模拟诊疗在留学生心血管内科实习带教中的应用研究

目的　探讨模拟诊疗在留学生心血管内科实习带教中的应用效果。方法　将35名留学生随机编号，分成试验组和对照组。试验组18名学生分成3组（每组6名）进行模拟诊疗教学，对照组17名学生进行常规实习。通过填写评价单和相关的临床能力测试对两组教学效果进行评价。选用SPSS 19.0软件进行t检验。结果　参与模拟诊疗教学的留学生均希望在各科实习中多采用此方法。在临床能力测试方面，试验组学生在病史采集[（22.16±2.17）vs.（20.33±2.03）]、体格检查[（23.05±1.79）vs.（19.78±2.05）]及回答问题[（23.50±1.47）vs.（19.56±1.92）]方面均优于对照组，差异有统计学意义（P<0.05）。但是在病历书写方面，两组差异无统计学意义（P>0.05）。结论　利用高仿真的模拟诊疗教学手段，弥补了留学生在实习期间不能充分接触患者的缺陷，有助于提高其临床操作技能、增强医患沟通能力等医学职业素养。     

Relationship between EMG-detected and ultrasound-detected fasciculations in amyotrophic lateral sclerosis: A prospective cohort study

ObjectivesFasciculation potentials (FP) are an important consideration in the electrophysiological diagnosis of ALS. Muscle ultrasonography (MUS) has a higher sensitivity in detecting fasciculations than electromyography (EMG), while in some cases, it is unable to detect EMG-detected fasciculations. We aimed to investigate the differences of FP between the muscles with and without MUS-detected fasciculations (MUS-fas).MethodsThirty-one consecutive patients with sporadic ALS were prospectively recruited and in those, both needle EMG and MUS were performed. Analyses of the amplitude, duration, and number of phases of EMG-detected FPs were performed for seven muscles per patient, and results were compared between the muscles with and without MUS-fas in the total cohort.ResultsThe mean amplitude and phase number of FP were significantly lower in patients with EMG-detected FP alone (0.39 ± 0.25 mV and 3.21 ± 0.88, respectively) than in those with both FP and MUS-fas (1.22 ± 0.92 mV and 3.74 ± 1.39, respectively; p < 0.0001 and p = 0.017, Welch’s t-test).ConclusionSmall FP may be undetectable with MUS. MUS cannot replace EMG in the diagnostic approach for ALS.SignificanceClinicians should use a combination of EMG and MUS for the detection and quantitative analysis of fasciculation in ALS.     

调整Kappa角及光学切削直径对准分子激光原位角膜磨镶术后的影响

目的　评价调整光学切削直径及Kappa角后对准分子激光原位角膜磨镶术（laser in situ keratomileusis，LASIK）后效果的影响。方法　选取2017年1月至12月在我院行LASIK手术的高度近视患者313例（626眼），根据切削直径分成两组，试验组157例314眼，切削直径设定为6.0 mm，对照组156例312眼，切削直径设定为6.5 mm。试验组患者激光切削前修正Kappa角，对照组不做修正。患者术前进行裸眼视力、主视眼确定、验光、眼压、暗室下瞳孔直径、泪液分泌试验、裂隙灯、散瞳验光、眼底检查、pentacam测量角膜厚度、角膜地形图测量角膜前后表面及Kappa角等检查。术后1 d、1周、1个月随访，并检查裸眼视力、角膜厚度、波前像差及夜间视力、光晕、眩光等情况。比较两组患者角膜厚度变化、手术所用时间以及两组患者术后的高阶像差的差异。结果　试验组与对照组患者年龄分别为18～44（24.19±5.33）岁、18～42（25.08±4.91）岁，屈光度分别为（-7.47±1.04）D、（-7.61±1.12）D。两组年龄、屈光度比较差异均无统计学意义（均为P>0.05）。试验组与对照组患者术前Kappa角分别为，X轴:（210±40）μm、（200±30）μm，Y轴:（190±30）μm、（220±40）μm，差异无统计学意义（P=0.210）。两组手术前后的角膜厚度及术后角膜基质床的厚度差异均无统计学意义（均为P>0.05）。试验组与对照组的手术时间分别为（15.56±1.89）s和（20.83±3.03）s，差异有统计学意义（P=0.000）。试验组的总高阶像差和垂直慧差的变化均明显低于对照组（均为 P<0.01），但两组间的水平慧差差异无统计学意义（P>0.05），对照组的球差低于试验组（P<0.01）。结论　LASIK手术中科学合理地调整Kappa角可有助于提高患者术后的视觉质量。     

