全身型幼年特发性关节炎并发巨噬细胞活化综合征2例报告并文献复习

目的探讨全身型幼年特发性关节炎（SOJIA）并发巨噬细胞活化综合征（MAS）的临床特征、诊断及治疗,以提高对该疾病的早期认识,降低病死率。方法分析2例SOJIA并发MAS患儿的临床症状、体征、辅助检查及病情进展、诊断、治疗、预后。结果 2例均有持续高热、肝脏及淋巴结进行性增大、肝功能急剧恶化、凝血功能障碍、外周血细胞骤减、血清铁蛋白急剧升高、中枢神经系统功能障碍、血沉和CRP下降、NK细胞减少等临床特征,经甲强龙冲击、环孢素A及丙种球蛋白静滴治疗后均好转出院。结论 SOJIA并发MAS早期表现为高热及外周血细胞减少等,及早予激素等治疗效果确切;临床医生应提高认识,以达到早期诊治、降低病死率的目的。     

成人斯蒂尔病与巨噬细胞活化综合征关联性的临床分析

目的 探讨成人斯蒂尔病(AOSD)与巨噬细胞活化综合征(MAS)的关系.方法 选择AOSD组为78例资料完整的AOSD;MAS组是从26例有组织学证据的噬血细胞综合征的随访治疗中确定11例为风湿免疫疾病相关的噬血细胞综合征.对以上患者的临床表现和实验室资料进行分析.结果 在AOSD组78例中,有9例(占12%)在使用治疗之前可以诊断为MAS,但无噬血组织学依据.在11例有噬血现象的MAS中,AOSD 6例,脂膜炎2例,系统性红斑狼疮、皮肌炎、系统性血管炎各1例.脾脏肿大、白细胞减低、贫血、血小板下降、高甘油三酯是AOSD出现MAS的相关临床指标.结论 AOSD继发MAS的现象比较常见,严重者可以有组织学的噬血表现.AOSD出现脾脏增大、血细胞降低时,需要作MAS的相关检查,包括骨髓检查以及甘油三酯、纤维蛋白原、自然杀伤(NK)细胞活性等,以便及时诊断MAS.     

大剂量沐舒坦治疗新生儿胎粪吸入综合征疗效观察

胎粪吸入综合征（MAS）是新生儿围产期呼吸衰竭的主要病因之一，常并发持续肺动脉高压（PPHN）、气胸、肺出血、缺血缺氧性脑病（HIE）、胃肠功能障碍等，病死率较高．目前无特效治疗。2002年2月-2005年10月，我们采用大剂量沐舒坦治疗MAS患儿36例，疗效满意。现报告如下。     

POEMS综合征13例临床分析

目的 分析研究POEMS综合征的临床特征、以提高对本症的诊治水平。方法 采用回顾性分析对13例POEMS综合征进行分析。结果 13例患者中,4例伴骨髓瘤,其他最主要临床表现为多发性神经病变（100%),皮肤色素沉着（84.6%）,水肿（84.6%),肝、肿大（69.0%及61.5%）及内分泌病变（84.6％）。院外13例均误诊,入院后误诊9例;11例经治疗后症状缓解,2例自动出院。结论 POEMS综合征仍是一个被人们认识不足的疾病,因此应予经重视。     

氨溴索气管内灌洗加肺表面活性物质治疗新生儿胎粪吸入综合征的临床观察

目的：探讨氨溴索气管内灌洗加肺表面活性物质（ PS）治疗新生儿胎粪吸入综合征（ MAS）的临床疗效。方法将75例新生儿MAS随机分为两组，对照组37例行常规治疗，观察组38例在常规治疗基础上加用PS注射用牛肺表面活性剂治疗，比较两组治疗0、24、48 h的肺顺应性值（ C值）、氧合指数（ OI）、呼吸指数（ RI），机械通气时间、氧暴露时间、住院时间，以及并发症发生率、病死率。结果两组治疗后24、48 h的C值、OI、RI均明显降低（P＜0．01或＜0．05），以观察组降低明显（P均＜0．05）；与对照组比较，观察组的机械通气时间、氧暴露时间、住院时间缩短，并发症发生率和病死率降低（P均＜0．05）。结论氨溴索气管内灌洗加PS治疗新生儿MAS可改善患儿的肺氧合能力，缩短机械通气时间、氧暴露时间及住院时间，降低并发症发生率，提高生存率。     

