血友病儿童生存质量与关节健康状况的相关性研究

目的：探讨血友病（Haemophilia）患儿关节健康状况对其生存质量（Quality of Life,QOL）的影响。方法：选取4～18岁男性A型血友病患者31例,分别采用中文版儿童生存质量测定量表（The Pediatric Quality of LifeInventory Measurement Models,PedsQL）4.0和中文版血友病关节健康评估表（Haemophilia Joint Health Score,HJHS）2.1同期评价患儿的QOL及其双肘关节、膝关节和踝关节的健康状况。结果：PedsQL中家长对患儿QOL及社交功能方面的评分显著高于患儿自评得分（P〈0.05）;而在生理功能、情感功能及学校表现方面,家长评分与患儿评分之间的差异无统计学意义。血友病患儿PedsQL评分及其中生理功能项和社交功能项的评分与HJHS评分呈正相关（P〈0.05）,而情感功能及学校表现项的评分与HJHS评分无明显相关性。结论：关节健康状况在一定程度上影响血友病患儿的QOL,应通过积极的综合防治改善患儿的关节健康状况,提高其QOL。     

血友病儿童日常生活活动能力与关节健康状况的相关性研究

目的探讨血友病儿童关节健康状况对患儿日常生活活动（ADL）能力的影响,为提高血友病患儿的功能独立性提供理论依据。 方法选取2012年1月至2013年6月在北京协和医院血友病门诊确诊的4～18岁男性血友病患儿68例,分别采用血友病功能独立性评分（FISH）量表和中文版血友病关节健康评分（HJHS）量表评价血友病儿童的ADL能力和关节健康状况,应用Pearson相关分析方法计算其相关系数。 结果68例血友病患者的FISH和HJHS评分分别为(25.28±5.96)和(22.82±13.09)分;血友病儿童的HJHS评分与FISH评分呈负相关关系（r＝-0.746,P<0.01）;HJHS中的关节肿胀情况、关节周围肌肉状况、关节活动度、关节疼痛及步态评分与FISH评分亦呈负相关关系（P<0.01）,其中关节周围肌肉状况、步态和关节活动度评分与FISH评分的相关程度较高（r值分别为-0.815、-0.661和-0.628）,而FISH评分与关节摩擦音评分之间无相关性（r＝-0.089,P>0.05）。 结论较差的关节健康状况对血友病儿童的ADL能力的负面影响较大,其中关节周围肌肉状况、关节活动度和步态是其主要的影响因素。     

血友病儿童45例关节健康状况分析及其与步态的相关性研究

目的研究中国血友病儿童关节健康状况,及其对步态的影响。方法分别收集在北京协和医院、广州南方医院、温州市第三人民医院、北京儿童医院就诊的4～18岁的儿童共45例,使用中文版血友病关节健康评估表(HJHS)2.0版评价患者的双膝、双踝、双肘关节的健康状况及步态,对结果进行总结,并将其中的步态分析得分与各关节的总评分及各关节评分做相关性分析。结果 93.3%的患者有至少1个关节受累,91.0%的患者有2个及以上关节受累,84.4%的患者有肘关节受累,93.0%的患者有膝关节受累,93.3%的患者有踝关节受累;年长儿童关节受累更严重;步态异常率为88.9%。步态异常与膝关节、踝关节受累明显相关,与关节疼痛、肌力下降、肌肉萎缩及关节屈曲度和伸展度下降有显著相关,而与关节肿胀、关节肿胀持续时间、关节运动时摩擦音无明显相关性。结论中国血友病儿童关节受累常见,步态异常常见,膝关节与踝关节的受累,以及下肢关节疼痛、肌力下降、肌肉萎缩、关节屈曲度和伸展度下降与步态异常明显相关。     

血友病儿童膝关节挛缩康复治疗效果的最小临床重要差异

目的 探讨血友病儿童膝关节挛缩康复治疗效果的最小临床重要差异（MCID）。 方法 回顾性收集2018年1月至2022年8月在北京协和医院康复医学科接受康复治疗10次及以上的血友病儿童的膝关节挛缩相关资料。患儿康复治疗效果以膝关节血友病关节健康评分（HJHS）为量化指标,采用均数差法、多元线性回归法、受试者工作特征曲线和分布法综合估计血友病患儿康复治疗后膝关节HJHS改善的MCID。 结果 共纳入28例符合标准的血友病膝关节挛缩患儿,平均年龄（13.89±3.00）岁。膝关节HJHS的均数差法MCID估计值为5.13,95%置信区间（CI）为（4.18,6.07）,多元线性回归MCID估计值为4.31,95%CI为（3.64,4.98）,受试者工作特征曲线MCID估计值为3.50,分布法MCID估计值为1.64;综合以上估计值,患儿康复治疗后膝关节HJHS改善的MCID为4.31。 结论 血友病膝关节挛缩患儿康复治疗后膝关节HJHS改善的MCID为4.31。当膝关节HJHS改善的MCID高于4.31时,改善有临床意义。     

