读片窗及答案

病例女,11岁,左面部渐进性弥漫性隆起3年,无面部麻木.CT检查:左侧上颌骨局部骨质膨大,正常骨结构消失,呈磨玻璃样稍高密度影,大小约2.7cm×3.5cm,周围骨皮质变薄,向下包绕上颌骨牙齿,向前面部突出,向后上颌窦窦腔变窄.余骨质形态密度未见异常.软组织未见明显肿胀(图1,2).     

巨大蛛网膜颗粒1例

患者女,33岁.无意间发现右头顶部包块就诊.CT轴扫:右顶骨右见一2.0 cm×1.7 cm大小似椭圆形骨质缺损区(图1),边缘清晰、锐利,周围轻度硬化,穿破颅骨内板及板障,外板仅骨皮相连,可见部分脑组织深入其内,局部软组织有隆起、层次清晰;改用冠状强化扫描,见右顶骨正中偏右见一约1.8 cm×0.5 cm大小之骨质凹陷区(图2A),到达骨外板内缘,外板骨皮菲薄,边缘光整、规则呈椭圆形,并可见部分脑组织深入其内,局部骨皮稍隆起,软组织无肿胀,层次清楚;强化未见骨缺损区增强,深入其内脑组织表面见血管影(图2B).CT诊断:巨大蛛网膜颗粒压迹.     

婴儿多部位骨皮质增生症1例

患儿男,3个月,无明显诱因右肩部活动障碍.体检:右肩部可见大小约5 cm×5 cm的包块,质软,界欠清,局部皮肤无发红,皮温不高,压之患儿哭闹.右肩活动较左侧少,右手活动好,桡动脉可及.实验室检查:碱性磷酸酶399 U/L,白细胞8.70×109/L.X线检查:右肩胛骨形态不规则膨大,密度不均匀,其内见斑点状高密度影,关节盂下缘见丘状、簇状堆积排列的团状骨膜增生影,肩胛骨骨质结构不完整,见低密度凹陷性骨质缺损,邻近软组织肿胀,无明显包块;同侧第6～8肋骨膨大,均见层状骨膜反应,且与正常骨质间存在线形透亮带(图1).X线诊断:婴儿骨皮质增生症.行两次肩胛骨部位穿刺活检术,病理结果:纤维黏液样组织;少许皮质骨组织,未见异常细胞.     

右足跗骨结核1例

患者男,80岁,主因"右侧足面持续肿胀2年外侧窦道形成"入院.查体:右足背软组织肿胀,远行时疼痛加剧,经窭道分泌腥臭黄白色脓性物.右踝关节正侧位X线摄片:右足第2～5跖骨基底部、楔骨、舟骨、距骨及骰骨和部分跟骨骨质破坏,骨关节间隙显示不清,足背软组织层次混浊、肿胀;右踝后方血管壁钙化,跟骨骨刺生成,跟腱韧带见条形钙化(图1).X线诊断:①右跗骨感染性病变;②右侧跟骨下棘骨质增生;③右侧腓动脉末端及跟腱韧带钙化.CT:右足广泛骨质稀疏,舟骨、骰骨、楔骨及邻近跟骨、距骨、跖骨基底部骨质广泛破坏,部分碎骨片外移;右侧附骨间关节、距跟舟关节、跗跖关节紊乱,关节间隙不规则变窄,关节面骨质弥漫性虫蚀样破坏;邻近跗跖部周围软组织肿胀,脂肪间隙消失(图2).CT诊断:右跗骨、跖骨及对应关节结核改变.病理诊断:跗骨结核.     

婴儿股骨尤因肉瘤膨胀性改变1例

患儿为8个月女婴,体质量约8 kg,因"发现右侧股骨渐进性异常增粗2个月"就诊.X线检查示右侧股骨明显增粗,骨皮质菲薄、欠连续,骨小梁消失,其内可见片状高密度影,骨骺密度减低,关节间隙正常,周围软组织层次不清(图 1A),CT:右侧股骨膨大,略显弯曲,骨皮质菲薄、欠连续,正常骨质结构消失,代之以密度不均匀的软组织密度影,其内可见多发斑片状钙化灶;骨骺可见类似改变,骨小梁消失,皮质尚连续(图1B、C).影像学诊断:右侧股骨占位性病变,良性中瘤可能性大.行有股骨肿瘤切除术,术中见肿瘤质地柔软,有包膜,切开包膜后出血为暗红色,约20 ml.术后病理诊断为尤因肉瘤(Ewing's Sarcoma,ES).     

