主动脉与肺动脉内径比值及相对位置在检查胎儿先天性心脏病中的价值

目的以主动脉与肺动脉的位置关系及内径的比值为线索来寻找胎儿心脏可能存在的缺陷。方法随机选取出生后心脏发育正常的胎儿497例,妊娠期孕龄16周～35+1周,在三血管切面中观察肺动脉与主动脉的位置关系,测量两者的内径,并计算出比值,采用SPSS13.0统计学软件,计算该比值均数的95%可信区间;回顾性分析2009～2012年检查出的先天性心脏病患者62例,观察肺动脉与主动脉的相对位置并计算出两者内径的比值,找出存在异常的病例。结果主肺动脉与主动脉的内径比值平均值为1.23,标准差为0.118,均数的95%可信区间为1.21～1.24。2009～2012年共确诊胎儿心脏先天性缺陷,且主动脉、肺动脉均有发育者62例,在三血管切面中存在的异常的有48例,占77.4%,其中主动脉与肺动脉位置异常的有6例,分别为右心室双出口1例,大动脉转位3例,气管位置异常2例;主肺动脉与主动脉内径比值<1.21的有23例,分别为法洛四联症17例,右心室发育不良2例,完全性心内膜垫缺损2例,肺动脉瓣狭窄2例;主肺动脉与主动脉的比值>1.24的19例,分别为室间隔缺损9例,左心室发育不良3例,主动脉弓离断3例,主动脉弓缩窄4例。结论主动脉与肺动脉的位置关系及内径比值固定,两者互为参照物,比单纯观察主动脉或肺动脉更容易发现异常,是寻找胎儿心脏畸形的重要线索。     

胎儿主-肺动脉间隔缺损的产前超声诊断

目的:探讨胎儿主-肺动脉间隔缺损的超声声像图特征,提高超声对此病的产前诊断水平.方法:对2010年1月-2013年12月经我院产前超声诊断为胎儿主-肺动脉间隔缺损的5例超声检查资料进行回顾性分析,总结主-肺动脉间隔缺损的超声特征.结果:产前超声诊断为胎儿主-肺动脉间隔缺损的病例共5例,按Richardson分型法,Ⅰ型4例,Ⅱ型1例;缺损大小为0.17～4.0 mm.其中1例合并有室间隔缺损.声像图主要表现在三血管观上可见主动脉与肺动脉之间出现回声中断,范围大小不等,缺损呈窗形或弧形.CDFI在缺损处可探及肺动脉向主动脉分流信号.结论:胎儿主-肺动脉间隔缺损具有特征性声像图,产前超声能够显示主-肺动脉间隔缺损的位置、大小及血流动力学改变等情况,是产前首选的检查方法.     

超声诊断胎儿A型主动脉弓离断并室间隔缺损1例

孕妇,29岁,孕1产0,自述早孕期感冒,未服药,1周自愈.产前常规超声检查发现四腔心观不对称而就诊.超声检查:左房7.9 mm,左室5.7mm,右房14.2 mm,右室11.5 mm,室间隔2.6 mm,左室后壁2.7 mm,主动脉内径3.6 mm,主肺动脉内径6.7 mm.卵圆孔直径5.5 mm,动脉导管内径4.5 mm,胎儿心脏位于胸腔内位置正常,心尖指向左前,心房正位,心室右襻,右心较左心明显大,二、三尖瓣回声及启闭未见明显异常,室间隔上部连续性中断,主、肺动脉连接及位置未见异常,主动脉内径较小,降主动脉于导管前连续性中断,主动脉弓部发出头臂干、左颈总动脉、左锁骨下动脉(图1),降主动脉与动脉导管连接,主肺动脉及动脉导管内径增宽.     

