超声诊断三尖瓣闭锁1例

患儿女,双胎之一,出生3天,因紫绀、听诊心脏杂音接受心脏彩色多普勒超声检查。心脏彩色多普勒超声:左心房、左心室增大(横径分别约18.0 mm、21.2 mm),右心室偏小(横径约10.1 mm),右心室、右心房连接处未见正常瓣膜回声,见较厚肌性组织回声(图1A),回声较强,未见瓣膜开放及关闭活动;房间隔及室间隔均可见回声连续中断,仅心房顶部见长约3 mm房间隔结构;主动脉起自左心室,肺动脉起自右心室,肺动脉瓣膜增厚,回声增强,动脉导管未闭。CDFI:房间隔见右向左分流信号,室间隔近十字交叉处见左向右分流信号(图1B),肺动脉瓣口测得高速血流信号,肺动脉与主动脉之间见左向右分流信号。超声诊断:三尖瓣闭锁合并室间隔缺损(膜周部)、房间隔缺损(继发孔型)、肺动脉瓣轻度狭窄、动脉导管未闭。患儿后转入上级医院接受手术治疗。     

超声诊断先天性三尖瓣闭锁1例

患儿女,8个月.以早发紫绀、活动后气急入院.查体:神志清,心前区隆起,心尖搏动强,胸骨左缘3、4肋间闻及Ⅲ级收缩期杂音,粗糙,传导广泛.心电图诊断:电轴左偏,左心室肥大.X线诊断:左房、左室增大,肺血及肺纹理减少.超声所见:左房(2.1cm)及左室(3.1cm)扩大,右室(0.7cm)明显减小.主肺动脉内径(1.1cm)正常,室壁厚度正常.心尖四腔心切面示心脏十字交叉结构存在,三尖瓣正常结构消失,右房室间无直接交通,仅见一纤维条带样强回声结构,随心室收缩舒张轻微活动(图1、右图).房间隔中间部位回声中断0.65cm,室间隔膜周部回声中断0.60cm.CDFI;右房室间无血流通过,房间隔水平右向左分流血流,室间隔水平左向右分流血流(图1、左图).连续波多普勒测室间隔水平分流血流速度4.32m/s.     

超声心动图诊断右肺动脉起源于升主动脉一例

患者女,53岁,因“动脉导管未闭结扎术后30余年,间断咯血10余年并进行性加重”来我院就诊,患者33年前因心脏杂音诊断动脉导管未闭,并经左腋下切口行动脉导管未闭结扎术,10余年前开始出现间断咯血,近期加重,并出现活动后胸闷、憋气及口唇发绀等表现。查体：血压130/85 mm Hg（1 mm Hg=0.133 kPa）,心率89次/min,律齐。听诊胸骨左缘2~3肋间可闻及2/6级收缩期杂音。超声心动图检查示：（1）左心房、左心室扩大,右心房、右心室大小正常;（2）大动脉短轴切面观可见主动脉与肺动脉主干位置正常,内径正常,肺动脉主干无分叉结构,仅向左侧延续发出一支肺动脉,未探及右侧肺动脉分支,于升主动脉近端左后方发出右肺动脉,右肺动脉扩张（图1）;（3）彩色多普勒血流成像显示右肺动脉开口处见由升主动脉至右肺动脉的低速连续性分流血流信号（图2）并见少量由右肺动脉至升主动脉的分流血流信号;（4）动脉导管未闭结扎处未见残余分流血流信号。心导管造影检查示：右肺动脉起源于升主动脉。     

