Neuronal ceroid lipofuscinoses and possible pathogenic mechanism

The neuronal ceroid lipofuscinoses (NCLs) consist of eight autosomal recessively inherited storage disorders characterized by lysosomal inclusions of autofluorescent lipofuscins and rapid neurodegenerative progression. The NCLs include eight forms that result from genetic deficiency on genes CLN(1) to CLN(8), respectively: four classic forms with clinical onset at varying ages-infantile (INCL), late-infantile (LINCL), juvenile (JNCL), and adult (ANCL)-and four variants of late-infantile onset-the Finnish variant LINCL (fLINCL), Portuguese variant LINCL (pLINCL), Turkish variant LINCL (tLINCL), and progressive epilepsy with mental retardation (EPMR). The genes CLN(1) and CLN(2) have been characterized to encode lysosomal hydrolytic enzymes, but CLN(3), CLN(5), and CLN(8) encode transmembranous proteins with unknown function. Although clinical and pathological abnormalities have been recognized to be similar in all eight forms, the molecular mechanism explaining NCL pathogenesis remains unclear. In this review, the molecular basis for NCLs and a possible pathogenic mechanism are discussed.     

急性心肌梗塞Q－T离散度分析－－附68例临床报告

目的：探讨急性心肌梗塞（AMI）患首次心电图Q－T离散度（Q－Td)及Q－Tc离散度（Q－Tcd）与严重室性心律失常发生的关系,对预后进行评估。方法：对68例AMI患首次心电图Q－Td及Q－Tcd进行测定。结果：18例AMI并室速室颤组患Q－Td,Q－Tcd显高于50例非室速室颤组患;结论：AMI患Q－Td及Q－Tcd值增大,室速室颤发生率增加,两呈正相关关系,易发生心源性猝死。故Q－Td及Q－Tcd可作为AMI病情危重预后差的标志,对判断预后有重要临床意义。     

葛根素对降低2型糖尿病患者微量白蛋白尿的临床观察

目的：观察葛根素对2型糖尿病早期肾脏病变的疗效。方法：对73例2型糖尿病24h尿微量白蛋白（UAE）在30－300mg/24h者随机分成两组,治疗组37例,每天静滴葛根素500mg,共2周;对照组用生理盐水静滴,共2周。观察血糖及尿UAE变化。结果：两组治疗前后比较差异有显著性意义,P＜0．01。结论：葛根素对2型糖尿病早期肾脏病变疗效明显,且无明显毒副反应。     

一氧化氮对犬下食管括约肌压力的作用

目的 :应用苄基二甲基十四烷氯化铵 (benzyldim ethyltetradecyl amm onium chloride,BAC)建立犬下食管括约肌(L ES)无神经动物模型 ,研究一氧化氮 (NO)对 L ES压力的作用。方法 :将 BAC环周注入犬 L ES,对照组注入等量生理盐水 ,均于注射前及注射后 6周测定 L ES压力 ;并观察 L -精氨酸、D -精氨酸、硝普钠及一氧化氮合酶 (NOS)抑制剂 N-硝基 - L -精氨酸 (L - NNA)对 L ES压力的影响 ;此外还测定了两组犬 L ES中 NO含量和 NOS活性。结果 :BAC处理组 L ES压力 [(4 2 .43±4.19) m m Hg,1m m Hg=0 .133 k Pa]显著高于对照组 [(2 2 .71± 5 .19) mm Hg]。 L -精氨酸可使对照组 L ES压力降低 ;L - NNA使其增高 ,但对 BAC处理组 L ES压力均无影响。硝普钠可降低两组犬 L ES压力。对照组 L ES中 NO为 (6 .0 5 8± 2 .0 6 7)μm ol/g,NOS为 (1.45 8± 0 .146 ) U /mg;而 BAC处理组 NO为 (1.797± 0 .873)μmol/g,NOS为 (0 .46 3± 0 .0 39) U /m g,均较对照组显著降低 (P<0 .0 1)。 结论 :BAC可使犬 L ES压力增高 ,其机制可能与 L ES局部 NO减少有关。     