超声诊断双绒毛膜双羊膜囊双胎之一右室双出口1例

孕妇,27岁,孕1产0,停经24周。超声检查:A胎儿未见明显异常。B胎儿心脏解剖结构异常:心房正位,心室右襻,主动脉内径5.5mm,主肺动脉内径3.8mm,右肺动脉内径2.7mm,左肺动脉内径2.6mm,左心室内径9.8mm,右心室内径7.8mm,四腔心可显示,二尖瓣及三尖瓣启闭可见,左室内径稍大于右室内径,室间隔顶端回声中断6.5mm(图1),主动脉前移至右前     

超声心动图诊断右肺动脉起源异常于升主动脉1例

患者男,15岁.因"反复咯血7年"就诊.心脏听诊:胸骨左缘第2肋间可闻及3/6级机器样连续性杂音.超声心动图显示:(1) 左室长轴观可见升主动脉后壁伸出管状无回声(图1),彩色及频谱多普勒显示升主动脉与该管状无回声间见双向血流(图2);(2)大动脉短轴观可见主肺动脉走行无异常,未见主肺动脉发出右肺动脉;(3)右室、左室增大,肺动脉增宽;(4)动脉导管未闭(patent ductus arteriosus,PDA).心导管检查:(1)右肺动脉异常起源于升主动脉和PDA;(2)肺动脉、右室高压.外科手术见:右肺动脉异常起源于升主动脉右后方伴PDA.     

1例矫正性大动脉转位室缺修补术后再行二尖瓣置换术的围术期护理

矫正性大动脉转位（corrected transposition of the great after-ies）又称左型转位,为大动脉转位的一种,是一种少见的先天性心血管畸形,约占先天性心脏病的0．6％-1．4％。本病在解剖上有大动脉转位,即形态学的右心室发出主动脉、左心室发出肺动脉,主动脉位于肺动脉的左前或右前方。     

右冠状动脉异常起源于肺动脉酷似左冠状动脉-右室瘘超声表现2例

正病例1患者男,30岁,心悸半年。超声显示:左冠状动脉起始段内径增宽达9.1mm,前降支与室间隔及心尖肌小梁部丰富的五彩镶嵌血流信号相通,部分血流信号与右室间似可见左向右分流。主动脉右冠窦内未见冠状动脉开口,主肺动脉内侧壁见一宽约5.6mm的管状结构发出,经升主动脉前方向右走行(图1),其内可检出舒张期为主的双期血流信号进入主肺动脉,峰值流速约1.4m/s(图2)。右心超声造     

产前超声诊断肺动脉交叉2例

病例1,28岁,孕3产2,既往体健,无特殊家族史.于妊娠第29周来我院行晚孕期胎儿畸形筛查:胎儿四腔心切面显示十字交叉结构消失,左右肺动脉分叉无法在同一切面上显示,左肺动脉由肺动脉主干右侧发出,右肺动脉由肺动脉主干左侧发出,左肺动脉与右肺动脉不在同一水平,左肺动脉于右肺动脉的上方发出(图1),彩色多普勒显示左右心房、心室血流相互混合.     

超声诊断胎儿主动脉弓离断合并无顶冠状静脉窦综合征1例

##正## 孕妇,30岁。孕26周,以"外院发现胎儿室间隔缺损"为主诉来我院行胎儿心脏超声检查。超声所见:胎儿心胸比例略大,大动脉关系正常,升主动脉内径约2.6 mm,主肺动脉内径约5.5 mm,动脉导管内径约4.9 mm。升主动脉自左室发出后向上方走行,可见其发出两分支,未见确切主动脉弓及其第3分支(图1)。室间隔上部可见约2.8 mm回声中断。超声诊断:胎儿室间隔缺损(膜周部),主动脉缩窄     

超声显像测量胎儿肺动脉与主动脉内径比值的意义

目的：探讨超声显像测量胎儿肺动脉与主动脉内径比值的意义方法应用超声显像对542例孕20~41周的胎儿心脏肺动脉与主动脉内径的比值进行测量。结果心脏发育正常的胎儿肺动脉与主动脉内径比值为（1.15＆#±0.09）。部分先天性心脏病胎儿肺动脉与主动脉内径比值显著异常,产前检查测量胎儿主肺动脉与主动脉内径比,对产前诊断先天性心脏病有临床意义。结论超声显像测量肺动脉与主动脉比值对胎儿先天性心脏病有意义,可作为胎儿心脏筛查的常规测量指标。     

儿童遗传性心律失常的诊治进展

回顾在遗传性心律失常领域最新发表的相关研究,主要关注与儿童心源性猝死关系密切的离子通道病,包括长QT综合征（LQTS）、短QT综合征（SQTS）、Brugada综合征（BrS）和儿茶酚胺敏感性多形性室性心动过速（CPVT）,总结它们在发病机制及诊治方面的进展。     

