year":"2008","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"e26885","DOI":"10.1002\/pbc.26885","article-title":"Acute chest syndrome among children hospitalized with vaso-occlusive crisis: A nationwide study in the United States","volume":"65","author":"Takahashi","year":"2018","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1056\/NEJM200006223422502","article-title":"Causes and outcomes of the acute chest syndrome in sickle cell disease","volume":"342","author":"Vichinsky","year":"2000","journal-title":"N. Engl. J. Med."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1056\/NEJM199509143331104","article-title":"Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases","volume":"333","author":"Bellet","year":"1995","journal-title":"N. Engl. J. Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"5978","DOI":"10.1038\/s41598-023-48527-1","article-title":"Risk factors for acute chest syndrome among children with sickle cell anemia hospitalized for vaso-occlusive crises","volume":"14","author":"Alghamdi","year":"2024","journal-title":"Sci. Rep."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1097\/00043426-200301000-00010","article-title":"Chlamydia pneumoniae and acute chest syndrome in patients with sickle cell disease","volume":"25","author":"Dean","year":"2003","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3431","DOI":"10.1182\/blood-2010-05-282194","article-title":"Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease","volume":"116","author":"Strouse","year":"2010","journal-title":"Blood"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"104","DOI":"10.1016\/j.htct.2020.11.005","article-title":"Acute chest syndrome and COVID-19 in sickle cell disease pediatric patients","volume":"43","author":"Elia","year":"2021","journal-title":"Hematol. Transfus. Cell Ther."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"891","DOI":"10.1136\/archdischild-2019-317315","article-title":"Respiratory pathogens and acute chest syndrome in children with sickle cell disease","volume":"105","author":"Ploton","year":"2020","journal-title":"Arch. Dis. Child."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/S0022-3476(05)81839-6","article-title":"Role of Chlamydia pneumoniae in acute chest syndrome of sickle cell disease","volume":"118","author":"Miller","year":"1991","journal-title":"J. Pediatr."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1002\/(SICI)1096-8652(199603)51:3<207::AID-AJH5>3.0.CO;2-0","article-title":"Sickle cell acute chest syndrome associated with parvovirus B19 infection: Case series and review","volume":"51","author":"Lowenthal","year":"1996","journal-title":"Am. J. Hematol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"5297","DOI":"10.1182\/blood-2010-11-261834","article-title":"How I treat acute chest syndrome in children with sickle cell disease","volume":"117","author":"Miller","year":"2011","journal-title":"Blood"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1136\/jcp.26.8.620","article-title":"Fat embolism in sickle cell disease","volume":"26","author":"Hutchinson","year":"1973","journal-title":"J. Clin. Pathol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1016\/S0889-8588(05)70401-9","article-title":"Systemic fat embolism and pulmonary hypertension in sickle cell disease","volume":"10","author":"Castro","year":"1996","journal-title":"Hematol. Oncol. Clin. N. Am."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1555","DOI":"10.1182\/blood.V94.5.1555","article-title":"Sickle cell acute chest syndrome: Pathogenesis and rationale for treatment","volume":"94","author":"Stuart","year":"1999","journal-title":"Blood"},{"key":"ref_25","first-page":"24","article-title":"Systemic corticosteroids in acute chest syndrome: Friend or foe?","volume":"15","author":"Ogunlesi","year":"2014","journal-title":"Paediatr. Respir. Rev."