acute sickle cell chest syndrome; chest crisis; pulmonary sickle crisis
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Etiology
- infection in patients with sickle cell disease:
Epidemiology
- occurs in up to 35% of hospitalized patients with hemoglobin SS disease
- most common cause of death in sickle cell patients
Clinical manifestations
Laboratory
Diagnostic procedures
- pulmonary function testing: restrictive pattern
Radiology
- chest X-ray:
- bilateral lower lobe consolidations are common
- diffuse pulmonary infiltrates
Complications
- associated with pulmonary hypertension, death
- pulmonary artery thrombosis (HR=1.17)[3]
Differential diagnosis
Management
- empiric broad-spectrum antibiotics
- intravenous cephalosporin & an oral macrolide[5]
- supplemental oxygen
- analgesia to diminish chest splinting
- avoidance of overhydration
- bronchodilators as needed
- erythrocyte transfusion for mild presentations of persistent hypoxia despite supplemental oxygen[2]
- erythrocyte exchange transfusion for rapid progression or persistent hypoxia despite supplemental oxygen[2]
More general terms
Additional terms
References
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 790-91
- ↑ 2.0 2.1 2.2 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 19. American College of Physicians, Philadelphia 2009, 2012, 2022
- ↑ 3.0 3.1 Mekontso Dessap A et al. Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med 2011 Nov 1; 184:1022. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21836136
- ↑ Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008 Nov 20;359(21):2254-65. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19020327
- ↑ 5.0 5.1 Green D Managing Sickle Cell Disease: An Update NEJM Journal Watch. Sept 16, 2014 Massachusetts Medical Society (subscription needed) http://www.jwatch.org
Yawn BP et al. Management of sickle cell disease: Summary of the 2014 evidence-based report by expert panel members. JAMA 2014 Sep 10; 312:1033 PMID: https://www.ncbi.nlm.nih.gov/pubmed/2520308 - ↑ Vichinsky EP et al. Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group. N Engl J Med 2000 Jun 22; 342:1855 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/10861320 <Internet> http://www.nejm.org/doi/full/10.1056/NEJM200006223422502
- ↑ Melton CW, Haynes J Jr Sickle acute lung injury: role of prevention and early aggressive intervention strategies on outcome. Clin Chest Med. 2006 Sep;27(3):487-502, vii. PMID: https://www.ncbi.nlm.nih.gov/pubmed/16880058
- ↑ Jain S, Bakshi N, Krishnamurti L. Acute chest syndrome in children with sickle cell disease. Pediatr Allergy Immunol Pulmonol 2017 Dec 1; 30:191 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29279787 PMCID: PMC5733742 Free PMC article