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[维生素E在地中海贫血治疗中的作用]

[The role of vitamin E in the therapy of thalassemia].

作者信息

Bianco L, Boccaccini R, Capalbo P, Morici G, Maestro M, Mandrino M

出版信息

Pediatr Med Chir. 1986 Jan-Feb;8(1):23-6.

PMID:3725609
Abstract

Serum vitamin E values are depressed in thalassaemia owing to increased consumption because of the oxidative stress imposed both to red cells and other tissues by haemochromatosis. A study of vitamin E deficiency was carried over a period of about 2 years in 161 transfusion dependent thalassaemic patients aged 4 months to 18 years (including 74 splenectomized subjects) all following the same transfusion and chelation protocol (pretransfusion Hb = 11 gr/dl and daily chelation with subcutaneous infusion of desferrioxamine 12 hr a day). Serum vitamin E levels were determined by Martinek's method. The mean value for the entire series was 0.45 +/- 0.21 mg/dl (normal value 0.76 +/- 0.22), with no differences between splenectomized (0.43 +/- 0.19) and not--splenectomized (0.45 +/- 0.21) subjects. Values of less than 0.32 mg/dl (mean-2SD) were found in 50 patients (31,1%). Below--normal values were noted in 5/11 patients at the time of diagnosis. 124 subjects with less than 0.54 mg/dl received 5-10 mg/Kg/day vitamin E per os. In 38 cases it has been possible to control vitamin E level after one year of therapy. Mean values before treatment were 0.36 +/- 0.13 mg/dl and 1.19 +/- 0.35 mg/dl after therapy. No patient failed to respond and no adverse effect was recorded. These results show that by no means all thalassaemic patients are vitamin E deficient to the point where replacement therapy is necessary, and oral administration can easily correct low serum levels, contrary to what has been found by other workers.

摘要

由于血色素沉着症对红细胞和其他组织造成氧化应激,导致维生素E消耗增加,地中海贫血患者的血清维生素E值会降低。对161名年龄在4个月至18岁的依赖输血的地中海贫血患者(包括74名脾切除患者)进行了一项为期约2年的维生素E缺乏症研究,所有患者均遵循相同的输血和螯合方案(输血前血红蛋白 = 11克/分升,每天皮下输注去铁胺进行12小时的螯合治疗)。血清维生素E水平采用马丁内克法测定。整个系列的平均值为0.45 +/- 0.21毫克/分升(正常值为0.76 +/- 0.22),脾切除患者(0.43 +/- 0.19)和未脾切除患者(0.45 +/- 0.21)之间无差异。50名患者(31.1%)的维生素E值低于0.32毫克/分升(平均值 - 2标准差)。在诊断时,11名患者中有5名的维生素E值低于正常水平。124名维生素E值低于0.54毫克/分升的受试者口服5 - 10毫克/千克/天的维生素E。在38例患者中,经过一年的治疗后能够控制维生素E水平。治疗前的平均值为0.36 +/- 0.13毫克/分升,治疗后的平均值为1.19 +/- 0.35毫克/分升。没有患者无反应,也未记录到不良反应。这些结果表明,并非所有地中海贫血患者都存在维生素E缺乏到需要替代治疗的程度,而且与其他研究人员的发现相反,口服给药可以轻松纠正低血清水平。

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