淋巴细胞亚群及脑脊液蛋白在儿童吉兰-巴雷综合征中差异性分析

目的 探讨外周血淋巴细胞亚群和脑脊液蛋白对评估急性期吉兰-巴雷综合征(GBS)早期疾病严重程度的意义.方法 回顾分析2011年11月至2020年3月住院确诊的60例GBS患儿的临床资料.根据入院时病情、Hughes量表评分将患儿分为轻症组和重症组,比较两组患儿的年龄、性别、有无前驱感染以及外周血淋巴细胞亚群、脑脊液蛋白...     

吉兰-巴雷综合征伴高颅压328例临床分析

目的 观察吉兰-巴雷综合征(GBS)患者颅内压变化的特点,GBS患者颅内压增高的相关因素.方法 对1984年1月至2019年2月白求恩国际和平医院收治的328例GBS患者的临床资料及颅压的变化进行分析,对颅压增高的各种原因进行了相关分析.结果 本组328例GBS患者中颅压>200mmH2O 30例,发生率为9.15％,...     

Ménétrier’s病致顽固性低蛋白血症1例

Ménétrier’s病(Ménétrier’sdisease,MD)是一种罕见的肥厚性胃炎,以胃黏膜皱襞粗大、肥厚致大量蛋白质丢失,黏液分泌增加,胃酸分泌减少及低蛋白血症为特征.本文报道MD致顽固性低蛋白血症1例.患者,女,55岁,以"食欲减退,颜面及双下肢水肿半年"入院.其血清总蛋白39.8g/L,白蛋白23.8g/L.CT示胃黏膜皱襞弥漫性、不均匀增厚.胃镜示胃底和胃体黏膜皱襞肥厚、粗大,胃内见大量黏液潴留病理示胃小凹显著变长,呈螺旋状改变,固有层内腺体增生,部分腺体呈囊性扩张,可见黏液积聚.本病例提示,对于无任何胃病症状而出现无法解释的顽固性低蛋白血症患者,及时采取CT和内镜检查有助于早期诊断.     

中国2016年疑似预防接种异常反应监测数据分析

卡介苗是国家规定的婴幼儿出生时就需要接种的疫苗,是预防和控制结核病的有效手段之一,一般比较安全,仅在个别情况下会引起局部溃疡和淋巴结肿大、化脓,罕见卡介苗骨髓炎、卡介苗全身播散等严重异常反应。但由于卡介苗是出生时就接种,有些个体存在出生缺陷等情况,同时卡介苗又是减活疫苗,所以特殊个体会产生非常严重的卡介苗反应。本文报道一例有出生免疫缺陷——高IgE血症的婴幼儿,接种卡介苗后出现了局部的严重脓肿反应的案例。     

泊松分布与概率论的发展——西蒙·丹尼尔·泊松

西蒙·丹尼尔·泊松（Siméon Denis Poisson,1781-1840）是法国著名的数学家、物理学家以及统计学理论奠基人。他通过刑事审判案例建立泊松分布模型,使自然科学和社会科学的诸多随机现象得以解惑。此外,他引入了出生统计与泊松推理理论、拉普拉斯定理与误差理论以及大数定律和判断概率等相关理论,推动了19世纪概率论的发展,开辟了物理学由实验研究转向理论研究的新风向。     

Les nouvelles techniques diagnostiques des tumeurs neuroendocrines pancréatiques*<br/>The New Diagnostic Techniques for Pancreatic Neuroendrocine Tumours

Pancreatic neuroendocrine tumours are the tumours developed at the expense of pancreas and require a specific diagnostic assessment. The imaging assessment of a pancreatic neuroendocrine tumour is useful for diagnosis as well as for surgical/medical treatment. Recently, a number of advances have been made in the field of imaging pancreatic neuroendocrine tumours, in particular in functional imaging using radiolabelled somatostatin analogues. In this review, we approach diagnostic progress by focusing on the advances of recent years. Thus, the interest of conventional imaging (scanner, abdominal ultrasound, and magnetic resonance imaging), ultrasound endoscopy and the place of functional imaging mainly with radiolabelled somatostatin analogues are discussed.

Résumé
Les tumeurs neuroendocrines pancréatiques (TNEp) sont des tumeurs développées aux dépens du pancréas et nécessitent un bilan diagnostique spécifique. Le bilan d’imagerie d’une TNEp est utile pour le diagnostic ainsi que pour le traitement chirurgical/médical. Récemment, un certain nombre de progrès ont été réalisés dans le domaine de l’imagerie des TNEp, en particulier en ce qui concerne l’imagerie fonctionnelle utilisant des analogues de la somatostatine radiomarqués. Dans cette mise au point, nous abordons les progrès diagnostiques en nous focalisant sur les avancées des derniè- res années. Ainsi, il est abordé l’intérêt de l’imagerie conventionnelle (scanner, échographie abdominale, imagerie par résonance magnétique), de l’échoendoscopie et la place de l’imagerie fonctionnelle, principalement avec des analogues de la somatostatine radiomarqués.     

