Sickle cell crisis: acute chest syndrome: fever, cough and chest pain were common.

Clinical bottom line (level 4)

  1. Fever, cough and chest pain were common in patients with acute chest syndrome.
  2. Around a third of patients had a normal chest examination.
Vichinsky et al: Blood 1997; 89 (5): 1787-1792
Expires October 2003

The study

Setting: 23 acute hospitals, USA

939 patients (aged :73% < 20 years old, ?% male) with sickle cell disease and acute chest syndrome

Independent unblinded reference standard, applied in all patients from a ?consecutive appropriate spectrum.
Reference standard:
  • new pulmonary infiltrate on chest X-ray or a perfusion defect on lung radioisotope scan
Diagnostic test: clinical findings and biochemistry

The evidence


diagnostic test number of patients sensitivity for
acute chest syndrome
(95% CI)		 LR+ LR-
fever 751 80%
(77% to 83%)
cough 695 74%
(71% to 77%)
chest pain 535 57%
(54% to 60%)
shortness of breath 263 28%
(25% to 31%)
productive cough 225 24%
(21% to 27%)
severe chest pain 207 22%
(19% to 25%)
chills 169 18%
(16% to 21%)
wheezing 103 11%
(9.0% to 13%)
haemoptysis 19 2.0%
(1.1% to 2.9%)
temperature > 39 C 291 31%
(28% to 34%)
respirations > 40/ minute 169 18%
(16% to 21%)
pulse > 140 beats/ minute 141 15%
(13% to 17%)
crackles 432 46%
(43% to 49%)
normal chest examination 329 35%
(32% to 38%)
dullness to percussion 291 31%
(28% to 34%)
total 939

Comments

  1. Better diagnostic techniques and aggressive management may improve outcomes.
  2. Fewer adults had fever (64%), but more had chest pain (84%), dyspnoea (47%) and a productive cough (44%).

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 clinical features