Déficit en sphingomyélinase acide (maladie de Niemann-Pick B) : une étude rétrospective multicentrique de 28 patients adultes

Introduction

Acid sphingomyelinase deficiency (ASMD) is an autosomal recessive disease with a clinical spectrum ranging from a neurovisceral infantile form (Niemann-Pick disease type A) to a chronic visceral form also encountered in adults (Niemann-Pick disease type B, NP-B).

急性ST段抬高型心肌梗死直接PCI并发MACCE及大出血事件的相关因素

目的:回顾性分析急性ST段抬高型心肌梗死（STEMI）患者住院行直接冠状动脉介入治疗术(PCI)后发生主要不良心脑血管事件（MACCE）及大出血的相关因素。方法:以2008年9月～2010年8月收住的235例STEMI在12 h内行直接PCI术的患者为研究对象,分为病例组（包括MACCE及大出血）和对照组。搜集手术、药物、实验室等相关因素,包括急性梗死相关血管、术中有无慢血流和无再流发生及处理、处理前后血流分级、急性梗死相关血管球囊预扩张及支架扩张最高压力等数据,同时搜集左室射血分值、血小板计数、嗜酸性粒细胞计数、嗜碱性粒细胞计数、凝血系列、肝肾功、血脂4项、空腹血糖及心肌标志物等检测指标。采用多因素Logistic回归的方法分析上述因素与MACCE及大出血的相关性。结果:单因素分析显示病例组在急性IRA行直接支架植入术的比率、凝血酶原时间、CK方面高于对照组(P<0.05),在年龄、急性IRA慢血流及无再流的发生率、术前术中IABP的使用率、Killip分级、并发CTO的血管数量、急性IRA预扩最高压力、FPG、Cr、CK-MB方面显著高于对照组(P<0.01);而病例组在冠脉内替罗非班使用率、行2次PCI的比率、急性IRA支架的最高压力方面低于对照组(P<0.05),在男性患者的比率、慢血流及无再流处理前和处理后TIMI分级、植入支架的总数量、UA、TC、LDL-C、HDL-C、左室射血分数值(LVEF)方面显著低于对照组(P<0.01)。多因素Logistic回归分析显示年龄、冠脉内注射替罗非班、LVEF值与MACCE及大出血具有相关性（P<0.05）,慢血流及无再流处理后TIMI分级与MACCE及大出血具有显著相关性（P<0.01）、其中年龄呈正相关,冠脉内注射替罗非班、慢血流及无再流处理后TIMI分级及左室射血分值呈负相关。结论:高龄、慢血流及无再流处理后TIMI分级小和LVEF值低是MACCE及大出血的危险因素;冠脉内注射替罗非班是保护因素。     

Scanning electron microscopic assessment of coating irregularities and their precursors in unexpanded durable polymer‐based drug‐eluting stents

Objectives : To assess and quantify coating irregularities on unexpanded and expanded durable polymer‐based drug‐eluting stents (DES) to gain insights into the origin of coating irregularities. Background: Previous scanning electron microscopy (SEM) studies in various expanded DES revealed differences in frequency and size of coating irregularities between DES types and specific distribution patterns, however, the origin of these irregularities is unclear. Methods: We assessed at bench side a total of 1,200 SEM images obtained in 30 DES samples (15 expanded and 15 unexpanded) of Cypher Select Plus, Taxus Liberté, Endeavor, Xience V, and resolute. Results: For most coating irregularities seen on expanded DES (72%; 23/32), a matching irregularity (n = 18/23) and/or its precursor (n = 11/23) was observed in unexpanded DES. Unexpanded Cypher select showed (small) crater lesions and cracks together with precursors of “peeling.” On unexpanded Taxus Liberté, thinning of polymer, small bare metal areas, wrinkles, and one precursor type were found. Unexpanded endeavor showed cracks, small bare metal areas, crater lesions, and precursors of the latter. Unexpanded Xience V and resolute mainly revealed crater lesions and their precursors. On unexpanded versus expanded DES, there was no difference in measured frequency of coating irregularities and precursors (P = ns) with the exception of more bare metal areas on expanded Taxus Liberte (P = 0.01). Conclusions : Most coating irregularities, or the potential to develop them, are inherent to the unexpanded DES. Important determinants of the formation of coating irregularities may be the stent geometry and the physical properties of the coating, while stent‐balloon interaction plays no major role. © 2011 Wiley Periodicals, Inc.     