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1080\/03630260600642260","article-title":"Secretory phospholipase A2 levels in patients with sickle cell disease and acute chest syndrome","volume":"30","author":"Ballas","year":"2006","journal-title":"Hemoglobin"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1022","DOI":"10.1164\/rccm.201105-0783OC","article-title":"Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease","volume":"184","author":"Deux","year":"2011","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"695","DOI":"10.1080\/13543784.2025.2551353","article-title":"Acute chest syndrome (ACS) in sickle cell disease (SCD): Pathogenesis and pharmacotherapies in early clinical development","volume":"34","author":"Chakravorty","year":"2025","journal-title":"Expert. Opin. Investig. Drugs"},{"key":"ref_29","first-page":"100","article-title":"Exploration of Leucine-Rich Alpha-2 Glycoprotein 1 (LRG1) and Its Association with Proangiogenic Mediators in Sickle Cell Disease: A Potential Player in the Pathogenesis of the Disease","volume":"42","author":"Balyen","year":"2025","journal-title":"Turk. J. Haematol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"eadl4922","DOI":"10.1126\/scitranslmed.adl4922","article-title":"Complement is activated in patients with acute chest syndrome caused by sickle cell disease and represents a therapeutic target","volume":"17","author":"Chonat","year":"2025","journal-title":"Sci. Transl. Med."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1182\/blood.V86.2.776.bloodjournal862776","article-title":"Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease","volume":"86","author":"Gill","year":"1995","journal-title":"Blood"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"E212\u2013217","DOI":"10.1002\/ajh.23819","article-title":"Factors predicting future ACS episodes in children with sickle cell anemia","volume":"89","author":"DeBaun","year":"2014","journal-title":"Am. J. Hematol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1002\/ajh.23975","article-title":"Age-dependent pathophysiology of acute chest syndrome in children with sickle cell disease","volume":"90","author":"Renella","year":"2015","journal-title":"Am. J. Hematol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1182\/blood.V84.2.643.643","article-title":"The acute chest syndrome in sickle cell disease: Incidence and risk factors. The Cooperative Study of Sickle Cell Disease","volume":"84","author":"Castro","year":"1994","journal-title":"Blood"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1186\/1476-7961-3-2","article-title":"Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease","volume":"3","author":"Nordness","year":"2005","journal-title":"Clin. Mol. Allergy"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1007\/s11325-018-1630-x","article-title":"Comorbid obstructive sleep apnea and increased risk for sickle cell disease morbidity","volume":"22","author":"Katz","year":"2018","journal-title":"Sleep. Breath."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"781","DOI":"10.1097\/PEC.0b013e31829829f7","article-title":"Clinical factors and incidence of acute chest syndrome or pneumonia among children with sickle cell disease presenting with a fever: A 17-year review","volume":"29","author":"Chang","year":"2013","journal-title":"Pediatr. Emerg. Care"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"718","DOI":"10.1053\/jpsu.2001.22944","article-title":"Acute chest syndrome after abdominal surgery in children with sickle cell disease: Is a laparoscopic approach better?","volume":"36","author":"Wales","year":"2001","journal-title":"J. Pediatr. Surg."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1016\/j.jpedsurg.2004.02.027","article-title":"Risk factors for acute chest syndrome in children with sickle cell disease undergoing abdominal surgery","volume":"39","author":"Kokoska","year":"2004","journal-title":"J. Pediatr. Surg."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1111\/j.1553-2712.2012.01364.x","article-title":"Risk factors for increased ED utilization in a multinational cohort of children with sickle cell disease","volume":"19","author":"Glassberg","year":"2012","journal-title":"Acad. Emerg. Med."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1002\/ajh.24281","article-title":"Secondhand smoke is associated with more frequent hospitalizations in children with sickle cell disease","volume":"91","author":"Sadreameli","year":"2016","journal-title":"Am. J. Hematol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"492","DOI":"10.1111\/bjh.13348","article-title":"Guideline on the management of acute chest syndrome in sickle cell disease","volume":"169","author":"Howard","year":"2015","journal-title":"Br. J. Haematol."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/S0196-0644(99)70273-8","article-title":"Clinician assessment for acute chest syndrome in febrile patients with sickle cell disease: Is it accurate enough?","volume":"34","author":"Morris","year":"1999","journal-title":"Ann. Emerg. Med."