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.     

产前超声筛查胎儿肺动脉闭锁的应用价值

目的探讨产前超声筛查胎儿肺动脉闭锁的应用价值。方法回顾分析12例经解剖证实的肺动脉闭锁胎儿的心脏超声资料，总结其声图像特征。结果在12例胎儿心脏超声各切面中：10例显示主肺动脉显著窄于主动脉，2例未显示主肺动脉；7例显示室间隔缺损伴主动脉骑跨，3例室间隔完整者均合并右室发育不良；11例心底部大动脉交叉存在，1例为右心室双出口；彩色多普勒血流显像示11例动脉导管内均可见反向血流，5例可见三尖瓣反流。结论应用彩色多普勒血流显像（CDFI）检出动脉导管内反向血流是诊断胎儿肺动脉闭锁的重要依据；三血管-气管（3VT）扫查平面是关键切面；产前超声筛查具有重要的临床意义。     

胎儿主动脉弓离断的超声诊断及畸形特征分析

目的 研究胎儿主动脉弓离断的产前超声诊断、鉴别诊断技巧及其畸形特征.方法 所有胎儿均常规获得四腔心切面、左右心室流出道切面、三血管气管切面(3VT)的灰阶和彩色多普勒血流显像.当发现左心和主动脉内径异常小,左/右心室比例和/或升主动脉内径/肺动脉内径比例失调怀疑主动脉弓异常时,进一步获得主动脉弓纵切面和冠状切面明确诊断.结果 产前超声共诊断12例主动脉弓离断,均表现为升主动脉内径相对细小,升主动脉/肺动脉内径比例失调;均合并室间隔缺损.通过解剖证实4例为Bb型主动脉弓离断合并胸腺缺如,2例为A型主动脉弓离断,3例为严重主动脉弓缩窄,3例失访.结论 主动脉内径相对细小,升主动脉内径/肺动脉内径比例失调及室间隔缺损等声像特征是产前诊断主动脉弓离断的重要线索,严重主动脉弓缩窄易误诊为主动脉弓离断,Bb型主动脉弓离断常合并胸腺缺如.     

先天性肺动脉闭锁的产前超声诊断

目的探讨先天性肺动脉闭锁的胎儿期超声图像特征、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行更详细的胎儿彩色多普勒超声心脏检查。分析22例产前及产后诊断的肺动脉闭锁的声像特征和病理特征。结果产前共诊断肺动脉闭锁20例,误诊为永存动脉干2例,根据产前和产后声像图及病理资料将其分为3型:I型,肺动脉闭锁不伴室间隔及不同程度的右室发育不良和三尖瓣异常;Ⅱ型,法洛四联症并肺动脉闭锁,即肺动脉闭锁伴室间隔缺损和主动脉骑跨;Ⅲ型,肺动脉闭锁合并其他大血管异常。主要声像图表现为:肺动脉内径细小、绝大部分病例于三血管-气管平面彩色多普勒显示动脉导管内血流反向。结论肺动脉内径细小,主动脉相对粗大,三血管-气管平面显示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-气管平面是诊断先天性肺动脉闭锁的关键切面,需注意与永存动脉干和主动脉闭锁进行鉴别。     

产前超声诊断胎儿主动脉缩窄

目的 探讨产前超声诊断胎儿主动脉缩窄的临床价值.方法 回顾分析10例经尸体解剖证实的主动脉缩窄胎儿的超声心动图资料,总结其异常声像图特点.结果 10例胎儿主动脉缩窄中,7例主动脉弓失去正常柔和的弯曲状,呈细窄僵直状,2例足月胎儿主动脉峡部直径小于2.6mm,1例主动脉弓部血管显示不清;10例均显示右心房室增大,肺动脉及动脉导管增宽,4例动脉导管呈瘤样扩张,3例伴左心发育不良,4例伴室间隔缺损,2例合并右室双出口,1例合并二尖瓣闭锁;产前超声诊断胎儿主动脉缩窄9例,误诊主动脉弓离断1例.结论 产前超声检查对诊断胎儿主动脉缩窄,具有重要的临床价值.     

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。     

胎儿期肺动脉闭锁合并室间隔缺损超声心动图的特征

【】目的 探讨胎儿期肺动脉闭锁合并室间隔缺损的超声心动图特征。方法 回顾性分析并总结产前超声心动图可疑肺动脉闭锁合并室间隔缺损并引产,尸体解剖证实的资料。结果 胎儿期肺动脉闭锁合并室间隔缺损的超声表现(1)直接征象：二维图像显示主肺动脉内径细窄或未能探及,彩色多普勒探及主肺动脉内由动脉导管逆灌的血流信号或者未能探及肺动脉内动脉导管逆灌血流信号;室间隔缺损;(2)间接征象：主动脉增宽骑跨、降主动脉周围探及体-肺动脉侧支形成。结论 产前超声心动图对胎儿肺动脉闭锁合并室间隔缺损具有较高的诊断准确率。     

