SFGH 1/12 am report pearls: acute chest syndrome

Pearls:

Takeaway pearl: acute chest syndrome is the leading cause of death for patients with sickle cell disease and requires prompt recognition and management

  • Sickle cell disease US data: 1 out of every 500 black children and 1 out of every 36,000 hispanic children is born with SCD

Acute Chest Syndrome

  • typical presentation: some combination of fever, >2% decrease SpO2, tachypnea, increased WOB, CP, cough, wheezing, rales, and new pulmonary infiltrate on CXR
  • hb often declines sharply
  • causes: infection, bone marrow or fat embolism, atelectasis, pulmonary edema, intrapulmonary aggregates of sickled cells
  • treatment: antibiotics, O2, pain control, fluids, simple vs exchange transfusion (based on severity)
  • data is limited, but when performing exchange transfusions, expert opinion is oftentimes to target a hemoglobin S percentage <30 percent and end-hemoglobin of 10 g/dL
  • Prevention: hydroxyurea: shown in trials to reduce ACS incidence by 50%

Ballas et al. Definitions of the phenotypic manifestations of sickle cell disease.  Am J Hematol. 2010; 85 (1):6.

Melton CW, Haynes J.  Sickle acute lung injury: role of prevention and early aggressive intervention strategies on outcome.  Clin Chest Med. 2006; 27(3): 487.

National Heart, Lung, and Blood Institute. Disease and conditions index. Sickle cell anemia: who is at risk? Bethesda, MD: US Department of Health and Human Services, National Institutes of Health, National Heart, Lung, and Blood Institute; 2009. Available from:http://www.nhlbi.nih.gov/health/dci/Diseases/Sca/SCA_WhoIsAtRisk.html

Evernote link: http://www.evernote.com/l/AoOjvwSaUEND-5WN_66fRItytxRUnkI-KvU/

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