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重链病(heavy chain disease,HCD)是一组临床罕见的B细胞克隆增殖性疾病,其特征是合成和分泌不完全单克隆免疫球蛋白重链[1-2].重链部分缺失,与轻链无共价结合,可能的原因是轻链合成缺乏或者重链和轻链不能形成二硫键结合.按重链抗原不同,可将本病分为γ、α、μ、δ重链病,ε重链病尚未见报道.HCD具有明显的异质性,临床表现多样,主要受累部位随HCD类型而不同.     

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??Abstract??With reference to a large number of stroke rehabilitation literature??The United States VA / DoD adult stroke rehabilitation guidelines??which revised the previous guidelines and provided the latest evidence-based evidence for the management and rehabilitation of stroke??has been of practical significance.This article proposed that China should make use of latest research results on stroke rehabilitation made by developed countries??and develop its own stroke rehabilitation guidelines suitable for China’s specific conditions.     

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慢性高原病(CMS)是青藏高原最常见的高原病,以红细胞过度增多和肺动脉高压显著增高为临床特征,国际上统一应用"青海标准"作为诊断标准。海拔3000 m以上人群患病率约为5%,汉族移居人群的患病率是世居藏族的5倍,居住海拔过高、劳动过强、男性、汉族、吸烟和坐业劳动是主要危险因素。周边化学感受器颈动脉体对低氧通气反应钝化是造成显著低氧血症和红细胞过度增生的原发机制。某些中、藏药物有一定的致适应作用,但有效控制危险因素和提高人体习服-适应能力是防治的关键。     

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肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种累及脊髓前角细胞、脑干运动神经核及锥体束,具有上下运动神经元损害并存的慢性进行性神经系统变性疾病,主要特征为选择性侵犯上下运动神经元(motor neuron,MN),出现进行性加重的肌肉萎缩、肌无力及锥体束征。其感觉系     

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??Abstract??Lupus nephritis is one of the most serious manifestations of systemic lupus erythematosis (SLE) and also an important predictor of poor outcome.Up to date??the clear etiology is still obscure.Data from a great of amount of studies support an important role of genetics in disease occurrence and progression.Genetic ancestry studies indicate that African and Asian genetic ancestries are associated with an increased risk of lupus nephritis as compared to European ancestry.In addition??based on the data from genome-wide association studies conducted in SLE??several novel genetic associations have been observed in lupus nephritis??providing key clues for further elucidating pathogenesis of this phenotype.Ongoing refined genetic studies centering lupus nephritis are underway.In this review??we will discuss highlights as well as drawbacks of these studies??hoping to contribute more to pathogenesis research and individualized treatment of lupus nephritis.     

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脊髓损伤(spinal cord injury,SCI)是中枢神经系统的严重创伤,因高致残率和高病死率一直成为医学研究的热门课题。传统观点认为,SCI是不可修复和再生的,近年来大量研究发现干细胞具有多向分化潜能,在特定的环境中对神经系统缺血、损伤、变性等原因造成的功能障碍有明显的修复作     

Sleep studies in children on long-term non-invasive respiratory support

Purpose

The aim of this study was to assess the impact of changes in respiratory support (RS) settings recommended after a titration polysomnography (PSG), in terms of daytime symptoms and quality of life.

Methods

A retrospective chart review of all RS (CPAP and bi-level ventilation) titration studies was carried out at our tertiary paediatric sleep laboratory in the past 5 years. All patients with at least two studies in the past 5 years were included in the analysis. Parents completed the obstructive sleep apnoea (OSA)-18 and Paediatric Daytime Sleepiness Scale (PDSS) questionnaires on the night of each PSG. Results are presented as means (SD).

Results

A total of 42 patients (25 on CPAP and 17 on bi-level ventilation, age 11 (6)?years) had 71 pairs of titration studies (41 CPAP and 30 bi-level). Changes in RS settings were recommended in 27 of 41 (65 %) CPAP studies and 11 of 30 (36 %) bi-level studies. Overall, changes were fully implemented by the treating physician in 55 % of cases. There was an improvement in total OSA-18 score between studies in 48 % of the paired CPAP studies and 65 % of bi-level studies. OSA-18 scores improved in 47 % of the studies where any recommended change had been implemented versus 0 % of those where none of the recommended changes had been made (p?=?0.1).

Conclusions

Titration studies frequently led to recommendations for a change in RS settings in these patients on long-term RS. Symptom scores were more likely to improve if recommendations for change were implemented by the time of the follow-up study.     

