• C Riddington and S Owusu-Ofori.
• Institute of Child Health, University of Liverpool, Alder Hey Children's Hospital, Eaton Road, Liverpool, UK, L12 2AP. cerir@liverpool.ac.uk
• Cochrane Db Syst Rev. 2002 Jan 1 (3): CD003427.

BackgroundPeople with sickle cell disease are particularly susceptible to infection. Infants and very young children are especially vulnerable, and the Cooperative Study of Sickle Cell Disease observed an incidence rate of 10 per 100 patient years of pneumococcal septicaemia in children under the age of three. Vaccines, including customary pneumococcal vaccines, may be of limited use in this age group. Therefore, prophylactic penicillin regimes may be advisable for this population.ObjectivesTo assess the effects of prophylactic antibiotic regimes for preventing pneumococcal infection in children with sickle cell disease.Search StrategyWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references identified from comprehensive electronic database searches, hand searching relevant journals and hand searching abstract books of conference proceedings. Date of the most recent search: December 2001.Selection CriteriaAll randomised or quasi-randomised controlled trials comparing prophylactic antibiotics to prevent pneumococcal infection in children with sickle cell disease with placebo, no treatment or a comparator drug.Data Collection And AnalysisBoth reviewers independently extracted data and assessed trial quality.Main ResultsFive trials were identified by the initial search, of which three trials met the inclusion criteria. All of the included trials showed a reduced risk of infection in children with sickle cell disease (SS or Sb0Thal) receiving prophylactic penicillin. For initiation of treatment the odds ratio was 0.37 (95% CI 0.16, 0.86), and for withdrawal OR= 0.49 (95% CI 0.09, 2.71). Adverse drug effects were rare and minor. Rates of pneumococcal infection were found to be relatively low in children over the age of five.Reviewer's ConclusionsProphylactic penicillin significantly reduces risk of pneumococcal infection in children with homozygous sickle cell disease, and is associated with minimal adverse reactions. Further research may help to determine the ideal age to safely withdraw penicillin.

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