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Acute chest syndrome

MedGen UID:
196643
•Concept ID:
C0742343
•
Disease or Syndrome
Synonyms: Acute Chest Syndrome; Acute Chest Syndromes; Syndrome, Acute Chest; Syndromes, Acute Chest
SNOMED CT: Acute chest syndrome (372146004); Acute chest syndrome in sickle cell disease (372146004)
 
Monarch Initiative: MONDO:0005632

Definition

A vaso-occlusive crisis of the pulmonary vasculature occurring in patients with sickle cell disease. It is characterized by the presence of a new radiodensity on a chest radiograph accompanied by fever, cough, sputum production, dyspnea, or hypoxia. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute chest syndrome

Professional guidelines

PubMed

American Society of Hematology 2021 guidelines for sickle cell disease: stem cell transplantation.
Kanter J, Liem RI, Bernaudin F, Bolaños-Meade J, Fitzhugh CD, Hankins JS, Murad MH, Panepinto JA, Rondelli D, Shenoy S, Wagner J, Walters MC, Woolford T, Meerpohl JJ, Tisdale J
Blood Adv 2021 Sep 28;5(18):3668-3689. doi: 10.1182/bloodadvances.2021004394C. PMID: 34581773Free PMC Article
Plastic Bronchitis in Adult and Pediatric Patients: A Review of its Presentation, Diagnosis, and Treatment.
Patel N, Patel M, Inja R, Krvavac A, Lechner AJ
Mo Med 2021 Jul-Aug;118(4):363-373. PMID: 34373673Free PMC Article
Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J
JAMA 2014 Sep 10;312(10):1033-48. doi: 10.1001/jama.2014.10517. PMID: 25203083

Recent clinical studies

Etiology

Hydroxyurea (hydroxycarbamide) for sickle cell disease.
Rankine-Mullings AE, Nevitt SJ
Cochrane Database Syst Rev 2022 Sep 1;9(9):CD002202. doi: 10.1002/14651858.CD002202.pub3. PMID: 36047926Free PMC Article
Posterior Reversible Encephalopathy Syndrome.
Gewirtz AN, Gao V, Parauda SC, Robbins MS
Curr Pain Headache Rep 2021 Feb 25;25(3):19. doi: 10.1007/s11916-020-00932-1. PMID: 33630183Free PMC Article
Acute chest syndrome in sickle cell disease.
Farooq S, Abu Omar M, Salzman GA
Hosp Pract (1995) 2018 Aug;46(3):144-151. Epub 2018 Apr 23 doi: 10.1080/21548331.2018.1464363. PMID: 29648482
Sickle cell disease.
Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, Smith WR, Panepinto JA, Weatherall DJ, Costa FF, Vichinsky EP
Nat Rev Dis Primers 2018 Mar 15;4:18010. doi: 10.1038/nrdp.2018.10. PMID: 29542687
Crises in Sickle Cell Disease.
Novelli EM, Gladwin MT
Chest 2016 Apr;149(4):1082-93. Epub 2015 Dec 28 doi: 10.1016/j.chest.2015.12.016. PMID: 26836899Free PMC Article

Diagnosis

Sickle Cell Disease: A Review.
Kavanagh PL, Fasipe TA, Wun T
JAMA 2022 Jul 5;328(1):57-68. doi: 10.1001/jama.2022.10233. PMID: 35788790
Plastic Bronchitis in Adult and Pediatric Patients: A Review of its Presentation, Diagnosis, and Treatment.
Patel N, Patel M, Inja R, Krvavac A, Lechner AJ
Mo Med 2021 Jul-Aug;118(4):363-373. PMID: 34373673Free PMC Article
Posterior Reversible Encephalopathy Syndrome.
Gewirtz AN, Gao V, Parauda SC, Robbins MS
Curr Pain Headache Rep 2021 Feb 25;25(3):19. doi: 10.1007/s11916-020-00932-1. PMID: 33630183Free PMC Article
Sickle cell disease.
Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, Smith WR, Panepinto JA, Weatherall DJ, Costa FF, Vichinsky EP
Nat Rev Dis Primers 2018 Mar 15;4:18010. doi: 10.1038/nrdp.2018.10. PMID: 29542687
Sickle-cell disease.
Rees DC, Williams TN, Gladwin MT
Lancet 2010 Dec 11;376(9757):2018-31. Epub 2010 Dec 3 doi: 10.1016/S0140-6736(10)61029-X. PMID: 21131035

