超声在产前诊断胎儿肺动脉闭锁中的应用价值

目的探讨产前超声诊断胎儿肺动脉闭锁的临床价值.方法回顾分析7例经解剖证实的肺动脉闭锁胎儿的超声心动图资料,总结其异常声像图特点.结果7例在二维超声心动图各切面观中仅1例显示主肺动脉显著窄于主动脉,6例未显示主肺动脉;5例显示室间隔缺损伴主动脉骑跨,2例室间隔完整者均合并右室发育不良;彩色多普勒血流显像示5例动脉导管反向血流,3例显示三尖瓣反流.产前超声诊断胎儿肺动脉闭锁5例,诊断右心发育不良1例,误诊永存动脉干1例.结论产前超声检出动脉导管反向血流是诊断胎儿肺动脉闭锁的重要依据,具有重要的临床意义.     

Berry综合征的产前超声心动图及病理解剖诊断研究

目的探讨Berry综合征产前超声心动图及病理解剖异常特征。 方法对2012年8月至2015年10月在广西壮族自治区妇幼保健院产前超声诊断的3例Berry综合征胎儿声像图特征进行分析,结合文献对引产后胎儿标本心脏解剖异常所见与产前超声心动图特点进行对照研究。 结果3例Berry综合征均因三血管气管观（3VT）首先发现主动脉弓异常和主动脉弓缩窄1例,主动脉弓离断2例。三血管肺动脉分支观（3VP）均显示主-肺动脉间隔远端缺损,右肺动脉起源于升主动脉,左肺动脉起源于主肺动脉,在此切面上升主动脉、主肺动脉、左肺动脉及右肺动脉可呈"羊角征"特征性声像改变;彩色多普勒显示主-肺动脉间隔缺损处均可测及主动脉至肺动脉分流信号。2例胎儿产前超声心动图诊断与引产标本心脏解剖病理诊断结果相符合,1例胎儿引产后未行病理解剖检查。 结论Berry综合征在三血管肺动脉分支具有特征性声像表现,三血管肺动脉分支观结合三血管气管观可对Berry综合征进行诊断。     

室间隔完整的肺动脉闭锁的产前超声诊断

目的探讨室间隔完整的肺动脉闭锁的胎儿期超声诊断声像特征、检测技巧及其鉴别诊断,提高此类畸形的产前检出率。 方法对33200例胎儿均采取胎儿心脏四腔心切面加胎儿头侧偏转法获得四腔心切面及左右心室流出道及主、肺动脉长轴切面快速筛查胎儿心脏畸形,对疑有胎儿心脏畸形者则更行详细的胎儿彩色多普勒超声心脏检查。分析8例产前及产后诊断的室间隔完整的肺动脉闭锁的声像特征和病理特征。 结果产前共诊断室间隔完整的肺动脉闭锁8例,其主要声像特征是8例均有肺动脉内径细小,彩色多普勒于三血管气管平面显示7例动脉导管内血流反向,7例右室右房扩大,6例右室壁厚,7例中重度三尖瓣反流,1例三尖瓣下移畸形,1例三尖瓣狭窄并右室小。 结论肺动脉内径细小、不同程度的右房右室扩大、三尖瓣畸形、右室发育不良、动脉导管内血流反向是肺动脉闭锁的主要声像特征,三血管气管平面是产前超声诊断室间隔完整的肺动脉闭锁的关键切面,需注意与主动脉闭锁、永存动脉干进行鉴别。     

时间-空间关联成像联合超声断层显像模式在产前诊断圆锥动脉干畸形中的应用

目的 探讨时间-空间关联成像联合超声断层显像模式(TUI-STIC)在产前诊断圆锥动脉干畸形中的应用价值.方法 分别对1508例先天性心脏病高危胎儿进行二维超声心动图(2DE)检查并采集TUI-STIC心脏容积数据分析,产前检查结果与产后新生儿超声心动图、多排螺旋CT及引产后尸检结果进行对比.结果 1508例胎儿中,2DE诊断圆锥动脉干畸形35例,TUI-STIC诊断圆锥动脉干畸形39例.TUI-STIC补充及纠正2DE诊断9例(共占22％),纠正2DE诊断右室双出口亚型3例,其诊断灵敏度为97.5％,特异度100％,阳性预测值100％,阴性预测值99.9％,准确率99％.2DE与TUI-STIC一致性检验Kappa=0.244,P=0.018 (P＜0.01),McNemar检验显示差异有统计学意义,P =0.004(P＜0.01).结论 TUI-STIC可以提高心室大动脉连接及位置关系的显示率及异常的发现率,有利于大动脉空间定位,对于完善圆锥动脉干畸形的诊断及鉴别诊断具有较大的应用价值.     