The acoustic characteristics of the skull

Many investigators have stated that the difficulties of imaging with acoustical energy through the skull result from the marked attenuation of the energy by the skull. In the literature measurements of total attenuation have been confused with those for absorption.Measurements made by us show that absorption by compact bone varies between 2–3 dB cm^?1 MHz^?1 and, in the low megaHertz region appears to be directly proportional to frequency.It has also between shown that the convoluted inner surface of the ivory bone of the inner table of the skull may degrade the collimation and directionality of the beam by refraction.Cancellous bone, such as is present in the dipole of the skull, greatly attenuates the energy. It is postulated that this largely results from scattering. It is also postulated that the energy propagates through cancellous bone as two components, one in the soft tissues and the other partly in the bony spicules. Observations suggest that attenuation due to scattering much more markedly affects the latter of these components and scatters more greatly the higher frequencies in a pulse of broad bandwidth.The energy in each component has varying propagation paths so that the later cycles in the pulse of each component are subject to increasing interference as a result of the variations in propagation times. The two components moreover may have different propagation times so that interference may occur between the pulses of each component as well.All of these phenomena degrade the collimation, coherence, directionality, beam width, pulse length, frequency and other properties of the ultrasonic energy upon which imaging through the skull depends.The interference effects described above are least for the first cycle in the pulse which usually is not the cycle of highest amplitude. Since, in the free field, most of the energy is concentrated around the beam axis, most of the energy in the field which is deflected from its normal propagation path is deflected away from the beam axis. Thus the directionality of the beam is least degraded in the beam axis. The effects of the skull in degrading the properties of the ultrasonic pulse would therefore be lessened if the amplitude of the first cycle of the pulse and the directionality of its energy could be used for imaging.     

Organ Preservation: Current Concepts and New Strategies for the Next Decade

SUMMARY: Organ transplantation has developed over the past 50 years to reach the sophisticated and integrated clinical service of today through several advances in science. One of the most important of these has been the ability to apply organ preservation protocols to deliver donor organs of high quality, via a network of organ exchange to match the most suitable recipient patient to the best available organ, capable of rapid resumption of life-sustaining function in the recipient patient. This has only been possible by amassing a good understanding of the potential effects of hypoxic injury on donated organs, and how to prevent these by applying organ preservation. This review sets out the history of organ preservation, how applications of hypothermia have become central to the process, and what the current status is for the range of solid organs commonly transplanted. The science of organ preservation is constantly being updated with new knowledge and ideas, and the review also discusses what innovations are coming close to clinical reality to meet the growing demands for high quality organs in transplantation over the next few years.     

2017年呼吸疾病主要进展

2017年,国内外学者在呼吸系统疾病的临床和基础领域均进行了深入研究,不仅对相关指南进行了更新,并且针对一些临床热点、难点问题达成专家共识,现就2017年呼吸疾病相关进展作一简单介绍。     

家族性噬血细胞性淋巴组织细胞增生症1例并文献复习

目的加强对家族性噬血细胞性淋巴组织细胞增生症（familially hemophagocytic lymphohistiocytosis,FHL）的认识。方法报道确诊为FHL的新病例1例,结合国内外报道的FHL的病例,对该病的临床特点进行汇总分析。结果FHL2常与PRF1基因突变相关,约20%~40%的患者存在穿孔素基因突变。结论对于有阳性家族史,基因诊断明确,应尽早行化疗或者造血干细胞移植。若无家族史,未发现与继发性HLH相关的原发病因,可考虑行基因筛查以明确是否存在FHL的可能。     

COVID‐19 and COVID‐19 vaccine‐related dermatological reactions: An interesting case series with a narrative review of the potential critical and non‐critical mucocutaneous adverse effects related to virus,therapy, and the vaccination

This narrative review article was conducted to lay out a summarized but exhaustive review of current literature over mucocutaneous manifestations in 4 dimensions of SARS‐CoV‐2 pandemic: virus itself, treatment‐related, vaccine‐induced, and alteration of chronic dermatologic diseases following infection. Virus and vaccine‐related were mainly self‐limited and non‐severe. Treatment‐related reactions could be life‐threatening.     