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"E332\u2013333","DOI":"10.1002\/ajh.24408","article-title":"Are we missing the mark? Fever, respiratory symptoms, chest radiographs, and acute chest syndrome in sickle cell disease","volume":"91","author":"Lazarus","year":"2016","journal-title":"Am. J. Hematol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"801","DOI":"10.1097\/PCC.0000000000001643","article-title":"Factors Associated With Mechanical Ventilation Use in Children With Sickle Cell Disease and Acute Chest Syndrome","volume":"19","author":"Takahashi","year":"2018","journal-title":"Pediatr. Crit. Care Med."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"614","DOI":"10.1111\/j.1365-2141.2010.08272.x","article-title":"Outcome of children with sickle cell disease admitted to intensive care\u2014A single institution experience","volume":"150","author":"Bartram","year":"2010","journal-title":"Br. J. Haematol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1016\/j.blre.2013.12.002","article-title":"Bone marrow necrosis and fat embolism syndrome in sickle cell disease: Increased susceptibility of patients with non-SS genotypes and a possible association with human parvovirus B19 infection","volume":"28","author":"Tsitsikas","year":"2014","journal-title":"Blood Rev."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1185","DOI":"10.1002\/ajh.24539","article-title":"Rapidly progressive acute chest syndrome in individuals with sickle cell anemia: A distinct acute chest syndrome phenotype","volume":"91","author":"Chaturvedi","year":"2016","journal-title":"Am. J. Hematol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1386","DOI":"10.1378\/chest.117.5.1386","article-title":"Acute chest syndrome in adults with sickle cell disease","volume":"117","author":"Maitre","year":"2000","journal-title":"Chest"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"36","DOI":"10.1016\/S0140-6736(84)90193-4","article-title":"Acute chest syndrome in sickle-cell disease","volume":"1","author":"Davies","year":"1984","journal-title":"Lancet"},{"key":"ref_51","doi-asserted-by":"crossref","unstructured":"Cond\u00e9, M., Bouvier, F., Brat, R., Ferrani, S., Hankard, R., Connes, P., and Dimitrov, G. (2025). Early risk factors for acute chest syndrome in sickle cell anemia: A pediatric study. PLoS ONE, 20.","DOI":"10.1371\/journal.pone.0336567"},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1111\/j.1365-2141.2012.09105.x","article-title":"Refining the value of secretory phospholipase A2 as a predictor of acute chest syndrome in sickle cell disease: Results of a feasibility study (PROACTIVE)","volume":"157","author":"Styles","year":"2012","journal-title":"Br. J. Haematol."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1148\/radiology.187.1.8451435","article-title":"Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion","volume":"187","author":"Bhalla","year":"1993","journal-title":"Radiology"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"1322","DOI":"10.1002\/pbc.24956","article-title":"Burden of diagnostic radiation exposure in children with sickle cell disease","volume":"61","author":"Vetter","year":"2014","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"S46","DOI":"10.1016\/j.annemergmed.2020.08.012","article-title":"Utility of Point-of-Care Lung Ultrasonography for Evaluating Acute Chest Syndrome in Young Patients With Sickle Cell Disease","volume":"76","author":"Cohen","year":"2020","journal-title":"Ann. Emerg. Med."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"932","DOI":"10.1111\/acem.13002","article-title":"Accuracy of Point-of-care Lung Ultrasonography for Diagnosis of Acute Chest Syndrome in Pediatric Patients with Sickle Cell Disease and Fever","volume":"23","author":"Daswani","year":"2016","journal-title":"Acad. Emerg. Med."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1164\/rccm.202108-1930OC","article-title":"Arginine Therapy and Cardiopulmonary Hemodynamics in Hospitalized Children with Sickle Cell Anemia: A Prospective, Double-blinded, Randomized Placebo-controlled Clinical Trial","volume":"206","author":"Onalo","year":"2022","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1111\/bjh.17831","article-title":"What is the role of chest X-ray imaging in the acute management of children with sickle cell disease?","volume":"196","author":"Griffin","year":"2022","journal-title":"Br. J. Haematol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"EVIDoa2500074","DOI":"10.1056\/EVIDoa2500074","article-title":"Validation and Application of a Predictive Score of Acute Chest Syndrome","volume":"5","author":"Kassasseya","year":"2026","journal-title":"NEJM Evid."