Elalfy Mohsen S, Adly Amira A M, Attia Atef A M, Ibrahim Fatma A, Mohammed Amer S, Sayed Abdelbasset M
Pediatric Department, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Hemoglobin. 2013;37(3):257-76. doi: 10.3109/03630269.2013.778866. Epub 2013 Apr 9.
To assess the effects of combined vitamin therapy on oxidant-antioxidant hepatic status and hemoglobin (Hb) derivatives on β-thalassemia major (β-TM), a prospective study of 60 β-TM patients aged 4 to 17 years, was conducted. Thirty-nine patients with initial low serum vitamins E, C and A, were treated with oral combined vitamins for 1 year compared to 21 patients with normal vitamin levels. Serum transaminases, serum ferritin, hepatic fibroscan elastography (TE) and magnetic resonance imaging R2* (MRI R2*) for liver iron concentration (LIC), were assessed before and after 6 and 12 months of therapy. Antioxidant capacity was assessed by levels of reduced glutathione (GSH), malondialdehyde (MDA), catalase, superoxide dismutase and GSH enzymes. The studied vitamins, reduced GSH and Hb levels were significantly elevated and paralleled by progressive decline in MDA and ferritin during therapy (p <0.001). Serum transaminase and superoxide dismutase were significantly decreased, while GSH reductase was significantly elevated during therapy (p <0.001). Improvement of hepatic fibrosis as 23.0% had TE (>12 kPa) at baseline compared to 20.5% after therapy (p >0.05), although LIC values were significantly decreased (p <0.001). Combined vitamin therapy improves the antioxidant/oxidant balance, LIC and hepatic fibrosis in young β-TM patients.
为评估联合维生素疗法对重型β地中海贫血(β-TM)患者肝脏氧化还原状态及血红蛋白(Hb)衍生物的影响,我们对60例年龄在4至17岁的β-TM患者进行了一项前瞻性研究。39例初始血清维生素E、C和A水平较低的患者接受了为期1年的口服复合维生素治疗,另有21例维生素水平正常的患者作为对照。在治疗前以及治疗6个月和12个月后,分别评估血清转氨酶、血清铁蛋白、肝脏瞬时弹性成像(TE)以及用于评估肝脏铁浓度(LIC)的磁共振成像R2*(MRI R2*)。通过还原型谷胱甘肽(GSH)、丙二醛(MDA)、过氧化氢酶、超氧化物歧化酶和谷胱甘肽酶的水平评估抗氧化能力。研究发现,治疗期间所补充的维生素、还原型谷胱甘肽和血红蛋白水平显著升高,同时丙二醛和铁蛋白水平逐渐下降(p<0.001)。治疗期间血清转氨酶和超氧化物歧化酶显著降低,而谷胱甘肽还原酶显著升高(p<0.001)。尽管肝脏铁浓度值显著下降(p<0.001),但肝脏纤维化有所改善,基线时23.0%的患者TE值>12kPa,治疗后这一比例为20.5%(p>0.05)。联合维生素疗法可改善年轻β-TM患者的抗氧化/氧化平衡、肝脏铁浓度和肝纤维化情况。