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acute sickle cell chest syndrome; chest crisis; pulmonary sickle crisis

Etiology: - infection in patients with sickle cell disease: a) Chlamydia b) Mycoplasma c) respiratory syncytial virus d) Staphylococcus aureus (coagulase positive) e) Streptococcus pneumoniae f) parvovirus g) rhinovirus Epidemiology: 1) occurs in up to 35% of hospitalized patients with hemoglobin SS disease 2) most common cause of death in sickle cell patients Clinical manifestations: 1) chest pain 2) fever 3) prostration Laboratory: - hypoxemia PaO2 < 50 mm Hg in 40% of patients Special laboratory: - pulmonary function testing: restrictive pattern Radiology: - chest X-ray: a) bilateral lower lobe consolidations are common b) diffuse pulmonary infiltrates Differential diagnosis: 1) pulmonary embolism - bone marrow fat embolism 2) pneumonia Complications: - associated with pulmonary hypertension, death - pulmonary artery thrombosis (HR=1.17) [3] 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]

Related

sickle cell (hemoglobin SS) disease

General

syndrome lung disease complication

References

  1. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 790-91
  2. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 19. American College of Physicians, Philadelphia 2009, 2012, 2022
  3. 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: 21836136
  4. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008 Nov 20;359(21):2254-65. PMID: 19020327
  5. 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: 2520308
  6. 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 PMID: 10861320 http://www.nejm.org/doi/full/10.1056/NEJM200006223422502
  7. 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: 16880058
  8. 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: 29279787 PMCID: PMC5733742 Free PMC article