药物超敏综合征19例临床分析

目的 分析药物超敏综合征的临床特征.方法 回顾分析我院19例药物超敏综合征患者的临床表现、实验室检查、治疗及预后.结果 （1）19例患者均有发热、皮疹及血液学异常,发生急性肝损伤13例（68.4%）,急性肾损伤7例（36.8%）,其中2例进展至尿毒症期,需行血液透析治疗;（2）19例患者中,只有5例住院期问确诊药物超敏综合征;死亡2例,病死率为10.9%;（3）别嘌呤醇药物超敏综合征较其它药物更易出现急性肾功能损伤（66.6%比23.1%,P〈0.01）.讨论药物超敏综合征是一种潜伏期长、易致内脏受累的临床综合征,死亡率较高,其发生难以预测,早期、足量激素治疗可以改善预后.     

Cronkhite-Canada综合征2例报告并国内外文献评价

目的总结Cronkhite—Canada综合征（CCS）的临床表现、消化道息肉特点、病理改变及诊断治疗，以提高临床医生对本病的认识。方法对我院诊断明确的2例CCS病例及1990年以来72篇文献报告的31例病例的临床资料回顾性研究。结果符合CCS诊断标准的入组病例33例，男20例，女13例；年龄17～77岁，中位年龄51岁。临床表现为脱发、指（趾）甲改变、皮肤色素沉着、腹部不适及低蛋白血症。全部患者均有消化道多发息肉，累及胃、小肠、大肠。息肉病理无特异性，其中8例癌变。治疗方法包括激素治疗17例，内镜下息肉摘除2例，因癌变、消化道梗阻及蛋白丢失性肠病手术11例（3例同时激素治疗）和其他治疗6例，其中1年内激素治疗有效14例，最长1例随访7年无复发。结论Cronkhite—Canada综合征（CCS）是以消化道多发息肉伴外胚层改变为主要临床特点的综合征，诊断较困难，激素治疗能改善症状，长期疗效仍待进一步研究。     

有机溶剂相关性肾病综合征(一种新发现的中毒性肾病?)

目的：分析有机溶剂相关性。肾病综合征患者的流行病学、临床表现及肾组织病理特点。万法：回顾性分析2004年10月至2008年4月在解放军肾脏病研究所住院经临床及肾活检诊断为有机溶剂相关性。肾病综合征18例患者临床资料，分析其接触有机溶剂的种类、临床表现、实验室检查、肾组织病理特点及初步随访结果。结果：（1）18例有机溶剂相关性肾病综合征患者多见于青年男性，其中男性15例，女性3例，平均年龄为（26．1±11．9）岁，所有患者均表现为中-重度水肿，大量混合性蛋白尿，平均尿蛋白（9．67±2．85）g／24h，其中4例患者入院时出现血压升高。（2）肾小管损伤标记物尿NAG酶及RBP均明显升高，其中尿NAG酶平均（67．5±40．4）u／g·cr。所有患者均无镜下血尿、糖尿及氨基酸尿。（3）18例患者血白蛋白均明显降低，平均（17．5±2．5）g／L，总胆固醇水平均明显升高，平均（11．5±2．87）mmol／L。9例患者入院时出现血肌酐升高，平均（130．8±73．7）μmoL／L。16例患者血免疫球蛋白IgG显著降低，平均为（3．93±2．61）g/L。（4）所有患者肾活检病理示肾小球病变轻，表现为轻度节段系膜增生性病变或无系膜增生性病变，但均可见足细胞数目减少，13例患者出现节段壁层上皮细胞增生，16例患者出现肾小管返流。所有患者均存在近端肾小管上皮细胞刷状缘脱落，但无间质炎细胞浸润。（5）18例患者对免疫抑制剂治疗均无明显疗效，随访时间1～43月，2例（11．1％）患者死亡，7例（38．89％）患者出现肌酐倍增，其中3例（16．67％）患者进入ESRD期。结论：有机溶剂相关性肾病综合征是一种新发现的肾脏疾病，多见于中青年男性，临床表现为超大量蛋白尿伴近端小管损伤，表现为尿NAG酶，尿RBP升高，混合性蛋白尿，尿渗量正常，无糖尿及氨基酸尿；肾活检病理表现为足细胞数目减少，节段壁层上皮细胞增生，肾小管返流以及近端小管病变；该病对免疫抑制剂治疗反应不佳，预后较差。     