Comparison of clinical and self-reported diagnoses for participants on a community-based arthritis self-management programme

OBJECTIVE: With the advent of community-based arthritis education programmes, it is important to determine the accuracy of participants' self-reported diagnoses. The purpose of this study was to determine the level of agreement between general practitioner (GP)-recorded and self- reported diagnoses of participants attending an Arthritis Self- Management Programme (ASMP). METHODS: Participants enrolling on the ASMP were asked to (a) identify their type of arthritis via a self- administered postal questionnaire and (b) obtain a written confirmation of their diagnosis from their GP. The sample (n = 613) comprised mainly women (83%) with a mean age of 58.8 yr (S.D. 12.6) and a mean disease duration of 15.4 yr (S.D. 12.5). RESULTS: Participants' self-reported diagnoses were confirmed by GPs in 534 cases [87.1%, 95% confidence interval (CI): 84.4 89.8%]. Confirmed diagnoses were reported by 86.9% (95% CI: 83.1-90.7%) of those with osteoarthritis (OA) and 96.1% (95% CI: 93.6 98.6%) of those with rheumatoid arthritis (RA). The concordance rate for all other types of arthritis combined was lower at 60.5% (95% CI: 49.5-71.5%). There were no significant differences with respect to age, gender, education, physical functioning, duration of disease and number of GP visits between those who correctly identified their type of arthritis and those who did not. CONCLUSIONS: This study suggests that the majority of RA and OA participants attending an arthritis education programme can correctly identify their specific type of arthritis.      

Th17 and non-Th17 interleukin-17-expressing cells in chronic lymphocytic leukemia: delineation, distribution, and clinical relevance

Background

The levels and clinical relevance of Th17 cells and other interleukin-17-producing cells have not been analyzed in chronic lymphocytic leukemia. The objective of this study was to quantify blood and tissue levels of Th17 and other interleukin-17-producing cells in patients with this disease and correlate blood levels with clinical outcome.

Design and Methods

Intracellular interleukin-17A was assessed in blood and splenic mononuclear cells from patients with chronic lymphocytic leukemia and healthy subjects using flow cytometry. Interleukin-17A-producing cells were analyzed in formalin-fixed, paraffin-embedded spleen and lymph node sections using immunohistochemistry and immunofluorescence.

Results

The absolute numbers of Th17 cells in peripheral blood mononuclear cells and the percentages of Th17 cells in spleen cell suspensions were higher in patients with chronic lymphocytic leukemia than in healthy subjects; in six out of eight paired chronic lymphocytic leukemia blood and spleen sample comparisons, Th17 cells were enriched in spleen suspensions. Circulating Th17 levels correlated with better prognostic markers and longer overall survival of the patients. Two “non-Th17” interleukin-17-expressing cells were identified in chronic lymphocytic leukemia spleens: proliferating cells of the granulocytic lineage and mature mast cells. Granulocytes and mast cells in normal spleens did not express interleukin-17. Conversely, both chronic lymphocytic leukemia and healthy lymph nodes contained similar numbers of interleukin-17+ mast cells as well as Th17 cells.

Conclusions

Th17 cells are elevated in chronic lymphocytic leukemia patients with better prognostic markers and correlate with longer survival. Furthermore, non-Th17 interleukin-17A-expressing cells exist in chronic lymphocytic leukemia spleens as maturing granulocytes and mature mast cells, suggesting that the microenvironmental milieu in leukemic spleens promotes the recruitment and/or expansion of Th17 and other IL-17-expressing cells. The pathophysiology of Th17 and non-Th17-interleukin-producing cells in chronic lymphocytic leukemia and their distributions and roles in this disease merit further study.     