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"447","DOI":"10.1164\/ajrccm.159.2.9806108","article-title":"Accuracy of pulse oximetry in sickle cell disease","volume":"159","author":"Ortiz","year":"1999","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"240005","DOI":"10.1183\/16000617.0005-2024","article-title":"Respiratory management of acute chest syndrome in children with sickle cell disease","volume":"33","author":"Ahmed","year":"2024","journal-title":"Eur. Respir. Rev."},{"key":"ref_62","first-page":"Cd006110","article-title":"Antibiotics for treating acute chest syndrome in people with sickle cell disease","volume":"9","author":"Conterno","year":"2019","journal-title":"Cochrane Database Syst. Rev."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.1001\/jamapediatrics.2017.2526","article-title":"Association of Guideline-Adherent Antibiotic Treatment With Readmission of Children With Sickle Cell Disease Hospitalized With Acute Chest Syndrome","volume":"171","author":"Bundy","year":"2017","journal-title":"JAMA Pediatr."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1016\/S0022-3476(95)70025-0","article-title":"Effect of transfusion in acute chest syndrome of sickle cell disease","volume":"127","author":"Emre","year":"1995","journal-title":"J. Pediatr."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1182\/bloodadvances.2019001143","article-title":"American Society of Hematology 2020 guidelines for sickle cell disease: Transfusion support","volume":"4","author":"Chou","year":"2020","journal-title":"Blood Adv."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"1630","DOI":"10.1177\/0009922818796661","article-title":"Bronchodilator Use for Acute Chest Syndrome Among Large Pediatric Hospitals in North America","volume":"57","author":"Kopel","year":"2018","journal-title":"Clin. Pediatr."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"3082","DOI":"10.1182\/blood.V92.9.3082","article-title":"Beneficial effect of intravenous dexamethasone in children with mild to moderately severe acute chest syndrome complicating sickle cell disease","volume":"92","author":"Bernini","year":"1998","journal-title":"Blood"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"1006","DOI":"10.1002\/pbc.21336","article-title":"Corticosteroids and increased risk of readmission after acute chest syndrome in children with sickle cell disease","volume":"50","author":"Strouse","year":"2008","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1097\/MPH.0b013e3181647bb2","article-title":"A single-institution experience with treatment of severe acute chest syndrome: Lack of rebound pain with dexamethasone plus transfusion therapy","volume":"30","author":"Isakoff","year":"2008","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1916","DOI":"10.1542\/peds.2006-1241","article-title":"Primary hemorrhagic stroke in children with sickle cell disease is associated with recent transfusion and use of corticosteroids","volume":"118","author":"Strouse","year":"2006","journal-title":"Pediatrics"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1002\/ajh.21565","article-title":"Corticosteroids for acute chest syndrome in children with sickle cell disease: Variation in use and association with length of stay and readmission","volume":"85","author":"Sobota","year":"2010","journal-title":"Am. J. Hematol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1001\/jama.294.1.81","article-title":"Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease","volume":"294","author":"Morris","year":"2005","journal-title":"JAMA"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"832","DOI":"10.1164\/rccm.202409-1727OC","article-title":"Comparison of Prophylactic and Therapeutic Doses of Anticoagulation for Acute Chest Syndrome in Sickle Cell Disease: The TASC Double-Blind Controlled Randomized Clinical Trial","volume":"211","author":"Habibi","year":"2025","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_74","doi-asserted-by":"crossref","unstructured":"Betensky, M., Azzam, M., Bercovitz, R.S., Bhat, R., Biss, T.T., Branchford, B.R., Brand\u00e3o, L.R., Chan, A.K., Faustino, E.V.S., and Hamarsha, Q. (2026). ASH ISTH 2026 guidelines for Anticoagulant Prophylaxis of Pediatric Patients at Risk of Venous Thromboembolism. Blood Adv.","DOI":"10.1182\/bloodadvances.2025019415"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1182\/bloodadvances.2017012336","article-title":"Prevalence and risk factors for venous thromboembolism in children with sickle cell disease: An administrative database study","volume":"2","author":"Kumar","year":"2018","journal-title":"Blood Adv."