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首页> 外文期刊>Orphanet journal of rare diseases >Central nervous system manganese induced lesions and clinical consequences in patients with hereditary hemorrhagic telangiectasia
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Central nervous system manganese induced lesions and clinical consequences in patients with hereditary hemorrhagic telangiectasia

机译：中枢神经系统锰诱发的遗传性出血性毛细血管扩张患者的病变及临床后果

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BackgroundAround 47–74% of patients with hereditary hemorrhagic telangiectasia (HHT) have hepatic vascular malformations (HVMs); magnetic resonance images (MRI) of the central nervous system (CNS) might show in T1 sequences a hyper-intensity signal in different areas, mainly in the basal ganglia (BG) as consequence of manganese (Mn) deposits as observed in cirrhotic patients. These patients might suffer from different neuropsychiatric disorders (hepatic encephalopathy). In HHT patients, even in the presence of hepatic shunts, hepatocellular function is usually preserved. Additionally, Mn shares iron absorption mechanisms, transferrin and CNS transferrin receptors. In iron deficiency conditions, the Mn may harbor transferrin and access BG. The objectives were to describe frequency of BG Mn deposit-induced lesions (BGMnIL) in HHT patients, its relationship with iron deficiency anemia (IDA) and HVMs. Finally, explore the association between neuropsychological and motor consequences. We performed a cross-sectional study. We determined HHT patients with or without BG-MnIL by the MRI screening of the CNS. We included all patients with lesions and a random sample of those without lesions. All patients underwent standardized and validated neuropsychological assessment to evaluate BG actions. Results were analyzed with multiple logistic regression, adjusting for potential confounders. ResultsAmong 307 participants from a cohort included in the Institutional HHT Registry, 179 patients had MRI performed and Cura?ao Criteria ≥3. The prevalence of BG-MnIL was 34.6% (95%CI 27.69-42.09). While neuropsychological symptoms were present in all patients, BG-MnIL patients performed poorly in three of the neuropsychological tests (serial dotting, line tracing time, number connection test A). HVMs frequency in BG-MnIL was 95.1%, versus 71.4% in those without lesions ( p p p ConclusionsPhysicians should assess BG-MnIL in HHT patients in CNS-MRI. IDA and HVMs present increased risk of lesions. Patients with BG-MnIL have neuropsychological impairment, and they might benefit from sparing IDA, or undergoing future therapeutic options. Trial registration NCT01761981 . Registered January 3rd 2013.
机译：背景约47–74％的遗传性出血性毛细血管扩张症（HHT）患者患有肝血管畸形（HVM）。中枢神经系统（CNS）的磁共振图像（MRI）可能会在T1序列中显示不同区域的高强度信号，这主要是在肝硬化患者中观察到的锰（Mn）沉积的结果，主要在基底神经节（BG）中。这些患者可能患有不同的神经精神疾病（肝性脑病）。在HHT患者中，即使存在肝分流，通常仍能保留肝细胞功能。另外，Mn具有铁吸收机制，转铁蛋白和CNS转铁蛋白受体。在缺铁条件下，Mn可能带有运铁蛋白并进入BG。目的是描述HHT患者BG Mn沉积物引起的病变（BGMnIL）的频率，其与缺铁性贫血（IDA）和HVM的关系。最后，探讨神经心理学与运动后果之间的关联。我们进行了横断面研究。我们通过中枢神经系统的MRI筛查确定了有或没有BG-MnIL的HHT患者。我们纳入了所有有病变的患者，并随机抽取了无病变的患者。所有患者均接受了标准化且经过验证的神经心理学评估，以评估BG行为。通过多元逻辑回归分析结果，并校正潜在的混杂因素。结果在来自机构HHT注册中心的一个队列中的307名参与者中，有179例接受了MRI检查且库拉索标准≥3。 BG-MnIL的患病率为34.6％（95％CI 27.69-42.09）。虽然所有患者均出现神经心理症状，但BG-MnIL患者在三种神经心理测试（串行点测，线描迹时间，数字连接测试A）中表现较差。结论BG-MnIL中HVM的发生率为95.1％，而无病灶的HVMs发生率为71.4％（ppp结论）医师应在CNS-MRI中评估HHT患者的BG-MnIL。IDA和HVM会增加患病风险。 ，他们可能会受益于节省IDA或正在接受未来的治疗选择。NCT01761981试用注册; 2013年1月3日注册。

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