1H-MRS评价新生儿缺氧缺血性脑病

目的 探讨¹H-MRS评价新生儿缺氧缺血性脑病（HIE）的价值。方法 收集经临床确诊的足月HIE患儿30例（HIE组）和无神经系统异常新生儿16例（对照组）。于出生后22天内行头颅¹H-MRS扫描,分别计算胆碱/肌酸（Cho/Cr）、N-乙酰天门冬氨酸/肌酸（NAA/Cr）、N-乙酰天门冬氨酸/胆碱（NAA/Cho）、乳酸/肌酸（Lac/Cr）比值,并进行统计学分析。结果 与对照组比较,HIE组Lac/Cr升高（P<0.05）,NAA/Cr,NAA/Cho均降低（P均<0.05）,Cho/Cr差异无统计学意义（P>0.05）。多因素Logistic回归分析结果示NAA/Cr、Lac/Cr是HIE的独立危险因素（P均<0.05）。结论 ¹H-MRS可客观反映HIE患儿缺氧缺血后代谢变化,更好地评价脑损伤的严重程度,有助于HIE的早期诊断及治疗。     

不同回波时间氢质子磁共振波谱多体素技术检出多发性硬化病灶和看似正常脑白质区

目的 探讨二维氢质子磁共振波谱(2D ¹H-MRS)中多体素长、短两种不同回波时间(TE)在检出多发性硬化(MS)各种病灶和看似正常脑白质(NAWM)方面的价值。 方法 对50例复发-缓解型MS(RRMS)患者(MS组)和20名健康志愿者(对照组)行常规MR扫描,并采用多体素长、短TE ¹H-MRS分析,测定各感兴趣区代谢物氮-乙酰天门冬氨酸(NAA)、胆碱(Cho)、肌酸(Cr)、脂质(Lip)、乳酸(Lac)和肌醇(mI)的浓度,分析NAA/Cr、Cho/Cr、NAA/Cho、(Lac+Lip)/Cr和mI/Cr在病灶和NAWM区中的变化。 结果 多体素长TE显示NAA/Cr、Cho/Cr、NAA/Cho在非强化病灶、强化病灶与对照组相应脑白质(WM)区比较差异均有统计学意义(P<0.01),NAA/Cr、NAA/Cho在非强化病灶、强化病灶间差异无统计学意义(P>0.01)。多体素短TE显示MS组NAA/Cr、Cho/Cr和(Lac+Lip)/Cr在强化病灶、非强化病灶、病灶对侧NAWM区与对照组相应WM区比较差异均有统计学意义(P<0.01)。比较多体素长、短TE检测NAA/Cr和Cho/Cr无明显差别,但短TE能检测到病灶内的Lip、Lac及mI峰。 结论 多体素长、短TE均能检测MS患者病灶及NAWM区的代谢异常,长TE能得到主要代谢物的波形,短TE能检测到更多代谢物,二者均可为MS的病理研究提供重要信息。     

足月缺氧缺血性脑病患儿1H-MRS与新生儿神经行为测定评分的相关性

目的 探讨足月缺氧缺血性脑病（HIE）患儿¹H-MRS与NBNA评分的相关性。方法 纳入足月HIE患儿101例,29例日龄≤3天、33例日龄4~7天、39例日龄8~14天;另选取同期足月健康新生儿37名作为正常对照。行常规MR及¹H-MRS检查,根据MRI表现对HIE进行分级,比较不同程度HIE患儿和正常新生儿N-乙酰天门冬氨酸（NAA）/胆碱复合物（Cho）、NAA/肌酸（Cr）及Cho/Cr差异;对HIE患儿行新生儿行为神经测定（NBNA）评分,比较不同程度HIE患儿NBNA评分差异;评价¹H-MRS指标与NBNA评分的相关性。结果 ≤3天重度HIE患儿,4~7天和8~14天中、重度患儿基底节NAA/Cho低于相应日龄正常新生儿（P均<0.05）,所有日龄中、重度HIE患儿基底节NAA/Cr和额叶NAA/Cho均低于相应日龄正常新生儿（P均<0.05）;≤3天和8~14天不同程度HIE患儿间基底节NAA/Cho、4~7天不同程度HIE患儿间额叶NAA/Cho差异有统计学意义（P均<0.05）。不同日龄重度HIE患儿NBNA评分均低于中度HIE患儿（P均<0.05）。HIE患儿基底节NAA/Cho和NAA/Cr、额叶NAA/Cho与NBNA评分呈正相关（r=0.238,0.221,0.202;P均<0.05）。结论 HIE患儿¹H-MRS与NBNA评分具有相关性,二者结合有助于早期检出足月儿HIE及评价脑损伤严重程度。     