Gitelman综合征9例临床分析

目的分析Gitelman综合征的临床特点。方法回顾性分析解放军总医院近25年9例住院治疗的Gitelman综合征病例。结果9例Gitelman综合征患者发病年龄14～33岁,男女比例为8：1。临床上以双下肢无力、发作性四肢软瘫、多饮、多尿、夜尿增加、手足抽搐等为主要表现,9例均有不同程度的下肢乏力,其中软瘫4例;实验室检查均表现为低血钾、代谢性碱中毒（9／9）,卧立位醛固酮试验显示,血肾素活性（9／9）、血管紧张素Ⅱ（9／9）及醛固酮（5／9）明显升高,而血压正常;血尿电解质检查显示,尿钾排出增加、低血镁、高尿镁、低尿钙、低尿钙肌酐比（〈0．2,5／5）;肾脏病理表现为肾小球旁细胞增生（2／2）;单纯补钾或联合消炎痛、安体舒通和门冬氨酸钾镁片等药物治疗后症状缓解,但血钾、血镁未升至正常（8／9）水平。结论Gitelman综合征的临床特点包括双下肢乏力、低血钾、高尿钾、血压正常,检查血镁、尿钙、尿镁或尿钙肌酐比和血肾素、血管紧张素Ⅱ、醛固酮水平可明确诊断;本病治疗应补钾、补镁、前列腺素合成酶抑制剂、醛固酮拮抗剂等多种药物联合应用,预后良好。     

急性呼吸窘迫综合征23例临床分析

目的探讨急性呼吸窘迫综合征（ARDS）的诊断和治疗。方法对23例ARDS患者的临床资料进行回顾性分析。结果23例患者死亡14例（死亡率60．9％）。结论多种危重病都有可能发生ARDS。监测患者的呼吸频率（RR）、经皮血氧饱和度（SpO2）、动脉血气（SaO2、PaO2）和PaO2／FiO2有利于ARDS的早期诊断；ARDS的治疗以机械通气为主，实施保护性肺通气策略，兼顾其他支持性治疗。     

Control of intraabdominal hemorrhage and shock: a comparison of fluid resuscitation, MAST, and balloon occlusion

Our study examined the efficacy of four treatment modalities in controlling hemorrhage and achieving hemodynamic stabilization in hemorrhagic shock: intravenous fluid replacement (IV); military antishock trousers used concomitantly with fluids (MAST); balloon occlusion at the level of the diaphragm with concomitant fluid replacement (balloon); and a combination of MAST inflation, balloon occlusion, and fluid resuscitation (MAST and balloon). Twenty-eight mongrel dogs were anesthetized, and the spleen was exposed and completely crushed. The abdomen was closed, and treatment was initiated and continued for four hours or until the dog died. For all conditions the hematocrit dropped during the course of the experiment; balloon occlusion was effective at slowing this drop (P less than .0001), but MAST had no statistically significant effect. Animals with balloons bled more slowly into the abdominal cavity than did animals in the other two groups (P less than .0001). MAST also were effective at slowing the bleeding (P less than .05). Of the balloon and the MAST and balloon dogs, all except one survived the entire four hours; this difference between balloon and nonballoon dogs is significant (P = .002). MAST did not have a statistically significant effect on survival. Perfusion pressure (PP) declined during the course of the experiment, and the balloon was effective at slowing this decline (P less than .0001); none of the other comparisons was statistically significant.     