糖尿病黄斑水肿患者病变程度与脉络膜厚度的关系

目的　利用频域光学相干断层扫描深度增强（enhanced depth imaging spectral domain optical coherence tomography，EDI SD-OCT）观察糖尿病黄斑水肿（diabetic macular edema，DME）患者脉络膜厚度（choroidal thickness，CT）的变化及结构特点，探讨DME病变程度与CT的关系。方法　纳入2型糖尿病患者共123例204眼，其中69眼诊断为DME（DME组），135眼无黄斑水肿为对照组。DME眼依据OCT形态学特点进一步分为视网膜弥漫性增厚（diffuse retinal thickness，DRT）型（34眼）、黄斑囊样水肿（cystoid macular edema，CME）型（19眼）和浆液性视网膜脱离（serous retinal detachment，SRD）型（16眼），利用EDI-OCT分别测量黄斑中心凹下CT和以黄斑为中心上、下、鼻、颞500 μm、1000 μm、1500 μm、2000 μm处CT。结果　DME组黄斑中心凹下CT为（326.72±90.15）μm，对照组为（320.17±106.46）μm，两组之间无统计学差异，但黄斑中心凹下CT与视网膜厚度间具有明显正相关关系（r=0.270，P=0.025）。DME亚型CT分别为:DRT型（303.94±81.47）μm、CME型（304.42±73.98）μm和SRD型（401.63±88.80）μm，SRD型CT明显高于其他亚型（P<0.05），此外，SRD型的周边CT同样呈现均匀一致的增厚；鼻侧CT从500 μm至2000 μm呈距离敏感性降低（P<0.05），但SRD型鼻侧CT降低幅度明显变缓（P=0.195）。结论　SRD型黄斑水肿患者CT在中心凹下及周边部均显著增厚，CT与DME病变程度之间有一定相关性。     

Five-year clinical outcomes using the bioresorbable vascular scaffold: Insights from the FRANCE ABSORB registry

Central illustration: cumulative major adverse cardiac events (MACE) and bioresorbable vascular scaffold (BVS) thrombosis rates after 1, 2, 3, 4 and 5 years.

     

经后颅窝乙状窦后入路三叉神经微血管减压术的快速康复外科护理

目的 探讨快速康复外科 （FTS）理念在经后颅窝乙状窦后入路三叉神经微血管减压术围术期患者护理中的应用效果。方法 选择2018年1月~2019年1月我院收治并行经后颅窝乙状窦后入路三叉神经微血管减压患者100例，随机分成观察组和对照组，每组50例。对照组应用常规方法进行围术期护理，观察组应用FTS理念进行围术期护理。比较两组焦虑情况、术后并发症发生率、平均住院日及平均住院费用。结果 观察组轻度焦虑多于对照组，中、重度焦虑少于对照组（P＜0.05）；观察组术后并发症发生率低于对照组[恶心呕吐（4.00% vs 16.00%）、尿路感染（4.00% vs 18.00%）、颅内感染（0 vs 8.00%）、颅内血肿（2.00% vs 14.00%）和术后应激性疼痛（16.00% vs 34.00%）]，差异有统计学意义（P＜0.05）；观察组平均住院日和平均住院费用均低于对照组[（9.13±1.14）d vs（12.44±0.89）d]；[（2.15±0.66）万元 vs （3.05±0.61）万元]，差异有统计学意义（P＜0.05）。结论 应用FTS理念能缓解经后颅窝乙状窦后入路三叉神经微血管减压术患者术前紧张恐惧的心理，减少术后并发症，缩短患者住院日，降低住院费用，护理效果较好。     

Treatment of thalassaemia: a review of the new approaches on transfusion safety and the novel therapeutics

