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通过电生理研究评估β地中海贫血患者的周围神经病变:铁螯合剂时代的重新评估

Assessment of Peripheral Neuropathy in Patients with β-Thalassemia via Electrophysiological Study: Reevaluation in the Era of Iron Chelators.

作者信息

Bayhan Turan, Ünal Şule, Konuşkan Bahadır, Erdem Onur, Karabulut Erdem, Gümrük Fatma

机构信息

a Department of Pediatric Hematology , Hacettepe University , Ankara , Turkey.

b Department of Pediatric Neurology , Hacettepe University , Ankara , Turkey.

出版信息

Hemoglobin. 2018 Mar;42(2):113-116. doi: 10.1080/03630269.2018.1469510.

Abstract

Peripheral neuropathy is one of the complications of β-thalassemia (β-thal) that has been investigated in limited reports. We aimed to detect the rate of peripheral neuropathy and risk factors for neuropathy development in patients with β-thal. The study was performed in patients with β-thal intermedia (β-TI) or β-thal major (β-TM). Prospective electrophysiological studies were achieved via standard procedures. A total of 27 patients were enrolled in the study. Electrophysiological studies for both motor and sensory nerves were within normal range. In motor nerve studies, delayed peroneal nerve latency was found in patients with high ferritin levels, increased ulnar nerve amplitude was detected in patients ≥20 years old, and increased tibial nerve amplitude was seen in patients with low copper levels. We could not show peripheral neuropathy in our patients. Increased ferritin level, older age, and copper deficiency may cause mild changes in electrophysiological studies of motor nerves.

摘要

周围神经病变是β地中海贫血(β-地贫)的并发症之一,相关报道有限。我们旨在检测β-地贫患者周围神经病变的发生率及神经病变发生的危险因素。该研究在中间型β-地贫(β-TI)或重型β-地贫(β-TM)患者中进行。通过标准程序进行前瞻性电生理研究。共有27例患者纳入该研究。运动和感觉神经的电生理研究均在正常范围内。在运动神经研究中,铁蛋白水平高的患者腓总神经潜伏期延迟,≥20岁的患者尺神经波幅增加,铜水平低的患者胫神经波幅增加。我们的患者未出现周围神经病变。铁蛋白水平升高、年龄较大和铜缺乏可能导致运动神经电生理研究出现轻微变化。

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