慢性肝硬化患者神经认知损害与背侧丘脑代谢改变的相关性

目的 分析慢性乙型肝炎肝硬化者认知功能改变与背侧丘脑¹H MRS改变的相关性。方法 收集慢性乙型肝炎肝硬化患者28例（肝硬化组）和同期健康体检者28名（对照组）,分别进行神经心理学测试数字连接试验A （NCT-A）、数字符号试验（DST）和MRS检查;计算各代谢物,包括N-乙酰天门冬氨酸（NAA）、胆碱（Cho）、谷氨酰胺复合物（Glx）和肌醇（mI）的峰下面积与肌酸（Cr）峰下面积的比值（NAA/Cr、Cho/Cr、Glx/Cr及mI/Cr）。结果 与对照组比较,肝硬化组Cho/Cr、mI/Cr减小,Glx/Cr升高,NCT-A时间延长、DST得分减少（P均<0.001）。肝硬化组NCT-A时间与Cho/Cr、mI/Cr呈负相关（r=-0.477,P=0.001;r=-0.695,P<0.001）,与Glx/Cr呈正相关（r=0.665,P<0.001）;DST得分与Cho/Cr、mI/Cr呈正相关（r=0.478,P=0.001;r=0.632,P<0.001）,与Glx/Cr负相关（r=-0.572,P<0.001）。结论 慢性乙型肝炎肝硬化患者神经认知功能障碍可能与背侧丘脑内代谢改变相关。     

1H-MRS定量分析鉴别诊断脑泡型包虫病与脑转移瘤

目的 探讨¹H-MRS鉴别诊断脑泡型包虫病和脑转移瘤的价值。方法 收集经术后病理及临床随访确诊为脑泡型包虫病和脑转移瘤患者各20例,分别进行脑常规MR、增强和¹H-MRS扫描。测量脑泡型包虫病、脑转移瘤病灶实质区、病灶水肿区及对侧正常脑实质N-乙酰天门冬氨酸（NAA）、胆碱（Cho）、肌酸（Cr）、乳酸和脂质峰（Lip+Lac）峰值,并计算Cho/Cr、Cho/NAA、（Lip+Lac）/Cr。结果 脑泡型包虫病病灶实质区Cho、Cho/Cr、Cho/NAA显著低于脑转移瘤,Lip+Lac、（Lip+Lac）/Cr明显高于脑转移瘤（P均<0.05）。脑泡型包虫病与脑转移瘤病灶实质区Cr、NAA,水肿区Cho、Cr、NAA、Lip+Lac、Cho/Cr、Cho/NAA、（Lip+Lac）/Cr差异均无统计学意义（P均>0.05）。脑泡型包虫病实质区Cr、NAA峰及水肿区NAA显著低于对侧正常脑实质,实质区及水肿区Lip+Lac、Cho/Cr、Cho/NAA、（Lip+Lac）/Cr明显高于对侧正常脑实质（P均<0.05）。脑转移瘤病灶实质区Cho、Lip+Lac、Cho/Cr、Cho/NAA、（Lip+Lac）/Cr及水肿区Cho/NAA、（Lip+Lac）/Cr明显高于对侧正常脑实质,实质区NAA峰低于对侧正常脑实质（P均<0.05）。结论 ¹H-MRS技术可显示脑泡型包虫病和脑转移瘤病灶及病灶周围水肿区组织代谢的特点,为鉴别诊断提供客观依据。     