Therapy

Sickle Cell Disease: A Review.
Kavanagh PL, Fasipe TA, Wun T
JAMA 2022 Jul 5;328(1):57-68. doi: 10.1001/jama.2022.10233. PMID: 35788790
Red cell transfusion and alloimmunization in sickle cell disease.
Linder GE, Chou ST
Haematologica 2021 Jul 1;106(7):1805-1815. doi: 10.3324/haematol.2020.270546. PMID: 33792218Free PMC Article
Crises in Sickle Cell Disease.
Novelli EM, Gladwin MT
Chest 2016 Apr;149(4):1082-93. Epub 2015 Dec 28 doi: 10.1016/j.chest.2015.12.016. PMID: 26836899Free PMC Article
Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J
JAMA 2014 Sep 10;312(10):1033-48. doi: 10.1001/jama.2014.10517. PMID: 25203083
Sickle-cell disease.
Rees DC, Williams TN, Gladwin MT
Lancet 2010 Dec 11;376(9757):2018-31. Epub 2010 Dec 3 doi: 10.1016/S0140-6736(10)61029-X. PMID: 21131035

Prognosis

Acute chest syndrome, airway inflammation and lung function in sickle cell disease.
De A, Williams S, Yao Y, Jin Z, Brittenham GM, Kattan M, Lovinsky-Desir S, Lee MT
PLoS One 2023;18(3):e0283349. Epub 2023 Mar 30 doi: 10.1371/journal.pone.0283349. PMID: 36996064Free PMC Article
Acute pain episodes, acute chest syndrome, and pulmonary thromboembolism in pregnancy.
Asare EV, DeBaun MR, Olayemi E, Boafor T, Oppong SA
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):388-407. doi: 10.1182/hematology.2022000376. PMID: 36485167Free PMC Article
Posterior Reversible Encephalopathy Syndrome.
Gewirtz AN, Gao V, Parauda SC, Robbins MS
Curr Pain Headache Rep 2021 Feb 25;25(3):19. doi: 10.1007/s11916-020-00932-1. PMID: 33630183Free PMC Article
Sickle-cell disease.
Rees DC, Williams TN, Gladwin MT
Lancet 2010 Dec 11;376(9757):2018-31. Epub 2010 Dec 3 doi: 10.1016/S0140-6736(10)61029-X. PMID: 21131035
Mortality in sickle cell disease. Life expectancy and risk factors for early death.
Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP
N Engl J Med 1994 Jun 9;330(23):1639-44. doi: 10.1056/NEJM199406093302303. PMID: 7993409

Clinical prediction guides

Acute chest syndrome, airway inflammation and lung function in sickle cell disease.
De A, Williams S, Yao Y, Jin Z, Brittenham GM, Kattan M, Lovinsky-Desir S, Lee MT
PLoS One 2023;18(3):e0283349. Epub 2023 Mar 30 doi: 10.1371/journal.pone.0283349. PMID: 36996064Free PMC Article
Hydroxyurea (hydroxycarbamide) for sickle cell disease.
Rankine-Mullings AE, Nevitt SJ
Cochrane Database Syst Rev 2022 Sep 1;9(9):CD002202. doi: 10.1002/14651858.CD002202.pub3. PMID: 36047926Free PMC Article
Sickle Cell Disease: A Review.
Kavanagh PL, Fasipe TA, Wun T
JAMA 2022 Jul 5;328(1):57-68. doi: 10.1001/jama.2022.10233. PMID: 35788790
Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, Jordan L, Lanzkron SM, Lottenberg R, Savage WJ, Tanabe PJ, Ware RE, Murad MH, Goldsmith JC, Ortiz E, Fulwood R, Horton A, John-Sowah J
JAMA 2014 Sep 10;312(10):1033-48. doi: 10.1001/jama.2014.10517. PMID: 25203083
Mortality in sickle cell disease. Life expectancy and risk factors for early death.
Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP
N Engl J Med 1994 Jun 9;330(23):1639-44. doi: 10.1056/NEJM199406093302303. PMID: 7993409

Recent systematic reviews

Acute pain episodes, acute chest syndrome, and pulmonary thromboembolism in pregnancy.
Asare EV, DeBaun MR, Olayemi E, Boafor T, Oppong SA
Hematology Am Soc Hematol Educ Program 2022 Dec 9;2022(1):388-407. doi: 10.1182/hematology.2022000376. PMID: 36485167Free PMC Article
Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.
Knight-Madden JM, Hambleton IR
Cochrane Database Syst Rev 2022 Dec 2;12(12):CD003733. doi: 10.1002/14651858.CD003733.pub5. PMID: 36458811Free PMC Article
Hydroxyurea (hydroxycarbamide) for sickle cell disease.
Rankine-Mullings AE, Nevitt SJ
Cochrane Database Syst Rev 2022 Sep 1;9(9):CD002202. doi: 10.1002/14651858.CD002202.pub3. PMID: 36047926Free PMC Article
Antibiotics for treating acute chest syndrome in people with sickle cell disease.
Martí-Carvajal AJ, Conterno LO, Knight-Madden JM
Cochrane Database Syst Rev 2015 Mar 6;2015(3):CD006110. doi: 10.1002/14651858.CD006110.pub4. PMID: 25749695Free PMC Article
Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.
Knight-Madden JM, Hambleton IR
Cochrane Database Syst Rev 2014 Aug 2;(8):CD003733. doi: 10.1002/14651858.CD003733.pub3. PMID: 25086371

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