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Asymptomatic intracranial aneurysms in beta-thalassemia: a three-year follow-up report.
Orphanet Journal of Rare Diseases ( IF 3.4 ) Pub Date : 2020-01-20 , DOI: 10.1186/s13023-020-1302-3
Renzo Manara 1 , Martina Caiazza 2 , Rosanna Di Concilio 3 , Angela Ciancio 4 , Elisa De Michele 5 , Caterina Maietta 2 , Daniela Capalbo 2 , Camilla Russo 6 , Domenico Roberti 2 , Maddalena Casale 2 , Andrea Elefante 6 , Fabrizio Esposito 1 , Sara Ponticorvo 1 , Andrea Gerardo Russo 1 , Antonietta Canna 1 , Mario Cirillo 7 , Silverio Perrotta 2 , Immacolata Tartaglione 2
Affiliation  

BACKGROUND No information is currently available regarding the natural history of asymptomatic intracranial aneurysms in beta-thalassemia, raising several concerns about their proper management. METHODS We performed a prospective longitudinal three-year-long MR-angiography study on nine beta-thalassemia patients (mean-age 40.3 ± 7.5, six females, 8 transfusion dependent) harboring ten asymptomatic intracranial aneurysms. In addition, we analyzed the clinical files of all adult beta-thalassemia patients (160 patients including those followed with MR-angiography, 121 transfusion dependent) referring to our Centers between 2014 and 2019 searching for history of subarachnoid hemorrhage or history of symptomatic intracranial aneurysms. RESULTS At the end of the three-year-long follow-up, no patient showed any change in the size and shape of the aneurysms, none presented new intracranial aneurysms or artery stenoses, none showed new brain vascular-like parenchymal lesions or enlargement of the preexisting ones. Besides, in our database of all adult beta-thalassemia patients, no one had history of subarachnoid hemorrhage or history of symptomatic intracranial aneurysms. CONCLUSIONS Incidental asymptomatic intracranial aneurysms do not seem to be associated, in beta-thalassemia, with an increased risk of complications (enlargement or rupture) at least in the short term period, helping to optimize human and economic resources and patient compliance during their complex long-lasting management.

中文翻译:

β地中海贫血无症状颅内动脉瘤:三年随访报告。

背景技术目前尚无关于β地中海贫血中无症状颅内动脉瘤的自然病史的信息,这引起了对其妥善治疗的担忧。方法我们对9例β-地中海贫血患者(平均年龄40.3±7.5,6名女性,8例依赖输血)进行了一项为期三年的前瞻性纵向MR血管造影研究,其中有10例无症状的颅内动脉瘤。此外,我们分析了2014年至2019年之间所有成人β型地中海贫血患者(包括MR血管造影患者,其中121例依赖输血的患者)的临床档案,以研究蛛网膜下腔出血史或有症状颅内动脉瘤史。结果在为期三年的随访结束时,没有患者显示出动脉瘤的大小和形状的任何变化,没有患者出现新的颅内动脉瘤或动脉狭窄,没有患者显示出新的脑血管样实质性病变或原有病变的扩大。此外,在我们所有成人β地中海贫血患者的数据库中,没有人有蛛网膜下腔出血史或有症状的颅内动脉瘤史。结论至少在短期内,β-地中海贫血偶然的无症状颅内动脉瘤似乎与并发症(肿大或破裂)风险增加无关,这有助于优化人力和经济资源以及患者复杂的长期依从性持久的管理。没有人显示出新的脑血管样实质性病变或原有病变的扩大。此外,在我们所有成人β地中海贫血患者的数据库中,没有人有蛛网膜下腔出血史或有症状的颅内动脉瘤史。结论至少在短期内,β-地中海贫血偶然的无症状颅内动脉瘤似乎与并发症(肿大或破裂)风险增加无关,这有助于优化人力和经济资源以及患者复杂的长期依从性持久的管理。没有人显示出新的脑血管样实质性病变或原有病变的扩大。此外,在我们所有成人β地中海贫血患者的数据库中,没有人有蛛网膜下腔出血史或有症状的颅内动脉瘤史。结论至少在短期内,β地中海贫血中偶然的无症状颅内动脉瘤似乎与并发症(肿大或破裂)的风险增加无关,这有助于优化人力和经济资源以及患者长期复杂的依从性持久的管理。
更新日期:2020-01-21
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