Burkitt's lymphoma of maxillary gingiva: A case report

Burkitt's lymphoma(BL) is an aggressive form of nonHodgkin's B-cell lymphoma with three variants namely endemic, sporadic, and immunodeficiency-associated types. It is endemic in Africa and sporadic in other parts of the world. While the endemic form is widely reported to occur in early childhood and commonly involves the jaw bones, the sporadic form typically presents as an abdominal mass. This presentation reports a rare case of sporadic form of BL clinically manifesting as a generalized gingival enlargement in an immunocompetent adult male which demonstrated an aggressive behavior. The patient reported with a prominent anterior gingival swelling of 6 mo duration which slowly enlarged in size and associated with multiple lymph node involvement. Microscopic examination of the lesion using H, E and immunohistochemical diagnosis confirmed the diagnosis as BL. The patient succumbed to the disease before any therapy could be instituted. Since a wide array of causes can be attributed to gingival enlargements, it is necessary to consider malignancies as one of the important differential diagnosis so as to facilitate the need for appropriate diagnosis and prompt treatment.     

Real‐life effectiveness of biological therapies on symptoms in severe asthma with comorbid CRSwNP

BackgroundWe aimed to evaluate the effectiveness of different antibody therapies on nasal polyp symptoms in patients treated for severe asthma.MethodsWe performed a retrospective analysis of patients with severe asthma and comorbid CRSwNP who were treated with anti‐IgE, anti‐IL‐5/R or anti‐IL‐4R. CRSwNP symptom burden was evaluated before and after 6 months of therapy.ResultsFifty patients were included hereof treated with anti‐IgE: 9, anti‐IL‐5/R: 26 and anti‐IL‐4R: 15 patients. At baseline median SNOT‐20 was similar among groups (anti‐IgE: 55, anti‐IL‐5/R: 52 and anti‐IL‐4R: 56, p = 0.76), median visual analogue scale (VAS) for nasal symptoms was 4, 7 and 8 (p = 0.14) and VAS for total symptoms was higher in the anti‐IL‐4R group (4, 5 and 8, p = 0.002). After 6 months SNOT‐20 improved significantly in all patient groups with median improvement of anti‐IgE: −8 (p < 0.01), anti‐IL‐5/R: −13 (p < 0.001) and anti‐IL‐4R: −18 (p < 0.001), with larger improvement in the anti‐IL‐4R group than in anti‐IgE (p < 0.001) and anti‐IL‐5/R (p < 0.001) groups. VAS nasal symptoms improved by median anti‐IgE: 0 (n.s.), anti‐IL‐5/R: −1 (p < 0.01) and anti‐IL‐4R: −3 (p < 0.001), VAS total symptoms by anti‐IgE: −1 (n.s.), anti‐IL‐5/R: −2 (p < 0.001) and anti‐IL‐4R: −2 (p < 0.001).ConclusionsTreatment by all antibodies showed effectiveness in reducing symptoms of CRSwNP in patients with severe asthma, with the largest reduction observed in anti‐IL‐4R‐treated patients.     

经颅黑质超声诊断帕金森病的临床评价

目的 通过对帕金森病患者及正常对照组进行经颅超声(transcranial sonography,TCS)检查,结合帕金森病患者的Hoehn Yahr（H Y）分级及帕金森病统一评分量表评分结果,了解我国大陆地区帕金森病患者有无黑质(substantia nigra, SN)回声增强表现。方法 对入选对象进行TCS检查,并对检查结果进行评价。结果 帕金森病患者 SN阳性率明显高于正常对照者（P＜0.05）。帕金森病SN异常组 H Y 分期明显高于帕金森病SN正常组(P＜0.05),表明帕金森病患者SN高回声面积与H Y分期相关。TCS检查对帕金森病的敏感性为80.5%,特异性为79.9%。结论 我国帕金森病患者SN强回声检出率显著高于对照组,说明我国帕金森病患者也存在SN回声增强这一现象,与国内外报道相一致。TCS检查对帕金森病的诊断具有一定的意义,敏感性及特异性较高。