成人烟雾病侧支循环的CT血管成像特点

目的:探讨烟雾病侧支循环形成的CT血管成像特点。方法:收集在医院就诊的120例烟雾病患者影像资料,所有患者均行CT血管成像(CTA),根据侧支循环代偿途径的不同将其分为Ⅰ组(15例),Ⅱ组(53例),Ⅲ组(38例),Ⅳ组(14例)。采用Lightspeed VCT对所有烟雾病患者行头颅CTA检查,评价4组患者的临床表现、烟雾病血管代偿模式以及脑底异常血管网情况。结果:Ⅰ组15例全部为缺血型烟雾病;Ⅱ组53例中缺血型烟雾病8例,出血型烟雾病45例;Ⅲ组38例中缺血型烟雾病7例,出血型烟雾病31例;Ⅳ组14例全部为出血型烟雾病。结论:缺血型烟雾病血管网以迂曲扩张或正常为主,而出血型烟雾病血管网以狭窄为主。烟雾病的类型与侧支循环血管代偿特点有密切关联。     

成年人烟雾病23例临床分析

目的 提高对成年人烟雾病的进一步认识,探讨其有效治疗方法,以期在基层医院开展烟雾病的外科治疗.方法 回顾性分析23例成年人烟雾病患者的发病年龄、性别、临床表现、CT血管造影(CTA)和数字减影血管造影(DSA)的影像学资料,并总结治疗结果.结果 23例患者中,以颅内出血表现为主17例(73.9%),以脑缺血表现为主6例(26.1%).出血部位依次为颅内出血伴破入脑室,单纯脑室出血,左、右颞单纯脑实质出血,蛛网膜下隙出血.DSA显示双侧颈内动脉(ICA)虹吸部、大脑前动脉(ACA)及大脑中动脉(MCA)起始部狭窄或闭塞5例,单侧2例;双侧ACA及MCA起始部狭窄或闭塞5例,单侧1例,合并双侧椎-基底动脉、大脑后动脉(PCA)狭窄1例.CTA及DSA检查共发现烟雾病合并动脉瘤6例(8枚).手术治疗(含栓塞治疗)10例:脑内血肿清除术、脑室外引流、主要动脉型动脉瘤及烟雾血管内动脉瘤介入栓塞治疗.再出血2例;死亡3例,病死率为13.0%.结论 (1)单纯的脑室出血或脑实质出血破入脑室,出血量较大或四脑室铸型并有脑室进行性扩大时行脑室外引流治疗,出血量较少者可保守治疗.(2)脑实质出血量大于30 ml可行手术清除血肿.注意保护颞浅动脉及其分支及重要的侧支循环血管.(3)微创YL-1型锥颅针方法对脑实质出血更有优势,可避免对已形成颅内外重要侧支循环的破坏.(4)对与出血有关的合并动脉瘤或异常血管网内微小动脉瘤,行动脉瘤介入栓塞治疗,减少再出血的机会.预后主要取决于颅内出血部位、出血量和有无再出血及治疗时机等.     

儿童与成人烟雾病数字减影血管造影对比分析

目的 探讨儿童型和成人型烟雾病患者脑血管造影的特征及与临床表现的关系。方法 回顾性分析儿童及成人20例烟雾病患者的临床表现及数字减影血管造影（DSA）的影像特点。结果 成人颈内动脉（ICA）、大脑前动脉（ACA）、大脑中动脉（MCA）双侧对称受累较儿童显著多见（P〈0．01）；ICA以狭窄为主，ACA、MCA段闭塞明显增多，成人闭塞比例较儿童更高（P〈0．05）。儿童44．44％，成人72．73％脑底有大量烟雾状血管；大脑后动脉及其分支增粗者儿童77．78％，成人81．82％；颈外动脉向颈内动脉的代偿：儿童组44．44％，成人组36．36％。结论 儿童型和成人型烟雾病患者的DSA表现明显不同，儿童型颈内动脉病变较轻，侧支代偿较差，临床表现以缺血多见；成人型颈内动脉病变较严重，侧支代偿较好，临床表现以出血为主。基底部烟雾状血管的广泛发展、扭曲紊乱与脑出血密切相关。     

经颅多普勒在烟雾病诊断中的应用

目的探讨经颅多普勒(transcranial doppler,TCD)在烟雾病诊断中的价值。方法回顾性分析2010年1月—2012年12月确诊的14例烟雾病患者,对TCD检查发现双侧前循环狭窄或闭塞性改变,结合临床表现、实验室检查怀疑可能为烟雾病的患者,行数字减影血管造影(digital subtraction angiography,DSA)检查明确诊断。结果共有14例患者确诊为烟雾病。其中有5例TCD显示大脑中动脉起始部高流速血流频谱;7例TCD显示双侧颈内动脉终末段血流速度减慢,2例显示血流速度增快,同一深度可探及多条频谱形态和方向不同的血流信号,PI值均减小。结论 TCD可以判断烟雾病病变血管的狭窄程度和侧支循环代偿情况,并且具有无创、操作简便、费用低等优点,可以作为烟雾病诊断、判断病变程度及定期随访的重要工具。     

