Significance of fever in Jamaican patients with homozygous sickle cell disease

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 anxillary temperature o 39.0§c (102.4§F). MAIN OUTCOME MEASURES: Diagnosis, death. RESULTS: There were 165 event in 144 patients (66 (45.8 percent) boys) with a median age of 6.1 years. Bacteraemia was found in 10 (6.1 percent) events (three Streptococcus pneumoniae, two Haemophilus influenzae type b, two Salmonella sp, one Escherichia coli, one Enterobactor sp, and one Acinetobacter sp), and urinary tract infections in four (2.4 percent). All cultures of cerebrospinal fluid were sterile. Acute chest syndrome occured in 36 (21.8 percent) events. A painful crisis was associated with 45 (27.3 percent) events and was the only pathology identified in 20 events (12.1 percent). 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 influenzae septicaemia, 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 percent of positive blood cultures. (AU)

Postsplenectomy course in homozygous sickle cell disease

OBJECTIVE: To determine whether children with homozygous sickle cell (SS) disease and splenectomy are at greater risk of death, overwhelming septicemia, or other complications. METHODS: A total of 130 patients with SS treated by splenectomy (46 recurrent acute splenic sequestration, 84 chronic hypersplenism) over a 22.5-year period at the Sickle Cell Clinic of the University Hospital of the West Indies, Kingston, Jamaica, were compared with a control group matched for sex, age, and duration of follow-up in a retrospective review. Deaths and bacteremias were examined over the whole study period. Painful crises, acute chest syndromes, and febrile episodes were compared in the 90 patients completing 5 years of postsplenectomy follow-up. FINDINGS: Mortality and bacteremic episodes did not differ between the splenectomy and control groups. Painful crises were more common in the splenectomy group than in the control group (P = .01) but did not differ between splenectomy indications. Acute chest syndrome was more common in the splenectomy group than in the control group (P < .01) and was more common in the acute splenic sequestration group than in the hypersplenism group (P = .01). Febrile events did not differ between the groups or between the indications for splenectomy. CONCLUSION: Splenectomy does not increase the risk of death or bacteremic illness in patients with SS disease and, if otherwise indicated, should not be deferred for these reasons (Au)

Septicemia caused by Salmonella infection: an overlooked complication of sickle cell disease

OBJECTIVE: To describe the characteristics of salmonella infections in sickle cell disease and to compare the features of osteomyelitis and those with bacteremia/septicemia without obvious bone changes. To search for risk factors for osteomyelitis, and to draw attention to the frequency and significance of salmonella bacteremia/septicemia. STUDY DESIGN:A retrospective review of all salmonella isolations from the blood, pus, or aspirates during a 22-year period. SETTING: The sickle cell clinic at the University Hospital of the West Indies, Kingston, Jamaica. SUBJECTS: Patients with all genotypes of sickle cell diseases. Incidence data and the frequency of associated cholelithiasis were derived for the cohort study based on follow-up of all children detected by neonatal screening. MAIN OUTCOME MEASURES: Osteomyelitis and bacteremia/septicemia. RESULTS: Of 55 patients with salmonella infections, 25 initially had osteomyelitis and 27 had bacteremia/septicemia. Three of the first group later had bacteremias for a total of 30 episodes of bacteremia/septicemia, and 4 of the second group later had osteomyelitis for a total of 32 episodes of osteomyelitis. The incidence of salmonella infection was 8.6 percent by 15 years and 96 percent of infections occurred before the age of 10 years. Preceding episodes of avascular necrosis of bone were frequent (p < 0.006) in patients with osteomyelitis. Patients with osteomyelitis were not more prone to gallstones. High fever (temperature > or = 40 degrees C or 104 degrees F) occurred in 41 percent, and occasionally marked bone marrow suppression mimicked the aplastic crisis. Twenty Salmonella serotypes were isolated; Salmonella enteritidis accounted for 36 percent of infections, but no serotype difference occurred between those with osteomyelitis and those with bacteremia/septicemia. There were no deaths in the 32 patients with osteomyelitis, but 7 (23 percent) of 30 patients with septicemia died. CONCLUSIONS: Anti-salmonella prophylaxis requires assessment in the management of bone necrosis. Anti-salmonella agents may be indicated in undiagnosed septic conditions in sickle cell disease pending culture results.(AU)

Acute aphasia complicating typhoid fever in an adult

Motor aphasia complicating bacteriologically confirmed typhoid fever in a 20-year old adult female is presented. Neither the cause of death nor the aetiology of this isolated neurological deficit could be determined from detailed post-mortem examination. An immune related mechanism is suggested based on the temporal characteristics of this complication and CSF abnormalities.(AU)

Coagulase-negative staphylococcal bacteraemia in severely malnourished Jamaican children - abstract

Immunosuppression increases the susceptibility to infection and changes the inflammatory response in children with severe protein-energy malnutrition. In this five-year prospective study, bacteraemia was documented in 16 percent of 336 severely malnourished children (2-34 months of age), who were hospitalized consecutively in the Tropical Metabolism Research Unit, UWI, Kingston, Jamaica. The fifty-three children had 60 episodes of noscomial and community-acquired bacteraemia with 69 blood isolates. Community-acquired bacteraemia accounted for 72 percent (43/60) of bacteraemic episodes. Thirty-five per cent (24/69) of the strains were coagulase-negative staphylococci, 19 percent (13/69) were Staphylococcus aureus and 11 percent (8/69) were Streptococcus Group D. Seventeen episodes of coagulase-negative staphylococcal bacteraemia were acquired in the community, and seven were nosocomial. These patients were more likely to have pneumonic consolidation than children with all other bacteraemias combined (p<0.02, Fisher Exact Test). The bacteraemia-related case fatality rate was 8 percent (5/60). Polymicrobial and gram-negative septicaemia were independent positive predictive factors for mortality when compared with single-agent and gram-positive sepsis (p<0.02). This 71 percent (49/69) prevalence of gram-positive organisms suggests a change in the epidemiology from the predominant gram-negative aetiologies described in previous reports (AU)