1H-MR波谱分析急性期抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎额叶代谢物变化

目的 采用¹H-MR波谱（¹H-MRS）观察急性期抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎双侧额叶代谢物变化及其与认知障碍的关系,评估其应用价值。方法 采集20例急性期抗NMDAR脑炎患者（脑炎组）和20名健康成年人（对照组）双侧额叶¹H-MRS,获得相应MRS及各代谢物N-乙酰天门冬氨酸（NAA）、胆碱（Cho）、肌醇（mI）、谷氨酸盐复合物（Glx）和肌酸（Cr）的峰下面积,计算NAA/Cr、Cho/Cr、mI/Cr及Glx/Cr比值。记录脑炎组脑脊液抗NMDAR抗体滴度检测结果,以简易精神状态检查量表（MMSE）和蒙特利尔认知评估（MoCA）量表评估2组认知功能,比较组间¹H-MRS参数差异,并分析其与抗NMDAR脑炎患者MMSE评分、MoCA评分及与脑脊液抗NMDAR抗体的相关性。结果 脑炎组双侧额叶NAA/Cr、Cho/Cr、mI/Cr、Glx/Cr差异均无统计学意义（t=1.03、2.29、－0.73、0.15,P均>0.05）。与对照组相比,脑炎组双侧额叶NAA/Cr下降、Glx/Cr升高（P均<0.05）,而双侧额叶Cho／Cr、mI/Cr差异均无统计学意义（P均>0.05）。脑炎组双侧额叶NAA/Cr、Cho/Cr、mI/Cr及Glx/Cr与MMSE评分、MoCA总分和脑脊液抗NMDAR抗体均无明显相关性（P均>0.05）。结论 ¹H-MRS有助于诊断急性期抗NMDAR脑炎及监测病情;抗NMDAR脑炎急性期双侧额叶NAA/Cr降低、Glx/Cr升高,但与认知障碍无明显相关。     

获得性免疫缺陷综合征局灶性脑炎1H-MRS分析

目的 探讨¹H-MRS对获得性免疫缺陷综合征(AIDS)局灶性脑炎的诊断及鉴别诊断价值。 方法 对确诊的23例AIDS局灶性脑炎患者进行MR检查,以¹H-MRS分析32个病灶,测量代谢物N-乙酰天门冬氨酸(NAA)、胆碱化合物(Cho)、肌酸(Cr)、肌醇(mI)峰下面积均值,观察乳酸(Lac)、脂质(Lip)峰出现的频数,计算NAA/Cr、Cho/Cr、mI/Cr、NAA/Cho、(Cho+mI)/NAA值。比较AIDS局灶性脑炎实性区、坏死区、水肿区及与相邻正常脑组织代谢物差异。 结果 AIDS局灶性脑炎病变各区均出现NAA、Cho、Cr、mI峰。与相邻正常脑组织比较,NAA峰在病变各区均降低,Cho、mI峰在实性区略升高,在坏死及水肿区降低。Lac、Lip峰在病变各区均出现,在实性及坏死区出现频数明显高于水肿区,且Lip峰出现频数较Lac峰高。病变各区之间NAA/Cr、NAA/Cho、Cho/Cr、(Cho+mI)/NAA值差异有统计学意义(P<0.05);与相邻正常脑组织比较,病变各区Cho/Cr、(Cho+mI)/NAA值差异均有统计学意义(P均<0.05)。 结论 ¹H-MRS能够发现AIDS局灶性脑炎的特征性物质及能量代谢变化,为定性、定量诊断和鉴别诊断提供依据。     