呼吸支持联合营养支持在高龄重症肺炎患者中的临床应用

目的探讨呼吸支持联合营养支持治疗在高龄重症肺炎患者中的应用价值。方法回顾性分析2003年2月至2011年12月收治的高龄重症肺炎48例患者的临床资料。将2009年前未积极采用规范性呼吸系统支持阶梯化管理并联合营养支持治疗者28例为对照组。观察组为2009年1月至2011年12月收治的病例20例。结果观察组治愈率明显高于对照组（90．0％VS71．4％）,死亡率明显低于对照组（10．0％w28．6％）,差异有统计学意义（P〈0．05）。结论对高龄重症肺炎患者进行规范性呼吸系统支持阶梯化管理并联合营养支持治疗可显著提高莺痒肺炎的治龠率。     

营养支持治疗对慢性阻塞性肺疾病合并呼吸衰竭的临床研究

目的评价慢性阻塞性肺疾病(COPD)合并呼吸衰竭(呼衰)时进行营养支持治疗后,患者营养状态变化和临床疗效。方法选择COPD急性期合并呼衰患者共43例,随机分为营养组(22例)与对照组(21例),2组均接受相同的抗感染和对症治疗,营养组加用了口服或管饲喂养肠内营养混悬液。结果3周后营养组与对照组体质量、皮褶厚度、上臂肌围、血清白蛋白、胆固醇、三酰甘油差异有显著性(P<0.05),营养组治疗前后各项指标差异也有显著性(P<0.05)。结论COPD急性加重期合并呼衰的患者提供合理的营养支持可显著改善其营养状况,提高临床疗效,缩短住院时间。     

应合理使用呼吸支持技术

近年来,呼吸支持技术进展迅速,国内呼吸危重病领域的学者们在长期临床实践中广泛吸纳和学习国际上的先进理论和经验,并结合国内的具体情况,进行了大量卓有成效的工作,缩小了与发达国家之间的差距,使严重呼吸衰竭患者的救治成功率显著提高.     

Clinical studies on the secondary bacterial infection in respiratory syncytial virus infection of children

Secondary bacterial infection was studied on 231 children admitted with Respiratory Syncytial virus (RSV) infection in the 10 years since 1987. Of the 231 children, 56 (24.2%) had dual bacterial infection possibly due to secondary bacterial invasion. The diagnoses of bacterial disease were sepsis (2), pyothorax (2), pneumonias (41), otitis media (7), nasopharyngitis (2) and urinary tract infection (2). Dual bacterial infections were more frequent in infants and children over 6 months than in infants younger than 6 months. The main etiologic agents were Staphylococcus aureus and enteric gram-negatives in infants, and Haemophilus influenzae, Streptococcus pneumoniae, beta streptococci and Branhamella catarrhalis in children over 1 year. The incidence of secondary bacterial infection was compared according to the usage of antibiotics just before admission. Patients who had been administered with penicillins or macrolides before admission had a significantly higher percentage of secondary bacterial infection (21/56, 37.5%) than those of no previous antibiotic therapy (11/64, 17.2%, p less than 0.025). The results indicate that the RSV infection itself sometimes predisposes to secondary bacterial infections, but indiscriminate use of antibiotics further increases the risk of secondary bacterial infections.     

Neonatal non-invasive respiratory support: Physiological implications

The introduction of assisted ventilation for neonatal pulmonary insufficiency has resulted in the successful treatment of many previously fatal diseases. During the past three decades, refinement of invasive mechanical ventilation techniques has dramatically improved survival of many high‐risk neonates. However, as with many advances in medicine, while mortality has been reduced, morbidity has increased in the surviving high‐risk neonate. In this regard, introduction of assisted ventilation has been associated with chronic lung injury, also known as bronchopulmonary dysplasia. This disease, unknown prior to the appearance of mechanical ventilation, has produced a population of patients characterized by ventilator or oxygen dependence with serious accompanying pulmonary and neurodevelopmental morbidity. The purpose of this article is to review non‐invasive respiratory support methodologies to address the physiologic mechanisms by which these methods may prevent the pathophysiologic effects of invasive mechanical ventilation. Pediatr Pulmonol. 2012. 47:837–847. © 2012 Wiley Periodicals, Inc.     

气道介入操作的呼吸支持选择现状

纤维支气管镜介入治疗在气道或肺疾病的诊断和治疗发挥着至关重要的作用,在临床中应用越来越广泛.操作过程选择合适的呼吸支持手段的关键是在保障患者安全的基础上,提供理想的手术条件而又能最大的限度气道内操作相关的并发症.目前临床中气道介入操作呼吸支持手段十分丰富,把握其应用指征尤为重要.具体方案选择需根据呼吸介入医生对病情的评估、医学中心可用的设备条件和医师对每种方法的熟悉程度等多种因素综合来决定.