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通过磁共振成像(MRI)评估地中海贫血疾病患者的临床无症状脑梗死(SCI)。

Clinical silent cerebral infarct (SCI) in patients with thalassemia diseases assessed by magnetic resonance imaging (MRI).

作者信息

Metarugcheep Pichest, Chanyawattiwongs Somsak, Srisubat Kamolrat, Pootrakul Pensri

机构信息

MRI Unit, Prasat Neurological Institute, Department of Neurology, Ministry of Health, Bangkok, Thailand.

出版信息

J Med Assoc Thai. 2008 Jun;91(6):889-94.

PMID:18697390
Abstract

BACKGROUND

Silent cerebral infarct (SCI) could be detected on magnetic resonance imaging. It seems to be associated with the risk of stroke. Ischemic stroke has been reported in sickle cell anemia. Sickle red cell in hypoxic state associated with hypercoagulopathy is the risk factor of blood vessel occlusion leading to ischemic stroke. Hypercoagulable state has been documented in patients with beta thalassemia/Hb E disease, which their red cells are abnormal in deformity.

OBJECTIVE

Explore SCI in patients with beta thalassemia/Hb E disease and provide a guideline for prevention of stroke.

MATERIAL AND METHOD

Sixty-seven patients (29 males and 28 females, age 10-59 yrs, with a mean age of 31) with beta-thal/Hb E disease who were in the steady state without any neurological sign and symptom and no other associated stroke related disease such as DM, HT were included for MRI scanning. The cerebral MRI protocals were axial Flair, T2 Gre and 3DTOFMRA (3-dimension time of flight magnetic resonance angiography) of the brain.

RESULTS

67 patients (29 males and 28 females) with beta-thal/Hb E disease who were in the steady state without any neurological sign and symptom and no other associate stroke related disease such as DM, HT were included for MRI scanning. The ages of the patients were 10 to 59 years with a mean of 31 years. The abnormalities of the brain were found in 16 of 67 (24%). Most of the lesions were lacunar infarcts with varying amounts in the deep cerebral white matter. One cortical and subcortical infarct was observed with irregularity and stenosis of the intracranial vessels noted by MRA. All cases showed increased vascularity compared to the normal control subject.

CONCLUSION

This preliminary prevalence of 24% of SCI in this genotype of thalassemia was higher than found in sickle cell disease (11%). It may be associated with coagulopathy and deformity of the red cell. Further study is needed.

摘要

背景

无症状性脑梗死(SCI)可通过磁共振成像检测到。它似乎与中风风险相关。镰状细胞贫血患者中曾有缺血性中风的报道。处于缺氧状态且伴有高凝状态的镰状红细胞是导致血管闭塞进而引发缺血性中风的危险因素。β地中海贫血/Hb E病患者也存在高凝状态,其红细胞存在畸形异常。

目的

探究β地中海贫血/Hb E病患者中的无症状性脑梗死情况,并为中风预防提供指导。

材料与方法

纳入67例处于稳定状态、无任何神经体征和症状且无其他相关中风相关疾病(如糖尿病、高血压)的β地中海贫血/Hb E病患者(29例男性,28例女性,年龄10 - 59岁,平均年龄31岁)进行MRI扫描。脑部MRI检查方案包括轴位液体衰减反转恢复序列(Flair)、T2加权像(T2 Gre)和三维时间飞跃磁共振血管造影(3DTOFMRA)。

结果

纳入67例处于稳定状态、无任何神经体征和症状且无其他相关中风相关疾病(如糖尿病、高血压)的β地中海贫血/Hb E病患者(29例男性,28例女性)进行MRI扫描。患者年龄为10至59岁,平均31岁。67例中有16例(24%)发现脑部异常。大多数病变为腔隙性梗死,在深部脑白质中数量不等。观察到1例皮质和皮质下梗死,磁共振血管造影显示颅内血管不规则和狭窄。与正常对照相比,所有病例均显示血管增多。

结论

这种基因型地中海贫血中无症状性脑梗死的初步患病率为24%,高于镰状细胞病(11%)。它可能与凝血异常和红细胞畸形有关。需要进一步研究。

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