Article Text

Download PDFPDF
Significance of fever in Jamaican patients with homozygous sickle cell disease
  1. K J J Wierenga,
  2. I R Hambleton,
  3. R M Wilson,
  4. H Alexander,
  5. B E Serjeant,
  6. G R Serjeant
  1. Sickle Cell Unit, formerly MRC Laboratories (Jamaica), University of the West Indies, Mona, Kingston, Jamaica, West Indies
  1. Dr Wierenga, Sickle Cell Trust, 14 Milverton Crescent, Kingston 6, Jamaica, West Indieskjwier{at}


OBJECTIVE To investigate the cause and outcome of high fever in Jamaican children with homozygous sickle cell disease.

DESIGN Retrospective review of febrile episodes in a three year period (1 September 1993 to 31 August 1996).

SETTING Sickle cell clinic, an outpatient clinic in Kingston run by the Medical Research Council Laboratories (Jamaica).

PATIENTS Patients with homozygous sickle cell disease under 17 years of age presenting with an axillary temperature ⩾ 39.0°C (102.4°F).


RESULTS There were 165 events in 144 patients (66 (45.8%) boys) with a median age of 6.1 years. Bacteraemia was found in 10 (6.1%) events (threeStreptococcus pneumoniae, twoHaemophilus influenzae type b, twoSalmonella sp, oneEscherichia coli, oneEnterobacter sp, and oneAcinetobacter sp), and urinary tract infections in four (2.4%). All cultures of cerebrospinal fluid were sterile. Acute chest syndrome occurred in 36 (21.8%) events. A painful crisis was associated with 45 (27.3%) events and was the only pathology identified in 20 events (12.1%). Hospital admission was necessary in 66 cases including all those with bacteraemia and 31 with acute chest syndrome. There were two deaths: a 5 year old boy with septic shock associated with H influenzaesepticaemia, and a 3 year old boy with the acute chest syndrome.

CONCLUSIONS Painful crisis and acute chest syndrome were the most common complications associated with high fever, but other important associated features included bacteraemia and urinary tract infection. Enteric Gram negative organisms accounted for 50% of positive blood cultures.

  • homozygous
  • sickle cell disease
  • fever
  • bacteraemia
  • acute chest syndrome
  • painful crisis

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.