胰岛的血液引流与胰腺外分泌的机能和临床意义

目的　研究胰岛的血液引流与胰腺外分泌的机能联系与临床意义。方法　运用微血管树脂 /墨汁灌注扫描电镜 /光镜观察法、保留动态及组织信息在静态样本的微循环观察法、FITC标记红细胞 (FITC- RBC)荧光活体显微镜观察法以及计算机图像处理微血管三维重建法 ,对 11例男性尸体、40只猴、2 4只狗、62只鼠、2 4只兔的胰岛血液引流通道与胰腺外分泌腺泡的关系进行了系统研究。结果　不同动物的内分泌胰岛血液主要经胰岛的三种类型输出血管引流至不同的外分泌腺泡区域 ,灵长类的部分胰岛血液还引流至邻近的小胰岛 ,研究者根据胰岛引流系统的特征对其进行了分类及命名 :1连续型引流系统 ,所有胰岛具有这类引流 (输出 )管道 ,其管径细 ,行程短、引流至胰岛周围的腺泡毛细血管区域。 2聚合型引流系统 ,为部分胰岛所有 ,其管径粗、行程长 ,引流至远离胰岛的腺泡毛细血管区域。 3跨越型引流系统 ,为部分胰岛所有 ,其输出管道越过小叶间隔引流至另一胰腺小叶的腺泡毛细血管区域 ,而这一小叶往往没有胰岛存在。 4胰岛 -胰岛型引流系统 ,灵长类部分胰岛的血液通过胰岛 -胰岛型引流通道回流至邻近的小胰岛。结论　胰岛具有完善联系外分泌腺泡的引流系统 ,提示含有高浓度胰岛内分泌激素的胰岛血液可能对外分泌腺泡的机能     

Risk factors and outcomes of cardiovascular disease readmission within the first year after dialysis in peritoneal dialysis patients

BackgroundIn the first year of dialysis, patients are vulnerable to cardiovascular disease (CVD) hospitalization, but knowledge regarding the risk factors and long-term outcomes of cardiovascular readmission within the first year after dialysis in incident continuous ambulatory peritoneal dialysis (CAPD) patients is limited.MethodsThis retrospective cohort study was conducted in incident CAPD patients. The demographic characteristics, laboratory parameters, and CVD readmission were collected and analyzed. The primary outcome was all-cause mortality, and the secondary outcomes included CVD mortality, infection-related mortality and technique failure. A logistic regression was used to identify the risk factors associated with CVD readmission within the first year after dialysis. Cox proportional hazards models were used to evaluate the association between CVD readmission and the outcomes.ResultsIn total, 1589 peritoneal dialysis (PD) patients were included in this study, of whom 120 (7.6%) patients had at least one episode of CVD readmission within the first year after dialysis initiation. Advanced age, CVD history, and a lower level of serum albumin were independently associated with CVD readmission. CVD readmission within the first year after dialysis was significantly associated with all-cause (HR 2.66, 95%CI 1.91–3.70, p < 0.001) and CVD (HR 3.42, 95%CI 2.20–5.31, p < 0.001) mortality, but not infection-related mortality or technique failure, after adjusting for confounders.ConclusionsOur findings suggest that an advanced age, a history of CVD, and a lower level of serum albumin were independently associated with CVD readmission. Moreover, CVD readmission was associated with all-cause and cardiovascular mortality in incident CAPD patients.     

Reply to Comments on “A Scientometric Analysis of Recent Literature on Arsenic Bioaccumulation and Biotransformation in Marine Ecosystems”

Bulletin of Environmental Contamination and Toxicology - In this letter, we formally replied to the comments on our recently published paper “A scientometric analysis of recent literature on...     

慢性肺源性心脏病人血液触变特性

本文用Low Shear 30流变测定仪,测定了55例肺心病患者全血触变特性的变化,分析了这些变化与肺心病发生和发展的关系。结果表明:肺心病患者血液的触变特性发生了异常改变。HCT、τ_o、η_s、μ、(η_s-μ)、A、ARC均升高,并随病情加重而逐渐明显。其原因主要在于肺心病缺氧所致的继发性红细胞增多及血液氧分压降低,和二氧化碳分压升高所致的红细胞变形性降低。血液触交特性的改变又使血液循环外周阻力增大,右心负荷增加,从而进一步加重了病情的发展。