Neuroimaging findings in infantile GM1 gangliosidosis

GM1 gangliosidosis is an autosomal recessive glycosphingolipid storage disease caused by defects in the enzyme beta-galactosidase. Three clinical forms (infantile-, juvenile-, and adult-onset) of the disease are recognized. Patients with infantile GM1 gangliosidosis present at birth or shortly thereafter with somatic and bony changes, followed by severe neurological deterioration ultimately leading to death within the first 2 years of life. We present the brain CT, MRI and MR spectroscopy (MRS) findings in a 17-month-old Turkish girl with infantile GM1 gangliosidosis. Neuroimaging findings in patients with infantile GM1 gangliosidosis have been reported only in a few cases. In this study, MRS of the thalamus was performed to study the metabolic changes in GM1 gangliosidosis. We showed a a decreased NAA/Cr ration and an increased Cho/Cr ratio. To our knowledge, this is the first report of magnetic resonance spectroscopy findings in type-1 GM1 gangliosidosis.     

奥利司他对肥胖IGT人群糖尿病预防效果

目的 探讨奥利司他(Orlistat,赛尼可)对肥胖糖耐量低减(IGT)患者的干预效果。方法 患者随机分两组奥利司他治疗组(50例)和对照组(51例),治疗为期1年,于治疗前后测量血脂、口服糖耐量试验(OGTT)、体重、身高等指标。结果1年后,治疗组上述指标明显改善(P<0.01),其恶化为糖尿病的发病率明显低于对照组(P<0.01)。结论 在肥胖IGT干预治疗中,奥利司他加饮食和运动治疗后可使体重减轻,改善糖耐量,明显减少糖尿病的发生。     

Protective Effects of Polydatin from Grapes and Reynoutria japonica Houtt. on Damaged Macrophages Treated with Acetaminophen

The unregulated use of acetaminophen (APAP), an antipyretic and analgesic drug, harms hepatocytes and kidney cells, leading to liver failure and acute kidney injury. Herein, we investigate whether APAP damages macrophages in the immune system by observing its effects on macrophage proliferation and apoptosis. Using proteomics, we analyzed the effects of APAP on macrophage protein expression profiles and evaluated whether polydatin, the active ingredient in grapes and wine, can repair the damaged cells. The results showed that APAP alters the morphology and physiological processes of macrophages, inhibits macrophage proliferation, and promotes apoptosis. We observed 528 differentially expressed proteins when 500 µg/mL APAP was administered to the cells. These proteins are involved in biological processes including cell division, apoptosis, and acute phase response. Overall, our findings demonstrate that APAP harms the immune system by damaging macrophages and that polydatin can repair this damage.     

逍遥散对慢性心理应激损伤大鼠海马神经元的影响

目的:观测逍遥散对多相性应激大鼠海马神经元结构的影响,探究逍遥散抵抗应激损伤大鼠海马神经元结构的机制.方法:大鼠采用慢性多相性应激模型,造模后取材进行Nissl体染色观测海马神经元内Nissl体及Sevier-Munger海马神经纤维,以Fura-2负载及荧光分光光度计检测大鼠海马突触体内Ca2 浓度.结果:造模组Nissl体数量明显减少,神经元胞体溃变、减少,神经纤维减少、排列紊乱;逍遥散治疗组Nissl体及神经元胞体及神经纤维数量有明显增加,排列更整齐;海马突触体内Ca2 浓度均明显升高.结论:多相性应激使大鼠海马神经元损伤从而致神经元内Nissl体减少,这种损伤是可逆或部分可逆的,逍遥散能明显抑制应激对大鼠海马神经元造成的损伤及神经元内Nissl体的减少.海马神经元细胞内Ca2 超载可能是慢性心理应激损伤大鼠空间学习记忆重要的神经机制,逍遥散也可能是通过抑制海马神经元细胞外Ca2 大量内流,阻止Ca2 超载,从而改善应激大鼠的学习记忆状况.     

去甲斑蝥素对G422脑胶质瘤抑制作用的体内研究

目的:研究去甲斑蝥素(NCTD)体内对脑胶质瘤G422细胞的抑制作用。方法:建立G422细胞小鼠移植瘤模型,将荷瘤小鼠随机分为模型组、CTX阳性对照组及NCTD高、中、低剂量组(100、50、25 mg.kg-1),每组10只,另设10只为正常对照组,正常对照组每日给生理盐水灌胃,CTX和NCTD组采用腹腔注射方式给药,10 d后双抗体夹心ABC-ELISA法测sIL-2R的含量;酶联免疫ELISA法测IFN-γ含量;剥瘤,计算抑瘤率;剥瘤的同时取脾脏,计算脾脏指数;MTT法检测NCTD对荷瘤小鼠脾脏T、B淋巴细胞增殖的影响。结果:NCTD可降低荷瘤小鼠血清sIL-2R含量,提高IFN-γ含量,抑制G422细胞小鼠移植瘤的生长;对脾脏指数影响较少,可促进荷瘤小鼠的T、B淋巴细胞增殖。结论:NCTD体内外可抑制G422细胞生长,并具有免疫调节作用。     

Efficacy analysis of Cheng’s GIRAFFE reconstruction after proximal gastrectomy for adenocarcinoma of esophagogastric junction