胎儿动脉导管异常的超声心动图表现

目的 总结胎儿动脉导管异常的声像图特征.方法 对8例经引产后尸体解剖及产后超声随访证实为动脉导管异常的胎儿产前超声心动图检查资料进行回顾性分析.结果 8例动脉导管异常胎儿中3例产前超声多切面扫查均不能显示动脉导管的管腔结构和血流,2例伴发法洛四联症,1例伴发永存动脉干,提示动脉导管缺如;2例胎儿动脉导管和主动脉弓血流方向相反,主动脉血流经动脉导管逆行灌注于肺动脉主干或左、右肺动脉,其中1例伴发于室间隔缺损型肺动脉闭锁,另1例伴发于室间隔完整型肺动脉闭锁,合并右心室发育不良,提示动脉导管逆行灌注;5例伴有复杂先天性心脏病的胎儿引产后经尸体解剖证实.2例胎儿动脉导管管腔细窄,血流速度增快,提示动脉导管狭窄;1例胎儿动脉导管管腔内充填实性低回声,内无血流信号,提示动脉导管早闭;2例动脉导管狭窄和1例动脉导管早闭的胎儿均表现右心房、右心室增大,伴有三尖瓣重度反流,并经产后超声心动图随访证实.结论 胎儿超声心动图能显示胎儿动脉导管缺如、逆行灌注、狭窄及早闭的异常声像图特征,在胎儿动脉导管异常的诊断中具有重要的临床价值.     

3VT切面上主动脉闭锁及肺动脉闭锁的超声诊断与鉴别诊断价值

目的:研究分析胎儿主动脉闭锁和肺动脉闭锁超声诊断使用3VT切面的价值,为临床诊断提供参考。方法:为6例主动脉闭锁和9例肺动脉闭锁病例使用3VT切面超声诊断,根据超声图情况来进行判断分析特点,对其临床价值进行分析。结果:主动脉闭锁的6例患者,可以观察到主动脉管腔变小,肺动脉管腔变大,经彩超诊断后,主动脉和左心室没有血流联通。使用3VT切面超声检查显示动脉导管弓、主动脉弓、上腔静脉三条血管的大小比例不平衡,主动脉弓有逆向血流情况存在,肺动脉为正向血流。肺动脉闭锁的9例患者有室间隔缺损情况,有右向左分流信号。肺主动脉的起始段模糊,有细小回声,主动脉壁和室间隔的回声间断,主动脉在缺损间隔上,彩超显示肺动脉和右心室没有血流联通。肺动脉闭锁患者的3VT切面图显示主动脉管径增加,肺动脉管径变小,动脉导管弓、主动脉弓、上腔静脉三条血管比例消失,彩超为肺动脉无前向血流,主动脉无逆向血流。结论:胎儿心脏大血管畸形可以使用3VT切面超声检查进行诊断,对主动脉闭锁、肺动脉闭锁进行超声诊断的价值非常高,实际使用的时候,要对系统的安全性,全面性进行观察分析,提供正确的诊断。     

超声血管增强技术联合断层超声显像技术在胎儿先天性心脏病诊断中的应用

目的探讨超声血管增强技术(VET)联合断层超声显像技术(TUI)在胎儿先天性心脏病诊断中的应用价值.方法应用VET-TUI技术对二维超声筛查诊断为先天性心脏病的17例孕22~30周中晚孕期胎儿进行检查,在获取满意的胎儿心脏容积数据后通过TUI模式,对胎儿心脏进行断层分析,并与产后临床检查结果进行对照分析.结果产前应用VET-TUI技术17例胎儿均获得满意的心脏容积数据及胎儿心脏断层成像图像,17例先天性心脏病胎儿中单一性先天性心脏病3例,复合性先天性心脏病14例.17例胎儿心脏畸形包括心内膜垫缺损5例、永存左位上腔静脉7例(其中1例下腔静脉离断合并异常连接)、右心室双出口4例(包括左侧心室解剖右心室双出口)、肺动脉狭窄6例、室间隔缺损8例、主动脉弓缩窄2例、大动脉转位1例、主动脉闭锁2例、右位主动脉弓3例、左心室发育不良2例、右心室发育不良1例、单心室1例、三房心1例、镜面右位心1例.产前超声诊断与引产后尸检结果相符合10例(10/17),与产后超声心动图复查结果相符合5例(5/17),误诊2例(1例超声诊断永存动脉干,尸检证实为升主动脉闭锁,1例超声诊断单心室,尸检证实为左心室发育不良).结论应用VET-TUI技术可增加超声图像的清晰度,使胎儿心脏与大血管容积图像清晰直观,易于辨别,较常规二维超声心动图能提供更多胎儿心脏细微解剖结构的诊断信息.     

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.     

Recalcitrant giant orf recurrence after amputation: A case report and review of the literature

Orf is caused by a parapoxvirus. We present a recurrent, giant digital orf case in a female patient with a history of hairy cell leukemia. In spite of shave excision, the lesion progressed and recurred after digital amputation. Treatment with topical imiquimod cream and systemic subcutaneous interferon alfa‐2a was successful.