NF1基因突变致1型神经纤维瘤病8例临床分析

目的了解1型神经纤维瘤病(NF)的临床表型特征及异质性。方法回顾分析8例NF患儿的临床及基因检测资料。结果 8例患儿中男5例、女3例,均因生后多发牛奶咖啡斑就诊,2例伴丛状神经纤维瘤,1例伴多处皮肤神经纤维瘤。8例患儿有7种突变形式,NF1基因全部缺失2例;4种缺失突变,分别为c.4974_4977delCTAT,p.Y1659TfsX17、c.3987_3988delAG,p.S1329fsX4、c.1511delC,p.P504QfsX22和c.6388delC,p.L2130fsX3;1种c.3975-2delA剪切突变;1种c.3721 CT,p.R1241X无义突变。除全基因大片段缺失和无义突变之外,其余均为未报道的新突变。患儿父母外周血均未检测出相应的突变。结论 NF1基因突变可致1型NF,此研究发现5种新的突变,且均为de novo突变。多发牛奶咖啡斑是1型NF最早的临床表现,对疑似患者应尽早行基因分析确诊。     

Curative resection with endoscopic submucosal dissection of early gastric cancer in Helicobacter pylori-negative Ménétrier’s disease:A case report

BACKGROUND Adult-onset Ménétrier’s disease is strongly associated with Helicobacter pylori(H.pylori)infection and an elevated risk of carcinogenesis.Cases of early-stage gastric cancer developed in H.pylori-negative Ménétrier’s disease are extremely rare.We report a case of early gastric cancer in H.pylori-negative Ménétrier’s disease that was curatively resected with endoscopic submucosal dissection(ESD).CASE SUMMARY A 60-year-old woman was referred to our hospital after her medical examination detected anemia.Contrast-enhanced upper gastrointestinal(UGI)radiography revealed translucency of the nodule-aggregating surface with giant rugae.Blood tests showed hypoproteinemia and were negative for serum H.pylori immunoglobulin G antibodies.The 99mTc-DTPA-human serum albumin scintigraphy showed protein loss from the stomach.UGI endoscopy showed a 40-mm protruding erythematous lesion on giant rugae of the greater curvature of lower gastric body,suggesting early-stage gastric cancer due to Ménétrier’s disease.En bloc resection with ESD was performed for diagnosis and treatment.Histology of ESD showed well-differentiated tubular adenocarcinoma.The cancer was confined to the mucosa,and complete curative resection was achieved.Foveolar hyperplasia and atrophy of the gastric glands were observed in non-tumor areas,histologically corresponding to Ménétrier’s disease.Three years after ESD,gastric cancer had not recurred,and Ménétrier’s disease remained in remission with spontaneous regression of giant gastric rugae.CONCLUSION Complete curative resection was achieved through ESD in a patient with earlystage gastric cancer and H.pylori-negative Ménétrier’s disease.     

结核疫苗的研究进展

结核病是由结核分枝杆菌引发的一种传染病。1882年，结核分枝杆菌(mycobacterium tuberculosis， Mtb)由Robert Koch发现，至今仍是致人类死亡主要病原体之一。更可怕是，多重耐药(MDR)和广泛耐药(XDR) Mtb患病率的明显增加。上世纪90年代，世界卫生组织(World Health Organization， WHO)宣布结核病处于全球性紧急状态。结核病的最佳预防措施是接种疫苗，但是卡介苗(bacille Calmette-Guérin，BCG)对成人的保护效力不完全。随着Mtb基因序列的检测成功，疫苗的研究取得了重大的进展，已经有十几种候选疫苗准备或已进人体临床试验阶段。当前热门的候选疫苗包括重组BCG疫苗、减毒Mtb活疫苗和亚单位疫苗等。本文将从这些方面进行综述。     

Reduced dose of Bacillus Calmette-Guérin versus full dose of Bacillus Calmette-Guérin for non-muscle-invasive bladder cancer after transurethral resection bladder tumor： a meta-analysis of randomized controlled trials

Background Bladder cancer is widely known as the most common malignant tumor in the urinary tract,with 75％-85％ of patients suffering from nonmuscle invasive bladder cancer （NMIBC）.However,the optimal dose of Bacillus Calmette-Guérin （BCG） remains controversial.The aim of this study was to compare the therapeutic efficacy of full dose （FD） with the reduced dose （RD） of BCG.Methods Randomized controlled trials （RCTs） were selected through the Cochrane Library,PubMed and Embase and were supplemented by hand searching of bibliographies.The end points include overall survival rate,recurrence rate,progression rate and side effects.Results Five RCTs that included a total of 1 473 patients （727 in the reduced dose group vs 746 in the full dose group）,with a median follow-up period from 33.5 month to 7.1 year.Disease in 80 of 687 （11.6％） patients assigned to the RD group progress to the muscular layer or distant metastasis,compared with 81 of 698 （11.6％） patients assigned to the FD group （RR=1.02; 95％ CI,0.77-1.36; P=0.89）.The incidence of recurrence at three year was reported in all five studies to be 41.1％ （299 of 727） and 36.1％ （269 of 746） in the RD and FD groups,respectively （RR=1.13; 95％ CI,1.00-1.29; P=0.05）.The 5-year survival rate was 75.9％ （502 of 662） in the RD group,and 75.8％ （510 of 673） in the FD group.In the RD group 41 of 655 （6.3％） patients and 56 of 663 （8.7％） patients in the FD group did not complete the treatment due to systemic or local side effects （RR=0.75; 95％ CI,0.51-1.10; P=0.14） Conclusions In general,the results of our study demonstrate a trend towards a reduction of the toxicity in reduced dose group without affecting the efficacy of treatment when compared with full dose.More trials with large sample size are still necessary to explore the prognosis of the patients with high risk of tumor in different dose group.