颞浅动脉-大脑中动脉搭桥术治疗烟雾病的护理配合

烟雾病（Moyamoya disease.MMD）是一种少见的,原因不明的慢性进行性颅底动脉血管进行性闭塞性病变,以双侧颈内动脉末端和大脑前动脉或大脑中动脉近端狭窄或闭塞,伴有颅底部异常血管网形成为特点[1]。该疾病最先由Takeuchi报告并由Shimizu依据脑血管造影所见形象的命名为烟雾病[2]。临床主要表现为缺血和出血两大类型。起病年龄有5岁和40岁左右的双峰分布,患儿以缺血为主要临床表现,成年患者缺血与出血表现基本同概率[3]。缺血型的烟雾病表现为头晕、头痛、癫痫、视觉改变、运动、感觉等功能异常,严重者可出现智力障碍。出血型的烟雾病主要表现为剧烈的头痛,喷射性呕吐,严重者出现昏迷甚至脑疝危及生命。患儿多采用内科保守治疗,外科手术是成人的主要治疗方法。2014年1月~2015年12月我院手术室行颞浅动脉—大脑中动脉搭桥手术治疗烟雾病9例取得满意效果,现总结报告如下。     

DSA在烟雾病诊断中的应用价值分析

目的：评价数字减影血管造影（DSA）对烟雾病的诊断价值。方法：回顾性分析12例烟雾病患者DSA检查。结果：12例烟雾病DSA表现为：不同程度颈内动脉分叉处血管狭窄或闭塞,侧支循环形成呈烟雾状。结论：DSA可清晰地显示烟雾病血管狭窄程度或闭塞的部位及侧支循环情况,为临床治疗提供直接的影像学资料。     

成年人出血性烟雾病22例治疗体会

目的 分析和总结成年人出血性烟雾病的临床特点,进一步提高诊治水平.方法 回顾性分析22例成年人出血性烟雾病患者的临床资料,包括发病年龄、临床表现、影像学表现、治疗方法及随访情况.结果 表现为蛛网膜下隙出血5例、单纯颅内出血4例、单纯脑室出血2例、颅内出血+脑室出血11例.所有患者均经CT血管造影(CTA)确诊为出血性烟雾病.13例行手术治疗(含介入治疗):脑内血肿清除4例,脑室外引流6例,主要动脉型动脉瘤介入治疗2例,烟雾血管内动脉瘤介入治疗1例.9例保守治疗者按常规予以改善微循环、脑保护剂、脱水等治疗.疗效优良者18例,1例恢复差,3例死亡.结论 成年人出血性烟雾病的治疗需结合出血部位、出血量、是否合并动脉瘤等多因素以采取不同治疗方式.预后主要取决于颅内出血部位、出血量、有无再次出血及治疗时机等.     

出血型烟雾病搭桥术后再出血现状及危险因素分析

目的:分析出血型烟雾病搭桥术后再出血现状及危险因素。方法:回顾性分析2013年6月至2017年8月复旦大学附属华山医院神经外科行搭桥术的90例出血型烟雾病患者临床资料,依据术后随访2年是否出现再出血将其分为观察组(术后随访2年内再出血)、对照组(术后随访2年内无再出血),比较两组临床资料,采用Cox回归模型分析影响出血...     

小儿烟雾病20例临床分析

目的:探讨小儿烟雾病临床检查及诊断。方法:对20例小儿烟雾病的临床资料,结合检查进行分析。结果:20例小儿烟雾病脑血管造影显示为双侧或一侧颈内动脉虹吸部末端、大脑中动脉、前动脉近端严重狭窄或闭塞,全部病例均可见底节区烟雾状异常血管网。核磁共振成像(MR I)检查与脑血管造影检查基本相符。结论:脑血管造影检查是烟雾病的主要检查手段,MR I检查可作为烟雾病的筛选检查手段。     

烟雾病行联合血管重建术的术后并发症及其护理分析

目的:分析烟雾病行联合血管重建术的术后并发症及其护理。方法:随机抽取我院2018-06～2019-09收治的200例烟雾病患者为研究对象,用随机数字分析法将其分为研究组合对照组,两组均给予联合血管重建术(颞浅动脉-大脑中动脉搭桥(STA-MCA bypass)+颞肌贴敷术(EMS),观察患者在术后出现的并发症情况,并给予研究组患者优质护理观察组患者常规护理,对比两组患者的干预效果。结果:200例烟雾病患者中术后出现13例癫痫发作(6.50%)、20例言语障碍(10.00%)、15例单侧肢体感觉活动减退(7.50%)及8例再出血(4.00%),经过早期发现和术后针对性护理后,均痊愈出院,平均住院时长为9.32±3.01d。结论:对于烟雾病行联合重建术治疗效果尤为显著,但术后易出现一系列并发症,因此需要及时给予有效的护理干预,积极处理并发症,以此来改善患者预后效果,缩短住院时长。     