Interplay between interferon-mediated innate immunity and porcine reproductive and respiratory syndrome virus

Innate immunity is the first line of defense against viral infection, and in turn, viruses have evolved to evade host immune surveillance. As a result, viruses may persist in host and develop chronic infections. Type I interferons (IFN-α/β) are among the most potent antiviral cytokines triggered by viral infections. Porcine reproductive and respiratory syndrome (PRRS) is a disease of pigs that is characterized by negligible induction of type I IFNs and viral persistence for an extended period. For IFN production, RIG-I/MDA5 and JAK-STAT pathways are two major signaling pathways, and recent studies indicate that PRRS virus is armed to modulate type I IFN responses during infection. This review describes the viral strategies for modulation of type I IFN responses. At least three non-structural proteins (Nsp1, Nsp2, and Nsp11) and a structural protein (N nucleocapsid protein) have been identified and characterized to play roles in the IFN suppression and NF-κB pathways. Nsp's are early proteins while N is a late protein, suggesting that additional signaling pathways may be involved in addition to the IFN pathway. The understanding of molecular bases for virus-mediated modulation of host innate immune signaling will help us design new generation vaccines and control PRRS.     

A Call to Action to Enhance Filovirus Disease Outbreak Preparedness and Response

The frequency and magnitude of recognized and declared filovirus-disease outbreaks have increased in recent years, while pathogenic filoviruses are potentially ubiquitous throughout sub-Saharan Africa. Meanwhile, the efficiency and effectiveness of filovirus-disease outbreak preparedness and response efforts are currently limited by inherent challenges and persistent shortcomings. This paper delineates some of these challenges and shortcomings and provides a proposal for enhancing future filovirus-disease outbreak preparedness and response. The proposal serves as a call for prompt action by the organizations that comprise filovirus-disease outbreak response teams, namely, Ministries of Health of outbreak-prone countries, the World Health Organization, Médecins Sans Frontières, the Centers for Disease Control and Prevention—Atlanta, and others.     

Disease Pandemics and Major Epidemics Arising From New Encounters between Indigenous Viruses and Introduced Crops

Virus disease pandemics and epidemics that occur in the world’s staple food crops pose a major threat to global food security, especially in developing countries with tropical or subtropical climates. Moreover, this threat is escalating rapidly due to increasing difficulties in controlling virus diseases as climate change accelerates and the need to feed the burgeoning global population escalates. One of the main causes of these pandemics and epidemics is the introduction to a new continent of food crops domesticated elsewhere, and their subsequent invasion by damaging virus diseases they never encountered before. This review focusses on providing historical and up-to-date information about pandemics and major epidemics initiated by spillover of indigenous viruses from infected alternative hosts into introduced crops. This spillover requires new encounters at the managed and natural vegetation interface. The principal virus disease pandemic examples described are two (cassava mosaic, cassava brown streak) that threaten food security in sub-Saharan Africa (SSA), and one (tomato yellow leaf curl) doing so globally. A further example describes a virus disease pandemic threatening a major plantation crop producing a vital food export for West Africa (cacao swollen shoot). Also described are two examples of major virus disease epidemics that threaten SSA’s food security (rice yellow mottle, groundnut rosette). In addition, brief accounts are provided of two major maize virus disease epidemics (maize streak in SSA, maize rough dwarf in Mediterranean and Middle Eastern regions), a major rice disease epidemic (rice hoja blanca in the Americas), and damaging tomato tospovirus and begomovirus disease epidemics of tomato that impair food security in different world regions. For each pandemic or major epidemic, the factors involved in driving its initial emergence, and its subsequent increase in importance and geographical distribution, are explained. Finally, clarification is provided over what needs to be done globally to achieve effective management of severe virus disease pandemics and epidemics initiated by spillover events.     

Barrett's Esophagus: Where Do We Stand?

