作者: Wai Cheng Foong , Jacqueline J Ho , C Khai Loh , Vip Viprakasit
DOI: 10.1002/14651858.CD011579.PUB2
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摘要: Background Non-transfusion dependent beta thalassaemia is a subset of inherited haemoglobin disorders characterised by reduced production the globin chain molecule leading to anaemia varying severity. Although blood transfusion not necessity for survival, it required when episodes chronic occur. This can impair growth and affect quality life. People with non-transfusion suffer from iron overload due their body's increased capability absorbing food sources. Iron becomes more pronounced in those requiring transfusion. higher foetal level have been found require fewer transfusions. Hydroxyurea has used increase level; however, its efficacy reducing transfusion, complications safety need be established. Objectives To assess effectiveness, appropriate dose regimen hydroxyurea people (haemoglobin E combined intermedia). Search methods We searched Cochrane Cystic Fibrosis Genetic Disorders Group's Haemoglobinopathies Trials Register, compiled electronic database searches handsearching relevant journals. also ongoing trials registries reference lists articles reviews.Date last search: 30 April 2016. Selection criteria Randomised or quasi-randomised controlled comparing placebo standard treatment different doses hydroxyurea. Data collection analysis Two authors independently applied inclusion order select inclusion. Both assessed risk bias extracted data. A third author verified these assessments. Main results No care were found. However, we included one randomised trial (n = 61) 20 mg/kg/day 10 24 weeks.Both levels lower at weeks mg group compared group, mean difference -2.39 (95% confidence interval - 2.8 -1.98) -1.5 -1.83 -1.17), respectively. Major adverse effects significantly common neutropenia ratio 9.93 1.34 73.97) thrombocytopenia 3.68 1.13 12.07). was reported minor (gastrointestinal disturbances raised liver enzymes). The effect on frequency reported.The overall outcomes graded as very low mainly because derived only small study an unclear method allocation concealment. Authors' conclusions There no evidence show whether any controls Administration resulted seems safer effects. It capable Large well-designed sufficient duration follow up are recommended.