Lifetime red cell concentrate (RCC) transfusions still account for significant iron overload‐related morbidity and mortality despite chelation therapy in thalassaemia. The cumulative risk of transfusion‐transmitted infections is substantial for thalassaemia patients. Pathogen reduction technologies for RCC may imply a proactive approach against new/re‐emerging pathogens and may be an ultimate safeguard for transfusion safety in the developing countries. Red cell alloimmunization may become a significant clinical challenge in thalassaemia. The availability of high‐throughput molecular blood group antigen typing in the donors may allow perfect match transfusion, beyond ABO‐D and CEK antigen‐matched transfusions. Allogeneic stem cell transplantation (A‐SCT) is the only available curative therapy in thalassaemia, but carries a substantial risk of serious adverse events and mortality. Gene addition therapy for correction of the α‐globin chain imbalance overcomes the problems of donor availability and immunological complications of A‐SCT. Gene editing by either gene disruption or correction emerged as a potential alternative to gene addition therapy in beta‐thalassaemia. A new era of novel therapeutics targeting α/β imbalance, ineffective erythropoiesis or iron dysregulation is unfolding in thalassaemia management, and a number of those now have agents in preclinical and clinical development. Hydroxyurea (HU) may improve globin chain imbalance and be beneficial for reducing or omitting transfusion requirement. Ruxolitinib has allowed steady decrease in spleen volume that may serve for avoiding splenectomy in beta‐thalassaemia. Luspatercept may restore normal erythroid differentiation and improve anaemia. Hepcidin mimetics or TMPRSS6 inhibitors may modulate ineffective erythropoiesis by iron restriction and improve anaemia and organ iron loading.     

Enteric anendocrinosis attributable to a novel Neurogenin-3 variant

Congenital diarrhea and enteropathies (CODEs) are a group of monogenic disorders that often present with severe diarrhea in the first weeks of life. Enteric anendocrinosis (EA), an extremely rare cause of CODE, is characterized by a marked reduction of intestinal enteroendocrine cells (EC). EA is associated with recessively inherited variants in Neurogenin-3 (NEUROG3) gene. Here we investigate a case of a male infant who presented with mysterious severe malabsorptive diarrhea since birth. Thorough clinical assessments and laboratory tests were successful to exclude the majority of differential diagnosis categories. However, the patient's diagnosis was not established until the genetic test using whole-exome sequencing (WES) was performed. We identified a novel homozygous missense disease-causing variant (DCV) in NEUROG3 (c.413C>G, p.Thr138Arg). Moreover, molecular dynamic simulation analysis showed that (p.Thr138Arg) led to a global change of the NEUROG3 orientation affecting its DNA binding capacity. To the best of our knowledge, this is the first time to apply WES to reach a differential diagnosis of patients with CODEs. Our study not only expands our knowledge about NEUROG3 variants and their clinical consequences but also proves that WES is a very effective tool for the diagnosis of CODEs. This might be of value in early diagnosis of diseases and prenatal CODEs detection.     

眼睑分裂痣的手术整形研究

目的：探究眼睑分裂痣的不同手术方法和治疗效果。方法随机选取该院在2014年1月—2015年12月收治的60例需要进行眼睑分裂痣手术治疗的患者作为观察对象,将其按照抽签法随机分为A组(n=20)、B组(n=20)和C组(n=20),A组患者采用全厚皮片移植术；B组患者采用转移或推进皮瓣术；C组患者采用皮片移植+皮瓣转移术。首先对3组患者的治疗效果进行观察,然后比较3组患者在治疗后的不良反应发生情况,对比不良反应的发生率。结果3组患者的治疗效果均较好,所有患者的上伤口在1期即全部愈合,未出现明显的睑缘切痕；A组患者中,出现不良反应的患者有1例,占5.0%。B组中,无患者出现不良反应,不良反应发生率为0.0%。C组中,出现不良反应的患者有1例,占5.0%。3组患者之间差异无统计学意义(P﹥0.05)。结论治疗眼睑分裂痣的方法较多,全厚皮片移植术、转移或推进皮瓣术以及皮片移植+皮瓣转移术均有较好的效果,患者无明显的不良反应,并且痊愈的效果较好,手术的操作也较为简便易行,具有较高的安全性和可行性,值得临床推广。