Pediatric intracranial aneurysms——clinical characteristics and therapeutic strategies

Objective To assess the radiological characteristics and therapeutic strategies of intracranial aneurysms in children. Methods From our dedicated neurovascular databank of patients, we reviewed 23 consecutive children who had 24 intracranial aneurysms. There were 14 boys and 9 girls with a mean age of 9.09 years ( range 1 - 14 years ). Results Intracranial aneurysms in children ≤ 14 years constituted 1.3% of all intracranial aneurysms. Internal Carotid artery (ICA) and middle cerebral artery (MCA) were the most frequent sites for aneurysms. About 58.3% of the aneurysms were complex, including dissecting, pseudoaneurysm, giant and fusiform aneurysm. 1/3 of all aneurysms were located in posterior circulation. Only 1 case had multiple aneurysms in this case series. Almost half of all cases presented with subarachnoid hemorrhage and others presented with mass effect. 14 cases underwent endovascular treatment. 4 patients received microsurgical therapy. 5 cases did not receive microsurgical or endovascular therapy, 2 of them whose aneurysms spontaneously thrombosed during follow up. One boy with left vertebral artery giant aneurysm died after endovascular therapy owing to gradual thrombosis in basilar artery. Another child had poor outcome because of rerupture of aneurysm before operation. Whereas the majority had a favorable outcome. Conclusions Intracranial aneurysms in children had many clinical and radiological characteristics different from those in adults : ( 1 ) remarkable male predominance; ( 2 ) ICA and MCA were the most common sites for aneurysms; (3) high incidence of large, traumatic, infectious, dissecting and fusiform aneurysms. (4)For pediatric intracranial aneurysms, both microsurgical approaches and endovascular treatment were effective. Endovaacular therapy was the best choice for complex aneurysms.     

脸色腰段椎间盘突出症21例临床分析

胸腰段椎间盘突出症罕见,约占椎间盘突出的0.6％～4％。随着CT及MRI的应用,其发病率有增加的趋势。因其发病率低,报道较少。但由于病变位于脊髓圆椎段,其发病较下腰段椎间盘突出有很大的特殊性,故有必要结合我院近年收治的21例对其作一回顾性分析。1 临床资料     

中欧地区COL7A1基因高发的第425位碱基A→G剪接位点突变和新发现的突变:对未来进行营养不良性大疱性表皮松解症突变检测的意义

背景:V II胶原(COL7A1)基因突变是引起显性和隐性营养不良性大疱性表皮松解症(DEB)的主要原因。对各个不同家族这些突变通常是特异的,仅有少数病例被鉴定是同一的突变引起。目的:对43例不同D EB表型的无亲缘关系的匈牙利和德国患者进行筛查,观察其是否存在新的CO L7A1突变或是为重复突变所致。方法:所有患者根据临床和遗传学检查,皮肤免疫荧光抗原图和电镜检查进行分类。用CO L7A1特异性引物采用聚合酶链反应(PCR)技术扩增基因组DN A,杂交双链分析和直接核甘酸测序对突变进行分析。限制性核酸内切酶消化法用于家族的筛选和突变的…     

α-1抗胰蛋白酶缺乏病和Henoch-Schnlein紫癜与免疫球蛋白A同种型的抗中性粒细胞胞浆抗体和抗内皮细胞抗体相关

α-1抗胰蛋白酶(A1AT)缺乏病是一种遗传性酶缺乏病,表现为致命性肺肝并发症。除了肝肺受累外,该病也常发生脉管炎综合征和自身免疫病,包括韦格纳肉芽肿,结节性多动脉炎和H enoch-Sch nlein紫癜(H SP)。HSP是一种系统性小血管炎综合征,以非血小板病性紫癜、关节痛、腹痛和肾炎为特征。A1A T缺乏和HSP与抗中性粒细胞胞浆抗体(A NCA)和抗内皮细胞抗体(A ECA)有关。作者报道1例40岁男性患严重的A1A T缺乏病,后发展为H SP,与免疫球蛋白A同种型的A ECA、A NCA和抗磷脂抗体有关。α-1抗胰蛋白酶缺乏病和Henoch-Schnlein紫癜与免疫…     

异位心(胸腹型)1例报告

异位心是罕见的先天性畸形,以胸部型心异位最为常见。我们遇到1例胸腹型异位心,为明确心内畸形作了全面检查,现报告如下.患者,女,19岁,自幼发现心脏杂音,平时易受凉发烧,活动后气喘,一岁后逐渐出现紫绀,无蹲踞及晕厥史.发育营养较差,口唇、面部紫绀,杵状指趾。BP110/70mmHg,P88次/分。胸部对称,     

幼年性非透明蛋白纤维瘤病:不伴有显著玻璃样变的幼年性透明蛋白纤维瘤病

幼年性透明蛋白纤维瘤病(JH F)是一种罕见的结缔组织常染色体隐性遗传病。本病以皮肤丘疹结节,皮下结节,伴齿龈肥大、溶骨及屈曲挛缩为特征。报道一例14岁女孩患JH F典型临床表现并死亡。我们发现患儿早期损害的多次皮肤活检结果缺乏应有的玻璃样变。据经验,该病的皮肤病理特征通常包括典型的玻璃样变。只有病期较长,皮损相对较大的患者可能表现为这些特点。因此,我们建议,JH F可能常常表现为幼年性非透明蛋白纤维瘤病:无明显玻璃样变的JH F。但这个事实不应改变现行的诊断标准和预后判断。幼年性非透明蛋白纤维瘤病:不伴有显著玻璃样…     

东芝KXO-15C 500mA X线机点片系统故障2例

故障现象1 点片架不能升降. 故障分析开机发现无论操作spotup或spotdown,升降电机都不运转,但继电器RY5、RY6、与RY9都动作,用万用表测得RY9(32、42)触点提供给电机运转绕组的电压正常,但给电机启动绕组供电的继电器RY7不动,说明RY7的线圈没有得电.     

新西兰有同性性行为HIV阳性男性肛周人乳头瘤病毒感染的患病率和清除率

本研究旨在确定有同性性行为男性(M SM)且H IV阳性者肛周人乳头瘤病毒(H PV)感染的患病率及随访数据与H PV感染患病率和清除率的相关性。比较两组被访者H PV患病率和H IV血清状态的数据。两组被访者均行常规性病学检查和肛周拭子H PV DN A检测。结果发现H PV16、18、31、33和52型在H IV阳性个体极易检出。H IV阳性M SM中H PV31型的持续存在非常常见(P=0.036),而H PV16型的存在可能与H IV阳性相关(P=0.059)。研究结果表明在H IV阳性M SM肛周区域极易检出更多高危H PV型。新西兰有同性性行为HIV阳性男性肛周人乳头瘤…