Sickle cell crisis: acute chest syndrome: few patients died.

Clinical bottom line (level 4)

  1. 6% of adults with sickle cell disease who developed acute chest syndrome died.
  2. 2% of children with sickle cell disease who developed acute chest syndrome died.
Vichinsky et al: Blood 1997; 89 (5): 1787-1792
Expires October 2003

The study

Prospective cohort study with unblinded, unobjective outcomes, not adjusted for confounding factors, not validated in an independent set of patients.

Setting: 23 hospitals, USA

939 patients (aged 73% < 20 years old, ?% male) with sickle cell anaemia (diagnosed electrophoretically) and 1722 episodes of acute chest syndrome (diagnosed as a new pulmonary infiltrate on chest X-ray or a perfusion defect on lung radioisotope scan).


?100% followed for 15 days
Outcomes studied:
  • death in children
  • death in adults

    • The evidence

    outcome time to outcome number of patients/total number %
    (95% CI)    		 NNF
    (95% CI)
    death in children 15 days 14/695 2.0%
    (0.97% to 3.1%)    		 50
    (33 to 100)
    death in adults 15 days 18/271 6.6%
    (3.7% to 9.6%)    		 15
    (10 to 27)

    • Patients spent a mean of 6.8 days in hospital.
    • Adults stayed longer in hospital if breathless, had a productive cough or haemoptysis.

    Citation

    1. Vichinsky EP, Styles LA, Colangelo LH: Acute chest syndrome in sickle cell disease: clinical presentation and course. Blood 1997; 89 (5): 1787-1792
    Contributor: Chris Ball and Clare Wotton, October 1999
    Reviewer: Mona Nabulsi

    Clinical Question.
    Patient sickle cell disease
    Intervention or Exposure acute chest syndrome
    Outcome death, clinical features