Global Trends in Infective Endocarditis Epidemiology

The global epidemiology of infective endocarditis is becoming better understood with the initiation of multi-center collaborative studies and with an increasing number of case series being reported from countries outside North America and Europe. However, there are still many knowledge gaps and a lack of population-based data. For endocarditis in developed countries, the role of rheumatic heart disease as a predisposing factor is diminishing; the population is increasingly elderly, staphylococci are becoming much more important pathogens, and proportionally more are healthcare-associated. In developing countries, the epidemiology of infective endocarditis remains similar to North America and Europe from the middle of the twentieth century, affecting a younger age group, is often associated with rheumatic heart disease, and is predominantly caused by streptococci.     

中西医结合的物理治疗和作业治疗

中国现代的康复治疗正朝着形成一个中西医结合新的康复治疗体系的方向前进,作者在分析了中国传统的和西方现代的物理治疗和作业治疗各自的优势和特点后,列举了中国传统运动疗法,物理因子疗法,针灸、推拿按摩等在康复治疗中应用的方式方法,作用和治疗用途,介绍了一些中西医结合物理治疗和作业治疗的方式和方法,最后,作者介绍了中国近20年来为促进在康复治疗上实行中西医结合所采取的措施和实行的政策。     

Relevance of early detection of HIV type 1 SI/CXCR4-using viruses in vertically infected children

The aim of the study was to investigate the prevalence and persistence of syncytium-inducing (SI) strains in HIV-1-infected children along time of infection and to evaluate the influence of antiretroviral therapy and host factors on viral tropism. This is a retrospective analysis carried out in 267 HIV-1 vertically infected children from an Argentinean cohort. The viral phenotype was screened in MT-2 cells and coreceptor usage confirmed by the GHOST cell assay. Also, CD4(+) T cell count, viral load, antiretroviral therapy, and human CCR5-Δ32 and CCR2-64I genotypes were analyzed. A high frequency of HIV-1 SI/CXCR4-using variants (22%) was found among children within the first trimester of life, reaching 46% after 10 years of infection. At acute infection, zidovudine prophylaxis did not significantly affect the proportions of SI HIV-1 strains, while their presence was favored by the CCR5(+)/Δ32 genotype. Interestingly, the majority of the early SI strains did not persist over time, probably due to a higher susceptibility to antiretroviral (ARV) treatment or immunologic pressure. At the chronic stage, SI variants emerged even in the presence of HAART reaching 36% at 120 months of infection. Also the HIV-1 SI phenotype was associated with lower CD4(+) T cell counts all along the course of infection. These findings highlight the need to evaluate the presence of SI/CXCR4 variants early at primary infection. This will make it possible to optimize the use of CCR5 inhibitors in children who are apparently carriers of the R5 virus preventing early therapeutic failure due to the reemergence of SI strains from reservoirs.     

β2-微球蛋白与缺血性卒中

β2-微球蛋白是由一条肽链组成的小分子蛋白质.以往研究证实,血清β2-微球蛋白是一种反映早期肾功能损伤的生物标志物,而肾功能损伤与缺血性卒中之间关系密切.近年来的研究显示,缺血性卒中患者血清β2-微球蛋白水平明显增高,可作为缺血性卒中风险的生物标志物.     

NSTEMI患者行非体外循环下冠状动脉旁路移植术的时机选择

目的:探讨非ST段抬高型急性心肌梗死(NSTEMI)患者行非体外循环下冠状动脉旁路移植术(OPCAB)的时机选择。方法:回顾性分析我院心脏外科2009年5月至2015年5月完成的261例行OPCAB的NSTEMI患者资料,按术前心肌肌钙蛋白Ⅰ(cTnⅠ)水平分为两组。A组cTnⅠ0.15ng/mL(n=103),B组cTnⅠ≤0.15ng/mL(n=158),比较两组患者手术时间、血流动力学参数、术后住院时间及术后并发症等情况。结果:术后30d内A组死亡4例(3.9%),B组死亡2例(1.3%),两组比较无统计学差异(P0.05)。多因素分析提示高龄、术前cTnⅠ0.15ng/mL、NSTEMI后10d内手术为术后30d内主要心脑血管不良事件(MACCEs)发生的独立危险因素(P0.05)。结论:为减少术后MACCEs事件的发生,建议于发生NSTEMI 10d后,且cTnⅠ降至0.15ng/mL以下时行OPCAB术。