ObjectiveReconstruction of the digestive tract for adenocarcinoma of esophagogastric junction (AEG) is in dispute. This study evaluated Cheng’s gastric tube interposition esophagogastrostomy with reconstruction of His angle and fundus (Cheng’s GIRAFFE anastomosis) in laparoscopic/open proximal gastrectomy for Siewert type II AEG, which was performed at Zhejiang Cancer Hospital and the First Affiliated Hospital of Zhejiang Chinese Medical University. Here, we discuss the preliminary results of gastric emptying and anti-reflux.MethodsFrom a retrospective database, 74 patients with advanced Siewert type II AEG underwent curative proximal gastrectomy with GIRAFFE anastomosis, and their gastric emptying and anti-reflux outcomes were evaluated by the Reflux Disease Questionnaire (RDQ) score, nuclide gastric emptying, 24-h impedance-pH monitoring and gastroscopy.ResultsSeventy-four patients successfully completed proximal partial gastrectomy with Cheng’s GIRAFFE esophagogastric anastomosis. RDQ score six months after the operation was 2.2±2.5. Results of nuclide gastric emptying examinations showed that the gastric half-emptying time was 67.0±21.5 min, the 1-h residual rate was (52.2±7.7)%, the 2-h residual rate was (36.4±5.1)%, and the 3-h residual rate was (28.8±3.6)%; 24-h impedance-pH monitoring revealed that the mean DeMeester score was 5.8±2.9. Reflux esophagitis was observed by gastroscopy in 7 patients six months after surgery.ConclusionsCheng’s GIRAFFE anastomosis is safe and feasible for Siewert type II AEG.     

巩膜隧道切口和透明角膜切口对白内障超声乳化摘除术患者术后泪膜的影响

目的 探究巩膜隧道切口和透明角膜切口对白内障超声乳化摘除术患者术后泪膜的影响。 方法 选取2014年2月至2015年10月接受白内障超声乳化摘除手术的白内障患者86例为研究对象,按手术切口不同分为实验组和对照组,每组43例。实验组患者采用透明角膜切口行白内障超声乳化摘除术,对照组患者采用巩膜隧道切口进行手术,分别于术后第1、7、15和30天观察两种切口白内障超声乳化摘除术对患者泪膜功能的影响。 结果 术后第1、7、15天,实验组患者的泪膜破裂时间均较术前缩短,且实验组患者的泪膜破裂时间显著短于对照组;术后第30天,两组患者的泪膜破裂时间均较术前明显延长,差异有统计学意义(P均<0.05);术后第1、7、15、30天,两组患者的泪液分泌量均较术前明显增多,且实验组患者的泪液分泌量明显多于对照组,差异均有统计学意义(P均<0.05)。 结论 与巩膜隧道切口行白内障超声乳化摘除术比较,透明角膜切口对患者术后泪膜功能的影响较小,是更值得在临床推广的白内障超声乳化摘除术的一种切口方式。     

The evaluation of Chronic Progressive External Ophthalmoplegia with computerized tomography

Introduction: Chronic progressiveexternal ophthalmoplegia is characterisedby limitation of ocular motility in alldirections of gaze and ptosis.Innervational or myogenic factors wereclaimed to be responsible for thismotility disorder. The aim of thisstudy was toinvestigate the extraocular muscles inCPEO with computerized tomography in an attemptto distinguish extraocular musclemorphology caused by this disorderfrom that occurring in normal individuals.Methods: Eighteen orbits from 9patients diagnosed with CPEOwere included in the study. Axialand coronal scans were obtained for CTevaluation of extraocular muscles and thedimensions of extraocular muscles were measured.The control group consisted of40 orbits belonging to 20 individuals and,the results were compared with a student'st test.Results: The thickness (the verticaldimension of vertical recti and thehorizontal dimension of horizontal recti)of all rectus muscles wassignificantly decreased in comparison with the controlgroup, whereas the width (the horizontal dimensionof vertical recti and the verticaldimension of horizontal recti) was similar inboth the diseased and normal orbits.In all the rectus musclesof the diseased orbits,the normal fusiform shape was lost and the muscles appeared asthin bands.Discussion: The differentiation ofCPEO from other myogenic and neurogenicdisorders may present difficulty,and a cluster of criteria are required fora final diagnosis. CT has provento be a valuable tool in assessing extraocularmuscles [1, 2]. In this study, an extremeatrophy of all rectus muscles wasdemonstrated by means of CT. This diagnostic method mayconsequently contribute to a properdiagnosis of CPEO.     

Incidental discovery of nonpaternity during prenatal testing of genetic disease

Many ethical considerations surround prenatal genetic diagnosis, some of which are not anticipated. We present 2 cases in which misattributed paternity was inadvertently discovered during the workup of a father and son and a father and fetus, and present arguments for and against disclosure of this information. We recommend that healthcare providers must be prepared to deal with this kind of issue.     

Hodgkin Lymphoma after Disseminated Mycobacterium genavense Infection,Germany

Mycobacterium genavense infection, a rare nontuberculous mycobacteria infection, occurs in heavily immunocompromised patients (i.e., those with advanced HIV disease, genetic disorders, or acquired immunologic disorders and those undergoing immunosuppressive therapy). We report a case of disseminated M. genavense infection preceding Hodgkin lymphoma in a patient without obvious risk factors for this infection.