超声心动图对复杂胎儿先天性心脏畸形的评价及临床意义

目的 探讨超声心动图对复杂胎儿先天性心脏畸形的评价及临床意义.方法 对产前超声检查发现的复杂胎儿先天性心脏畸形声像图特征行产后尸解和病理验证.结果 超声心动图产前诊断复杂胎儿先天性心脏畸形20例.各类畸形包括:单心室4例,大动脉转位2例,右室双出口2例,永存动脉干2例,左心发育不良综合征3例,心内膜垫缺损3例,右心室发育不良2例,法洛四联症1例,肺动脉闭锁1例.超声产前诊断率100%.结论 超声心动图可以对复杂胎儿先天性心脏畸形进行综合评价,对帮助临床采取相应的决策和产前孕妇及家属知情具有重要意义.     

彩色多普勒超声诊断胎儿左心发育不良综合征

目的探讨彩色多普勒超声诊断胎儿左心发育不良综合征的价值。方法对29436例胎儿进行彩色多普勒超声系统筛查,主要观察心腔大小,二尖瓣和主动脉瓣的活动情况,卵圆孔、室间隔缺损情况,主动脉和肺动脉状况等。结果超声筛查出的15例胎儿左心发育不良综合征中,主要表现为右房、右室明显增大,左心发育不良,二尖瓣狭窄或闭锁,主动脉瓣狭窄或闭锁,二尖瓣或主动脉瓣发育不良等。结论彩色多普勒超声对诊断胎儿左心发育不良综合征具有重要价值。     

胎儿心室发育不良的产前超声心动图诊断价值

目的　探讨产前超声心动图对胎儿心室发育不良的诊断价值。方法　回顾性分析我院 2 6 4 8例行中孕超声筛查和高度怀疑胎儿心脏畸形的晚孕孕妇。首先确定胎儿心脏的位置及心轴 ,然后心脏三节段分析法进行分析。二维超声显示四腔心、左室流出道、右室流出道、心底短轴、主动脉弓、动脉导管弓及三血管切面 ,重点观察二尖瓣、三尖瓣、主动脉瓣和肺动脉瓣的启闭情况 ,再用多普勒超声和 M-型超声观察胎儿心脏各瓣膜的血流情况。结果　 2 6 4 8例行常规超声筛查孕妇中检出先天性心脏畸形 2 1例 ,其中心室发育不良 6例 (3例左心发育不良综合征和 3例右心发育不良 )。 6例心室发育不良均已引产并有 3例尸检 ,尸检结论与超声完全一致。结论　产前超声心动图对检出胎儿心室发育不良及其分型具有极重要的价值。二维超声心动图是根本 ,而多普勒超声和 M-型超声心动图也不可或缺。     

胎儿超声三血管气管观诊断胎儿心脏大血管异常的价值

目的 探讨胎儿三血管气管观对胎儿心脏大血管发育异常的诊断价值.方法 分析产前筛查为胎儿大血管异常的孕妇39例,评价分析其三血管气管观的图像特征及意义.结果 经尸检证实的39例大血管发育异常中,永存动脉干(7例)、大动脉转位(7例)、主动脉缩窄(5例)、法洛四联症(4例)、肺动脉狭窄(3例)、左心发育不良综合征、永存左上腔静脉、肺动脉瓣闭锁合并单心室(各2例)、三尖瓣下移畸形伴肺动脉狭窄、主动脉弓离断、右心发育不良、右位主动脉弓、动脉导管狭窄(各1例)均有不同程度三血管观异常表现.右室双出口(2例)中,仅在1例合并肺动脉狭窄时出现肺动脉内径减小.按照胎儿大血管发育异常中超声三血管气管观表现进行分类,分为大血管排列异常(10例)、大血管内径异常(21例)、大血管数目异常(9例)及大血管内血流方向异常(4例)4类.结论 三血管气管观是四腔观等的有益补充,在胎儿先天性心脏病的产前超声诊断中具有重要价值.     

胎儿右心发育不良综合征超声心动图回顾分析

目的 回顾分析胎儿右心发育不良综合征(HRHS)的产前超声诊断,以提高超声对右心发育不良诊断及转归的认识.方法 回顾分析孕20～37周67例潜在性右心发育不良(LHRH组)及10例HRHS胎儿(HRHS组)的临床资料,分析产前超声测值.结果 HRHS组:超声表现以三尖瓣中、重度狭窄或闭锁多见,多合并肺动脉重度狭窄,并且...     

室间隔完整的肺动脉狭窄和闭锁的产前超声检查分析

例,右室正常大小2例,右室发育不良4例,右室壁肥厚10例.肺动脉干轻度发育不良2例,中度发育不良2例,重度发育不良及闭锁6例.动脉导管出现反向血流5例,三尖瓣反流6例.结论 心尖四腔心不对称和主、肺动脉比例失调是产前超声诊断PS/IVS和PA/IVS的基本线索和条件.动脉导管是否出现反向血流与PS/IVS或PA/IVS胎儿的预后密切相关.     

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。