Screen-detected gallstone disease and cardiovascular disease

Knowledge about temporal associations for screen-detected gallstone disease and cardiovascular disease is limited. The objective of this study was to determine if screen-detected gallstones or cholecystectomy was associated with development of cardiovascular disease. A cohort study of three randomly selected groups from the general population of Copenhagen was performed. Participants (n = 5928) were examined 1982–1992 and underwent abdominal ultrasound examination to detect gallstone disease and were not informed of their gallstone status. Participants were followed up for occurrence of cardiovascular disease through nationwide registers until December 2014. Multivariable Cox regression analyses were performed including traditional cardiovascular disease risk factors and apolipoprotein E genotype. Gallstone disease was identified in 10% (591/5928) of participants at baseline of whom 6.8% had gallstones and 3.2% had cholecystectomy. The study population was followed for a period of 32 years with only 1% lost to follow-up. Gallstone disease was associated with all cardiovascular disease (hazard ratio (HR) 1.36, 95% confidence interval (CI) [1.17;1.59]) and to the subgroups coronary artery (HR 1.34, 95% CI [1.10;1.64]), cerebrovascular (HR 1.22, 95% CI [0.97;1.52]), and peripheral artery disease (HR 1.57, 95% CI [1.15;2.13]). No differences in estimates were identified for gallstones detected at ultrasound or cholecystectomy. Adjustment did not change estimates substantially. Gallstone disease seems to be independently associated to cardiovascular disease. Associations cannot be explained through traditional cardiovascular disease risk factors, apolipoprotein E4 allele, or detection bias. Future studies should explore the link between gallstone and cardiovascular disease further and gut microbiota may be a candidate mechanism.     

From gene to disease; Gaucher disease

Gaucher disease is an autosomal recessive inherited lysosomal storage disorder due to mutations in the glucocerebrosidase gene located on chromosome 1q21. Hepatosplenomegaly and bone disease due to massive accumulation of undegraded glucocerebroside in macrophages found in the liver, spleen and bone marrow dominate the clinical picture in type 1 disease. In rare instances (type 2 and 3 disease) the central nervous system is involved. Phenotype-genotype correlations are poor. Diagnosis is possible by enzyme assay at clinical genetic centres in the Netherlands. The availability of effective therapies emphasizes the need for early recognition of the disease.     

Niemann-pick disease type C and Crohn's disease

A five-year-old girl with Neimann-Pick disease type C subsequently developed Crohn's Disease. This association has only been presented once previously in the literature. This report discusses the options for managing one chronic disease in the presence of another life limiting condition.     

Infectious bursal disease (Gumboro disease)

Infectious bursal disease (IBD) (Gumboro disease) has been described throughout the world, and the socio-economic significance of the disease is considerable world-wide. Various forms of the disease have been described, but typing remains unclear, since antigenic and pathotypic criteria are used indiscriminately, and the true incidence of different types is difficult to determine. Moreover, the infection, when not fatal, leads to a degree of immunosuppression which is often difficult to measure. Finally, the control measures used are subject to variations, and seldom follow a specific or standardised plan. In the context of expanding international trade, the authors provide an overview of existing knowledge on the subject to enhance available information on the epidemiology of IBD, the identification of reliable viral markers for diagnosis, and the implementation of specific control measures to ensure a global and co-ordinated approach to the disease.     

Hunger disease

This paper examines three aspects of hunger disease: the effect of initial fat stores on macronutrient fuel selection during total starvation (no energy) and how it influences survival; the effects of different rates of weight loss on tissue and body function; and the importance of appetite sensations, including hunger, during malnutrition and during enteral and parenteral nutritional support. Long-term starvation studies in humans reveal major differences in fat carbohydrate and protein metabolism between lean and obese subjects, including a 2-4-fold lower contribution of protein oxidation to energy expenditure in obese subjects, which ensures that more of the excess body fat is oxidized. The rate of weight loss, determined by recent dietary intake, can have major effects on tissue and body function, including wound healing, the acute phase protein response, muscle fatigue and psychological/behavioural function in both clinical and non-clinical settings. In depleted states uncomplicated by disease, changes in appetite sensations can result in energy intakes as high as 6000 to 10,000 kcal/day ( 25-42 MJ/day). Long-term enteral tube feeding and parenteral nutrition are associated with frequent disturbances in appetite sensations, and in those able to eat normally they tend to add rather than replace oral intake to an extent that appears to depend on the regimen. It is concluded that 1) differences between lean and obese subjects in macronutrient fuel selection during starvation are adaptive because they optimize survival in both groups of subjects; 2) the rate of weight loss in health and disease has a major effect on certain tissue and body functions, independently of the magnitude of weight loss; and 3) clinically relevant disturbances in appetite sensations are common subjects receiving long-term enteral and parenteral nutrition. The clinical modulation of all these variables would be aided by greater knowledge of the mechanisms involved.