• C Riddington and L Williamson.
• 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. 2001 Jan 1 (3): CD003149.

BackgroundSickle cell disease is one of the most common inherited diseases in the world, and causes haemolytic anaemia, vaso-occlusive crises and dysfunction in virtually any organ system in the body. Surgical procedures are often required. Blood transfusion regimes can be used preoperatively in an attempt to increase transport of oxygen around the body and dilute the sickled red blood cells, thus reducing the risk of vaso-occlusion.ObjectivesTo assess the relative risks and benefits of preoperative blood transfusion regimes in patients with sickle cell disease undergoing surgery of any type in any setting.Search StrategyWe searched the Cochrane Cystic Fibrosis and Genetic Disorders Group specialist trials register which comprises references identified from comprehensive electronic database searches, handsearching relevant journals and handsearching abstract books of conference proceedings. Date of the most recent search: January 2001Selection CriteriaAll randomised or quasi-randomised controlled trials comparing preoperative blood transfusion regimes to different regimes or no transfusion in patients with sickle cell disease undergoing surgery.Data Collection And AnalysisBoth reviewers independently assessed trial quality and extracted data from the study included.Main ResultsOnly one trial met the inclusion criteria for the review. The trial compared an aggressive transfusion regime (decreasing sickle haemoglobin to less than 30%) to a conservative transfusion regime (increasing haemoglobin to 10 g/dl) in 604 elective operations in patients with sickle cell disease. The conservative regime was found to be as effective as the aggressive regime in preventing perioperative complications, and was associated with less transfusion related adverse events. No randomised controlled trials were found for transfusion compared to no transfusion.Reviewer's ConclusionsWhile in general conservative therapy appears to be as effective as aggressive in preparation for surgery in sickle cell patients, further research is needed to examine the optimal regime for different surgical types, and to address whether preoperative transfusion is needed in all surgical situations.

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