PubMed · 10608339
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N M Rosenberg, T G Givens, C Ledwith, K M Smith. 1999. Expeditious disposition.. https://doi.org/10.1097/00006565-199912000-00021
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BACKGROUND: Sickle cell disease comprises a group of genetic haemoglobin disorders. The predominant symptom associated with sickle cell disease is pain resulting from the occlusion of small blood vessels by abnormally 'sickle-shaped' red blood cells. There are other complications, including chronic organ damage and prolonged painful erection of the penis, known as priapism. Severity of sickle cell disease is variable, and treatment is usually symptomatic. Priapism affects up to half of all men with sickle cell disease, however there is no consistency in treatment. We therefore need to know the best way of treating this complication in order to offer an effective interventional approach to all affected individuals. OBJECTIVES: To assess the benefits and risks of different treatments for stuttering (repeated short episodes) and fulminant (lasting for six hours or more) priapism in sickle cell disease. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. We also searched the internet for further references. Date of the most recent search of the Group's trials register: August 2004. SELECTION CRITERIA: All randomised or quasi-randomised controlled trials comparing non-surgical or surgical treatment with placebo or no treatment, or with another intervention for stuttering or fulminant priapism. DATA COLLECTION AND ANALYSIS: The reviewers independently extracted data and assessed study quality. MAIN RESULTS: Only one study of 11 participants was identified and met the criteria for inclusion in this review. This study compared stilboestrol to placebo. The only outcome specified in this review, which was assessed in the study, was reduction in frequency of stuttering priapism and there was no difference between the groups, relative risk 0.18 (95%CI 0.01 to 2.54). REVIEWERS' CONCLUSIONS: There is no evidence for the benefits or risks of the different treatments for both stuttering and fulminant priapism in sickle cell disease. This systematic review has clearly identified the need for well-designed, adequately-powered, multicentre randomised controlled trials assessing the effectiveness of specific interventions for priapism in sickle cell disease.
BACKGROUND: Avascular necrosis of bone is a frequent and severe complication of sickle cell disease and its treatment is not standardised. It is therefore important to gather evidence about the safety and effectiveness of different interventions. OBJECTIVES: To determine the impact of surgery compared to non-surgical management on both the short- and the long-term outcomes (efficacy, safety, and adverse events) for people with sickle cell disease-related avascular necrosis. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group trials register, which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Additional randomized controlled trials were sought from the reference lists of the trials found and reviews identified by the search strategy. Date of the most recent search: April 2004. SELECTION CRITERIA: All randomized or quasi-randomized controlled trials (published or unpublished). DATA COLLECTION AND ANALYSIS: One ongoing clinical trial has been identified but no data are available for inclusion in the review. MAIN RESULTS: Five trials were identified by the searches. Four trials were not eligible for inclusion and one is an ongoing clinical trial. REVIEWERS' CONCLUSIONS: We were unable to find any evidence from completed randomized controlled trials assessing treatments for avascular necrosis in people with sickle cell disease. We await the results of an ongoing clinical trial to assess the benefits and risks of a surgical approach compared to a non-surgical approach as a means of improving survival and quality of life for people with sickle cell disease-related avascular necrosis of bone.