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"1762","DOI":"10.1182\/bloodadvances.2022007802","article-title":"Venous thromboembolism prophylaxis in sickle cell disease: A multicenter cohort study of adolescent inpatients","volume":"7","author":"Davila","year":"2023","journal-title":"Blood Adv."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1186\/s13613-025-01479-3","article-title":"Guidelines for the management of emergencies and critical illness in pediatric and adult patients with sickle cell disease","volume":"15","author":"Dauger","year":"2025","journal-title":"Ann. Intensive Care"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"e2225141","DOI":"10.1001\/jamanetworkopen.2022.25141","article-title":"Incidence of Acute Chest Syndrome in Children With Sickle Cell Disease Following Implementation of the 13-Valent Pneumococcal Conjugate Vaccine in France","volume":"5","author":"Assad","year":"2022","journal-title":"JAMA Netw. Open"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1097\/MPH.0b013e31821ed4ce","article-title":"The use of incentive spirometry in pediatric patients with sickle cell disease to reduce the incidence of acute chest syndrome","volume":"33","author":"Ahmad","year":"2011","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"e29136","DOI":"10.1002\/pbc.29136","article-title":"SNAP: Supportive noninvasive ventilation for acute chest syndrome prevention in children with sickle cell disease","volume":"68","author":"Guenther","year":"2021","journal-title":"Pediatr. Blood Cancer"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1016\/S0140-6736(12)61726-7","article-title":"The Transfusion Alternatives Preoperatively in Sickle Cell Disease (TAPS) study: A randomised, controlled, multicentre clinical trial","volume":"381","author":"Howard","year":"2013","journal-title":"Lancet"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"5300","DOI":"10.1182\/blood-2009-04-146852","article-title":"How I use hydroxyurea to treat young patients with sickle cell anemia","volume":"115","author":"Ware","year":"2010","journal-title":"Blood"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1016\/S0140-6736(11)60355-3","article-title":"Hydroxycarbamide in very young children with sickle-cell anaemia: A multicentre, randomised, controlled trial (BABY HUG)","volume":"377","author":"Wang","year":"2011","journal-title":"Lancet"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"852","DOI":"10.1111\/bjh.12323","article-title":"The effect of hydroxcarbamide therapy on survival of children with sickle cell disease","volume":"161","author":"Lobo","year":"2013","journal-title":"Br. J. Haematol."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"158","DOI":"10.1097\/01.mph.0000157789.73706.53","article-title":"Chronic transfusion therapy for children with sickle cell disease and recurrent acute chest syndrome","volume":"27","author":"Hankins","year":"2005","journal-title":"J. Pediatr. Hematol. Oncol."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1067\/mpd.2001.119593","article-title":"Impact of chronic transfusion on incidence of pain and acute chest syndrome during the Stroke Prevention Trial (STOP) in sickle-cell anemia","volume":"139","author":"Miller","year":"2001","journal-title":"J. Pediatr."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1056\/NEJMoa1715971","article-title":"A Phase 3 Trial of l-Glutamine in Sickle Cell Disease","volume":"379","author":"Niihara","year":"2018","journal-title":"N. Engl. J. Med."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1177\/1535370219900637","article-title":"L-glutamine for sickle cell disease: Knight or pawn?","volume":"245","author":"Sadaf","year":"2020","journal-title":"Exp. Biol. Med."