Barrett''s esophagus (BE) is a precursor for esophageal adenocarcinoma, which has an increased incidence rate over the last few decades. Its importance stems from the poor five-year survival of esophageal adenocarcinoma and current data that suggest a survival benefit when surveillance programs are implemented. In this review, we will cover the pathophysiology and natural history of BE and the different endoscopic findings. The prevalence of BE in different geographic areas and the incidence of high-grade dysplasia and adenocarcinoma in this patient population is reviewed. Recent recommendation for screening and surveillance of BE has been covered in this review as well as the efficacy of nonconventional imaging modalities and endoscopic ablation therapies.     

The Technology Transfer from Europe to China in the 17th–18th Centuries: Non-Invasive On-Site XRF and Raman Analyses of Chinese Qing Dynasty Enameled Masterpieces Made Using European Ingredients/Recipes

Two masterpieces of the Qing Dynasty (1644–1912 CE), one in gilded brass (incense burner) decorated with cloisonné enamels stylistically attributed to the end of the Kangxi Emperor’s reign, the other in gold (ewer offered by Napoleon III to the Empress as a birthday present), decorated with both cloisonné and painted enamels bearing the mark of the Qianlong Emperor, were non-invasively studied by optical microscopy, Raman microspectroscopy and X-ray microfluorescence spectroscopy (point measurements and mapping) implemented on-site with mobile instruments. The elemental compositions of the metal substrates and enamels are compared. XRF point measurements and mappings support the identification of the coloring phases and elements obtained by Raman microspectroscopy. Attention was paid to the white (opacifier), blue, yellow, green, and red areas. The demonstration of arsenic-based phases (e.g., lead arsenate apatite) in the blue areas of the ewer, free of manganese, proves the use of cobalt imported from Europe. The high level of potassium confirms the use of smalt as the cobalt source. On the other hand, the significant manganese level indicates the use of Asian cobalt ores for the enamels of the incense burner. The very limited use of the lead pyrochlore pigment (European Naples yellow recipes) in the yellow and soft green cloisonné enamels of the Kangxi incense burner, as well as the use of traditional Chinese recipes for other colors (white, turquoise, dark green, red), reinforces the pioneering character of this object in technical terms at the 17th–18th century turn. The low level of lead in the cloisonné enamels of the incense burner may also be related to the use of European recipes. On the contrary, the Qianlong ewer displays all the enameling techniques imported from Europe to obtain a painted decoration of exceptional quality with the use of complex lead pyrochlore pigments, with or without addition of zinc, as well as cassiterite opacifier.     

Formoterol Delivered via the Dry Powder Aerolizer® Inhaler Versus Albuterol MDI and Placebo in Mild-to-Moderate Asthma: A Randomized, Double-Blind, Double-Dummy Trial

The objectives of this study were to compare the efficacy and tolerability of twice-daily formoterol dry powder 12 µg and 24 µg (Foradil) delivered via Aerolizer inhaler with four times daily albuterol (salbutamol) 180 µg delivered via metered dose inhaler (MDI) and placebo. A total of 554 adolescents and adults (ages 12-75 years) with mild-to-moderate asthma were randomized to this 12-week, multicenter, double-blind, double-dummy, placebo-controlled, parallel-group study. Twelve-hour spirometry measurements were taken at weeks 0, 4, 8, and 12. A total of 484 patients completed the study (122, 116, 127, and 119 given formoterol 12 µg, formoterol 24 µg, albuterol, and placebo, respectively). For the primary efficacy variable, the forced expiratory volume in 1 second (FEV₁), both formoterol 12 µg and 24 µg were statistically superior to placebo at all time points on all test days (p ≤ 0.017) and to albuterol at most time points on all test days (p ≤ 0.001). The onset of improvement in FEV₁ was rapid, with 15% increase within 5 min in 57%, 71%, and 65% of formoterol 12 µg, formoterol 24 µg, and albuterol patients, respectively. Formoterol was also superior to placebo and albuterol in terms of secondary efficacy variables: FEV₁ area under the curve, percentage of predicted FEV₁, forced vital capacity and forced expiratory flow, asthma symptom scores, and peak expiratory flows. In conclusion, both formoterol doses were superior to placebo in all lung function measurements. Overall, compared with albuterol, both formoterol doses produced superior bronchodilation. Formoterol and albuterol were safe and well-tolerated.