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地中海贫血中的周围神经病变

Peripheral neuropathy in thalassaemia.

作者信息

Sawaya Raja A, Zahed Laila, Taher Ali

机构信息

Department of Internal Medicine, American University Medical Center, Beirut, Lebanon.

出版信息

Ann Saudi Med. 2006 Sep-Oct;26(5):358-63. doi: 10.5144/0256-4947.2006.358.

Abstract

BACKGROUND

Patients with thalassemia may complain of numbness and weakness of the lower extremities. The aim of the study was to document whether these patients suffer from a polyneuropathy and to determine any contributing factors for the development of neuropathy.

PATIENTS AND METHODS

We examined 30 patients with thalassemia major and intermedia, clinically and electrophysiologically. We correlated these findings with demographics, blood status, and treatment and compared electrophysiologic data with 30 age- and sex-matched normal subjects or historical controls.

RESULTS

We found that 78% of thalassemic patients suffered from a mild sensory polyneuropathy. The neuropathy seemed to be worse in the intermedia type. Thalassemic patients who received blood transfusions and deferoxamine had better nerve function than those who did not, irrespective of the dose of deferoxamine. The neuropathy was worse for older patients, irrespective of sex. The hemoglobin level, and the fact that some patients underwent splenectomy, did not affect the status of the patients' nerves.

CONCLUSION

Patients with thalassemia may suffer from a sensory polyneuropathy especially as they grow older and if they are not optimally treated.

摘要

背景

地中海贫血患者可能会抱怨下肢麻木和无力。本研究的目的是记录这些患者是否患有多发性神经病,并确定导致神经病发生的任何相关因素。

患者与方法

我们对30例重型和中间型地中海贫血患者进行了临床和电生理检查。我们将这些结果与人口统计学、血液状况和治疗情况相关联,并将电生理数据与30名年龄和性别匹配的正常受试者或历史对照进行比较。

结果

我们发现78%的地中海贫血患者患有轻度感觉性多发性神经病。中间型患者的神经病似乎更严重。接受输血和去铁胺治疗的地中海贫血患者的神经功能比未接受治疗的患者更好,与去铁胺的剂量无关。无论性别如何,老年患者的神经病更严重。血红蛋白水平以及部分患者接受脾切除术这一事实并未影响患者的神经状况。

结论

地中海贫血患者可能患有感觉性多发性神经病,尤其是随着年龄增长且未得到最佳治疗时。

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