股骨骨化性纤维瘤1例

患者男,20岁,武警战士,右膝关节疼痛半年余,近期加重.查体:右膝关节活动正常,股骨下端局部未触及包块,皮温不高,表面无曲张的血管,稍压痛.X线平片:右股骨下端内侧、偏前方可见偏心性、多发肥皂泡样低密度区,最大者约2.0 cm×1.0cm,病变内缘骨质硬化增厚,外缘骨皮质变薄,轻度膨胀,未见中断(图1).CT检查:右股骨下端骨皮质下见一约3.0 cm×1.8cm大小的骨质缺损区,内见分隔及点状高密度影,边缘硬化,髓腔侧明显,未见骨膜反应及软组织肿块(图2).诊断:右股骨下端骨肿瘤,骨化性纤维瘤.术后病理:右股骨骨化性纤维瘤.     

双侧胫骨对称性毛细血管型骨血管瘤1例

患儿男,11岁.双侧胫骨前下段肿块2年.2年前发现双小腿胫前肿块,逐渐增大,伴疼痛.查体:双侧小腿前方中段可扪及约2 cm×2 cm质硬肿块,轻压痛.X线检查:双侧胫骨前缘骨皮质增厚,中段增厚骨皮质内见类三角形低密度区,右侧一个,大小约6 mm×10 mm×12 mm,左侧两个,大小分别3 mm×5 m×6 mm、5 mm×8 mm×10 mm,边缘轻度硬化,内见点、横条状致密影,局部髓腔稍变窄,周围软组织未见明显肿胀及静脉石(图1～3).     

多层螺旋CT诊断腮裂癌1例

患者男,43岁,半年前发现"颈部肿物",无明显不适。入院查体于颈左侧下颌角后方可触及肿物,质硬、固定,无压痛。CDFI示左侧颈动脉前方椭圆形不均质回声包块,边界清晰、形态规则,其内可见丰富血流信号,左侧颈部多发淋巴结影。64层螺旋CT平扫显示左侧颈静脉外侧软组织团块影,约3.84cm×2.52cm×4.43cm,密度不均匀,其内见多发囊状低密度影,周围多个淋巴结肿大(图);增强     

先天性早发型骨梅毒跟骨受累1例

患儿男,3个月,手脚肿胀1个月.化验检查:血常规见单核细胞、淋巴细胞升高,血沉130 mm/h,C反应蛋白14 847 μg/ L.快速血浆反应素环状卡片实验(RPR)阳性,滴度为1∶32,梅毒螺旋体被动颗粒凝集试验(TPPA)阳性.其父母:快速血浆反应素环状卡片实验(RPR)阳性,滴度为1∶4,TPPA阳性.患儿X线检查:双侧股骨、胫骨干骺端浸润性骨质破坏和平行状骨膜增生(图1),1～5掌骨、1～5指的1、2节指骨、1～5蹠骨和近节趾骨、跟骨双侧对称性的浸润性骨质破坏,伴软组织肿胀,但第3蹠骨干骺端破坏两侧不对称(图2).周围无骨质增生硬化.     

右耻骨血管周上皮样细胞肿瘤伴双肺转移1例

<正>患者女,38岁,右髋部无明显诱因疼痛肿胀3月余;既往无特殊病史。查体:右髋巨大肿物,触痛、压痛不明显,皮温稍高;右下肢肿胀。实验室检查:红细胞2.43×10¹²/L,血红蛋白68g/L,神经元特异性烯醇化酶236.80μg/L。超声:盆壁右侧至盆腔内23cm×21cm×14cm不规则团块状异常回声,内见不均匀低回声伴强回声,包绕耻骨使其连续性中断;CDFI于肿块内见丰富血流信号(图1A)。盆部CT:右耻骨17.5cm×16.1cm×24.5cm肿块突向盆腔及右侧大腿内侧间隙,内见点、片状瘤骨或钙化,耻骨上支呈溶蚀状骨质破坏(图1B),右髋部受累,右大腿软组织肿胀;     

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。