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"3668","DOI":"10.1182\/bloodadvances.2021004394C","article-title":"American Society of Hematology 2021 guidelines for sickle cell disease: Stem cell transplantation","volume":"5","author":"Kanter","year":"2021","journal-title":"Blood Adv."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"625","DOI":"10.3109\/03630269.2011.621149","article-title":"Pulmonary complications of sickle cell disease","volume":"35","author":"Khoury","year":"2011","journal-title":"Hemoglobin"},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"167.e1","DOI":"10.1016\/j.jtct.2020.10.007","article-title":"Systematic Review\/Meta-Analysis on Efficacy of Allogeneic Hematopoietic Cell Transplantation in Sickle Cell Disease: An International Effort on Behalf of the Pediatric Diseases Working Party of European Society for Blood and Marrow Transplantation and the Sickle Cell Transplantation International Consortium","volume":"27","author":"Iqbal","year":"2021","journal-title":"Transplant. Cell. Ther."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1182\/hematology.2021000322","article-title":"Evidence-Based Minireview: In young children with severe sickle cell disease, do the benefits of HLA-identical sibling donor HCT outweigh the risks?","volume":"2021","author":"Shah","year":"2021","journal-title":"Hematol. Am. Soc. Hematol. Educ. Program."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.pedhc.2024.07.007","article-title":"Gene Therapy: A New Hope in Sickle Cell Disease Treatment","volume":"39","author":"Christy","year":"2025","journal-title":"J. Pediatr. Health Care"},{"key":"ref_94","first-page":"288","article-title":"Cerebrovascular accidents in sickle cell disease: Rates and risk factors","volume":"91","author":"Weiner","year":"1998","journal-title":"Blood"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1182\/blood-2002-04-1183","article-title":"Reversible posterior leukoencephalopathy syndrome and silent cerebral infarcts are associated with severe acute chest syndrome in children with sickle cell disease","volume":"101","author":"Henderson","year":"2003","journal-title":"Blood"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.jpeds.2005.12.059","article-title":"Impact of acute chest syndrome on lung function of children with sickle cell disease","volume":"149","author":"Sylvester","year":"2006","journal-title":"J. Pediatr."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1007\/s00408-010-9255-2","article-title":"The impact of recurrent acute chest syndrome on the lung function of young adults with sickle cell disease","volume":"188","author":"Forrester","year":"2010","journal-title":"Lung"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1067\/mpd.2001.111824","article-title":"Prevalence and reversibility of lower airway obstruction in children with sickle cell disease","volume":"138","author":"Koumbourlis","year":"2001","journal-title":"J. Pediatr."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1513\/AnnalsATS.201510-706OC","article-title":"Pattern of Lung Function Is Not Associated with Prior or Future Morbidity in Children with Sickle Cell Anemia","volume":"13","author":"Cohen","year":"2016","journal-title":"Ann. Am. Thorac. Soc."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/S0022-3476(05)83418-3","article-title":"Pulmonary function abnormalities in childhood sickle cell disease","volume":"122","author":"Pianosi","year":"1993","journal-title":"J. Pediatr."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1513\/AnnalsATS.201606-452ED","article-title":"Acute Chest Syndrome, Asthma, and Lung Function in Sickle Cell Disease. Which Is the Chicken, and Which Is the Egg?","volume":"13","author":"Koumbourlis","year":"2016","journal-title":"Ann. Am. Thorac. Soc."}],"container-title":["Children"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2227-9067\/13\/5\/670\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,22]],"date-time":"2026-05-22T10:49:55Z","timestamp":1779446995000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2227-9067\/13\/5\/670"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,12]]},"references-count":101,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2026,5]]}},"alternative-id":["children13050670"],"URL":"https:\/\/doi.org\/10.3390\/children13050670","relation":{},"ISSN":["2227-9067"],"issn-type":[{"value":"2227-9067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,12]]}}}