Significance of fever in Jamaican patients with homozygous sickle cell disease

Objective-To investigate the cause and outcome of high fever in Jamaican ch ildren with homozygous sickle cell disease. Design-Retrospective review of febrile episodes in a three year period (1 S eptember 1993 to 31 August 1996). Setting-Sickle cell clinic, an outpatient clinic in Kingston run by the Med ical Research Council Laboratories (Jamaica). Patients-Patients with homozygous sickle cell disease under 17 years of age presenting with an axillary temperature greater than or equal to 39.0 degr eesC (102.4 degreesF). (Main outcome measures-Diagnosis, death. Results-There were 165 events in 144 patients (66 (45.8%) boys) with a medi an age of 6.1 years. Bacteraemia was found in 10 (6.1%) events (three Strep tococcus pneumoniae, two Haemophilus influenzae type b, two Salmonella sp, one Escherichia coli, one Enterobacter sp, and one Acinetobacter sp), and u rinary tract infections in four (2.4%). All cultures of cerebrospinal fluid were sterile. Acute chest syndrome occurred in 36 (21.8%) events. A painfu l crisis was associated with 45 (27.3%) events and was the only pathology i dentified in 20 events (12.1%). Hospital admission was necessary in 66 case s including all those with bacteraemia and 31 with acute chest syndrome. Th ere were two deaths: a 5 year old boy with septic shock associated with H i nfluenzae septicaemia, and a 3 year old boy with the acute chest syndrome. Conclusions-Painful crisis and acute chest syndrome were the most common co mplications associated with high fever, but other important associated feat ures included bacteraemia and urinary tract infection. Enteric Gram negativ e organisms accounted for 50% of positive blood cultures.