超声诊断感染性心内膜炎合并动脉导管开口、肺动脉赘生物一例

患者女,28岁,以"反复发热4个月,咳嗽、咳痰半个月"入院,同时伴有腹胀、四肢乏力、视物黑曚等.入院查体:体温38.6℃,心率112次/min,呼吸 22次/min,血压113/60 mm Hg(1 mm Hg=0.133 kPa).神志清,贫血面容.口唇明显苍白,胸骨左缘闻及3/6级收缩及舒张期杂音.入院后腹水培养为草绿色链球菌感染,直接Coombs实验弱阳性.超声心动图检查示:左肺动脉与降主动脉之间见连续性分流,分流束直径7 mm,主肺动脉管内、导管开口处可见大量泪滴样回声,考虑赘生物可能,最大直径约23 mm.赘生物随心脏收缩、舒张而摆动;左心房、左心室扩大(图1,2).CDFI示:动脉导管双期分流,左向右,流速4m/s,压差(主动脉与肺动脉之间)64 mm Hg,二尖瓣重度反流,三尖瓣轻度反流,反流峰压差约64 mm Hg,估测肺动脉收缩压约70 mm Hg.超声心动图提示:(1)先天性心脏病,动脉导管未闭;(2)二尖瓣、主肺动脉、导管开口处赘生物形成;(3)二尖瓣重度反流;(4)左心房左心室扩大;(5)肺动脉高压.患者转往上级医院,对症抗感染治疗,病情稳定后,手术彻底清除瓣膜及肺动脉腔内赘生物后行二尖瓣置换术.并于术中缝闭动脉导管内口.术后复查二尖瓣无明显反流,动脉导管无残余分流,手术效果良好.     

超声诊断动脉导管未闭并主动脉瓣二叶畸形漏诊右肺动脉异常起源1例

患儿男,4岁,因反复咳嗽、气促1月入院。超声心动图检查：左心室长轴观示左心增大,主动脉内径约28mm,主动脉根部后壁结构稍紊乱,彩色多普勒血流显像示主动脉起始部漩流。大动脉短轴观示主动脉瓣为右上、左下二叶,回声增强;肺动脉内径稍增宽,肺动脉瓣前移;肺动脉分叉似存在;主肺动脉与降主动脉之间见管状结构,长15mm,内径9mm（图1）。彩色多普勒血流显像示该异常管腔内血流信号呈双期双向,色彩黯淡,左向右分流时限短;血流频谱失常,类似层流,左向右分流Vmax1.1m/s,右向左分流Vmax0.97m/s（图2）。心尖四腔观示收缩期三尖瓣微量反流,流速2.9m/s,     

实时三维超声心动图诊断房间隔缺损伴三房心1例

患者男,31岁,以心脏杂音2个月入院。体格检查:于胸骨左缘第二、三肋间可闻及2/6级收缩期吹风样杂音。常规二维心脏超声检查(图1):房间隔中部中断34 mm,多普勒探及左向右分流,分流峰速0.8 m/s,右心增大(右心室内径:36 mm,右心房面积:61 mm×53 mm),肺动脉内径增宽(32 mm)。左心房内可见隔膜回声,将左心房分为真房、副房,肺静脉均回流     

超声心动图诊断原因不明慢性肺动脉栓塞1例

病例男,42岁,因"咳嗽、胸闷、活动后口唇紫绀4月"入院就诊,超声心动图所见:右心增大,左肺动脉根部见2.0 cm×0.8 cm、右肺动脉根部见1.8 cm×1.3 cm稍高回声团,致左肺动脉血流束变细,右肺动脉无明显血流充盈。估测肺动脉收缩压约85 mmHg(1mmHg=0.133kPa)。超声提示:①左、右     

右肺动脉异位连接于升主动脉伴动脉导管未闭1例

患者男,19岁,以活动后心悸、气短一月余就诊。查体:一般情况好,呼吸、心率、血压正常。心界向左下扩大,胸骨左缘1～2肋间闻及连续性杂音,Ｐ2亢进、分裂,周围血管征阴性,临床诊断:先天性心脏病,动脉导管未闭。ＥＣＧ:窦性心率,电轴轻度右偏,不完全右束支传导阻滞。胸片:双肺多血,心影大,肺动脉段略凸,主动脉结稍大,诊断为先心病左向右分流,或为导管轻度肺动脉高压。二维超声心动图检查示左心扩大,肺动脉增宽(31ｍｍ),降主动脉与左肺动脉间见一导管相通,直径7～9ｍｍ,长度10ｍｍ;彩色血流示导管左向右五彩分流束;频谱多普勒录得左向右双期分流…     