1H-MRS评价颞叶癫痫患者杏仁核代谢物比值

目的 采用¹H-MRS探讨颞叶癫痫(TLE)患者双侧杏仁核的各代谢物比值改变.方法 对15例TLE患者(TLE组)和15名健康志愿者(正常对照组)行杏仁核¹H-MRS扫描,比较两组杏仁核的N-乙酰天门冬氨酸(NAA)/、NAA/Cr、NAA/Cho和Cho/Cr比值,分析TLE患者双侧杏仁核各代谢物比值与癫痫病程和发病年龄的相关性.结果 TLE组患侧杏仁核NAA/(Cho+Cr)和NAA/Cr比值与正常对照组比较均明显减低(P均 <0.05);TLE组患侧杏仁核NAA/(Cho+Cr)、NAA/Cr、NAA/Cho和Cho/Cr比值与TLE组对侧比较差异均无统计学意义(P均 >0.05);TLE组对侧杏仁核NAA/(Cho+Cr)、NAA/Cr和NAA/Cho比值与正常对照组比较均明显减低(P均 <0.05).TLE组双侧杏仁核NAA/(Cho+Cr)、NAA/Cr和NAA/Cho比值与癫痫病程及发病年龄均无相关性(P均 >0.05).结论 TLE患者双侧杏仁核存在显著的代谢异常,双侧杏仁核可能作为传导通路的一个环节,参与TLE的发病过程.     

多体素1H-MRS观察原发性三叉神经痛患者丘脑代谢异常

目的 采用多体素¹H-MRS分析原发性三叉神经痛（ITN）患者丘脑各亚区主要代谢物改变及其与临床病情的关系。方法 选取丘脑前部、板内区、后外侧部及后内侧部作为ROI进行¹H-MRS扫描,获取N-乙酰天门冬氨酸（NAA）、胆碱（Cho）、肌酸（Cr）峰下面积,计算患者组和正常对照组的NAA/Cr以及Cho/Cr比值,同时记录视觉模拟评分（VAS）,以便为影像学发现提供支持。 结果 与对照组比较,患者组患侧丘脑后外侧部及后内侧部NAA/Cr比值有所降低,患侧丘脑板内区NAA/Cr及Cho/Cr比值亦低于对照组;其中患侧丘脑后内侧部NAA/Cr比值与VAS评分（r=－0.58,P<0.01）及病程（r=－0.48,P<0.05）呈显著负相关。结论 ITN患者丘脑部分亚区代谢物存在异常;检测患侧丘脑后内侧部NAA/Cr比值可能反映疼痛程度。     

1H-MRS检测帕金森病伴轻度认知障碍患者后扣带回代谢物

目的 探讨¹H-MRS波谱技术联合线性拟合模型（LCmodel）软件在帕金森病（PD）伴认知障碍中的诊断价值。方法 选取PD患者35例（PD组）和健康体检者22名（对照组）,并根据是否伴有认知功能障碍,将PD组分为PDN亚组和PDMCI亚组。采用¹H-MRS波谱技术联合LCmodel软件获取PD组与对照组后扣带回（PCG）区域的波谱及代谢物的绝对浓度。比较2组各代谢物绝对浓度,并分析各代谢物绝对浓度与认知功能评分的相关性。结果 PDN亚组各代谢物绝对浓度与对照组差异均无统计学意义（P均>0.05）,PDMCI亚组总肌酸（tCr）、N-乙酰天门冬氨酸（NAA）、肌醇（mI）和胆碱复合物（tCho）的绝对浓度均较对照组降低（P均<0.05）;PDMCI亚组tCr绝对浓度较PDN亚组降低（P<0.05）。tCr（r=0.444,P=0.01）、谷胱甘肽（GSH;r=0.393,P=0.024）绝对浓度与MMSE评分存在相关性;tCr（r=0.367,P=0.035）、GSH（r=0.376,P=0.031）及tCho（r=0.375,P=0.031）绝对浓度与MoCA评分存在相关性。结论 ¹H-MRS技术联合LCmodel软件可定量分析PCG区域代谢物变化,有助于评估PD伴认知障碍。     

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.