超声诊断右心发育不良综合征1例

患儿男,55天,以"生后颜面、口唇、指趾青紫,近两日加重伴呼吸困难"就诊.体格检查:一般状况差,面色灰暗,全身皮肤黏膜发绀.三凹征(+).心率:110次/分.胸骨左缘第2肋间闻及3～4级收缩期杂音.胸部X线示心影略大.超声心动图:内脏位置正常,内脏心房连接正常;房间隔中部回声失落约6～8 mm,房水平探及右向左为主双向分流信号(图1);可见左右房室环及十字交叉结构,右侧房室间未见正常瓣膜样结构,代之以厚约2 mm强回声结构(图2),中央可见约1 cm2较薄的膜性结构,随心动周期膨向右心室或右心房,未探及右侧房室间血流沟通.左侧房室瓣为二叶结构,开关良好.右心室腔较小,几何形变,心尖部近乎闭塞,室间隔上部呈弧形膨向左心室侧(图2).室间隔连续完整,未探及分流.肺动脉瓣口未显示正常瓣膜结构(图3),瓣环内径明显减小,约4 mm,未见跨肺动脉瓣口血流.肺动脉主干较细,内径约3～4 mm,其内可见来自动脉导管的全心动周期血流(图3),血流束宽约2～3 mm,峰速约3.5～4.5 m/s.主动脉弓左降,结构及血流未见异常.超声提示:①右心发育不良综合征(右室发育不良、三尖瓣闭锁、肺动脉瓣闭锁);②房间隔缺损(继发孔型),房水平右向左为主双向分流;③动脉导管未闭,大动脉水平左向右分流.     

超声诊断室间隔完整型肺动脉闭锁1例

患儿女,6个月,2011年9月因口唇发紫、食欲不振、吃奶量少、活动后呼吸费力来我院就诊.查体:体格发育正常,口周及指端发绀,面色发青,心前区隆起,胸骨左缘2、3肋间闻及Ⅲ级全收缩期舒张期杂音,腹软,肝脾无肿大.门诊心脏超声检查:①右房增大,右心室壁增厚,右心室缩小(图1);②室间隔回声连续,房间隔可见回声中断约7 mm,彩色多普勒血流显像(CDFI)示该处左向右分流;③主动脉内径增宽,右室流出道变窄,肺动脉瓣瓣膜回声增强,收缩期未见开放,CDFI示瓣口未见血流通过,肺动脉主干内径约7.5 mm;④三尖瓣较小,瓣口可见少许血流通过,瓣口可测及轻度反流;⑤右冠状动脉扩张,内径约5 mm,与右室窦隙相通,瘘口约3.5 mm,脉冲多普勒(PW)测及双期双向血流,CDFI示右室逆行灌注右侧冠状动脉,呈蓝色血流信号(图2);⑥降主动脉与左肺动脉起始部之间测及一管状无回声区,内径约2 mm,CDFI示该处左至右分流,PW在肺动脉主干内测及收缩和舒张期分流频谱.超声提示:先天性心脏病——室间隔完整型肺动脉闭锁,三尖瓣发育不良,右室发育不良型(Ⅰ型),右冠状动脉-右室窦隙形成,房间隔缺损(Ⅱ孔型),动脉导管未闭.患儿在全麻下行上腔静脉-右肺动脉吻合术(GLEEN),术后诊断:先无性心脏病——肺动脉闭锁(室间隔完整型)、右侧冠状动脉右室窦隙形成、三尖瓣、右室发育不良、房间隔缺损(Ⅱ孔型)、动脉导管未闭,与术前超声诊断基本一致.     

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.     

动态心电图临床操作标准化方法

动态心电图,又称Holter或Holter检查,是一种评价各种心脏病患者心电图异常的简便、高效、准确、安全的无创检查,广泛用于心律失常的相关症状评价,心肌缺血的诊断,心脏病患者的预后和日常生活能力评估,药物疗效评价,起搏器等埋藏式心脏电治疗装置监测等领域。目前动态心电图已广泛用于于临床各级医疗机构,为了更好地发挥其作用,有必要对该项技术进行规范化培训。本文参考相关指南、共识及专家建议,结合作者经验,撰写动态心电图临床操作标准化方法供临床使用时参考。