成人血友病性关节病超声评分与其临床功能的相关性研究

目的：评估成人血友病患者关节的超声HEAD-US-C评分与其临床功能评分的相关性。方法：本研究采用超声HEAD-US-C评分对40例成人血友病患者的膝、肘、踝关节进行超声评分,并采用血友病关节健康评估表2.1版(HJHS 2.1)及功能独立性评分表(FISH)对关节功能状况进行评估。结果:评估了40例患者的240个关节,HEAD-US-C评分为22.50(8.00,30.50),HJHS评分为31.50(15.50,46.75),FISH评分为27.00(21.50,32.00)。轻度与中度、重度之间的HEAD-US-C评分、HJHS评分及FISH评分均有显著差异(P<0.001)。A、B型血友病患者之间的HEAD-US-C评分与HJHS评分、FISH评分差异无明显统计学意义(r=0.844,r=-0.825,P<0.001)。膝关节和肘关节、踝关节的HEAD-US-C评分与HJHS评分、FISH评分均显著相关 (P<0.001)。结论：超声HEAD-US-C评分与HJHS、FISH评分具有很好的相关性,可以更客观地为临床提供有价值的关节损伤情况评估。     

成年血友病患者日常生活活动能力及其影响因素分析

目的:了解成年血友病患者的日常生活活动(activity of daily living,ADL)能力并探讨其可能的影响因素。方法:选取19—61岁成年男性血友病患者72例。分别采用血友病功能独立性评分(functional independence score in haemophilia,FISH)和血友病关节健康评估表(haemophilia joint health score,HJHS)2.1版对患者的日常生活活动(activity of daily living,ADL)能力及双肘、双膝和双踝关节的健康状况进行评定。结果:72例患者的FISH平均分值为(20.65±4.42)分,98.61%的患者FISH评分低于正常值,其中自我照顾、转移和移行功能评分低于正常值的患者百分比分别为80.56%、93.06%和94.44%。HJHS平均分值为(37.94±15.10)分。患者受累关节数和HJHS评分对FISH评分有反向影响(P0.05),Pearson系数分别为-0.460和-0.606。FISH评分与HJHS中关节周围肌肉状况、关节活动度、关节疼痛及步态评分呈反比(P0.05),其中与关节周围肌肉状况、步态及关节活动度评分的Pearson系数较高。结论:多关节出血及关节肌肉功能减退对血友病患者的ADL能力有不利影响。其中肌肉无力、步态异常和关节活动度减小是主要的影响因素。     

血友病患儿生活质量与关节健康状况的相关性

  目的  了解血友病患儿关节健康状况与其生活质量(quality of life, QoL)的相关性。  方法  选取2009年11月至2011年5月在北京协和医院血友病门诊确诊为血友病的68例男性患儿,年龄4~18岁。采用中文版血友病关节健康评估表(hemophilia joint health score,HJHS)2.1及中文版儿童QoL测定量表(the pediatric quality of life inventory measurement models, PedsQL)4.0分别评价血友病患儿的关节健康状况和QoL,相关性研究应用Pearson相关分析方法进行。  结果  血友病患儿PedsQL4.0评分为(1.84±0.78)分, HJHS2.1评分为(22.82±13.09)分, 两者呈正相关(P＜0.05, Pearson系数为0.527)。PedsQL4.0总分及生理功能项评分与HJHS2.1中关节肿胀、关节周围肌肉状况、关节活动度、关节摩擦音、关节疼痛及步态评分均呈正相关(P＜0.05),其中PedsQL4.0总分与关节活动度、关节周围肌肉状况及步态评分的相关性较高,Pearson系数分别为0.477, 0.465, 0.377。  结论  关节健康状况变坏对血友病患儿QoL有不良影响,关节周围肌肉状况、关节活动度和步态是主要因素。     

按需治疗血友病患者关节健康状况临床分析

目的:调查血友病患者关节健康情况。方法:收集2016年3月至2017年10月间于天津市第一中心医院就诊的血友病患者,记录患者基本信息,使用血友病关节健康评分表(HJHS)2.1评价关节健康状况,采用健康状况调查问卷(SF-36)了解血友病患者生活质量,而关于关节功能与生活质量相关性研究使用Pearson相关分析方法进行。结果:共收集按需治疗血友病患者196例,平均年龄27.81(2-73)岁,血友病A共189(96.43%)例,血友病B共9(3.57%)例。所调查关节未受累及者、1个关节受累者、2个及以上关节受累者分别占3.57%、11.72%及84.71%;肘关节、膝关节及踝关节受累率分别为71.93%、80.61%及82.91%;轻型、中间型及重型血友病患者关节累及率分别为61.60%、72.40%及80.73%;中间型及重型血友病患者平均HJHS分别为23.59±17.02分、26.69±17.68分,无统计学差异(P 0.05)。血友病患者关节功能与生活质量呈负相关(r=0.676)。结论:血友病患者关节炎发病率高,10岁以上患者至少一个关节受累,且多数患者存在多关节受累,关节功能受损,影响患者日常行为和活动。     

Mulligan手法治疗血友病患儿膝关节炎的疗效观察

目的 观察Mulligan手法治疗血友病出血性膝关节炎患儿的临床疗效。 方法 采用随机数字表法将40例血友病出血性膝关节炎患儿分为观察组及对照组,每组20例。2组患儿均给予常规运动干预（主要为患侧膝关节屈、伸训练）,观察组患儿在此基础上辅以Mulligan手法治疗（主要包括膝关节屈曲、伸直状态下关节松动及膝关节内、外旋状态下关节松动）。于治疗前、治疗4周后分别采用肌骨超声测量各组患儿膝关节滑膜厚度,采用Lysholm膝关节评分量表、血友病患者功能独立性量表（FISH）评定患儿膝关节运动功能及日常独立生活能力情况。 结果 治疗后观察组患儿膝关节髌上髁、内侧髁及外侧髁滑膜厚度［(7.82±2.48)mm vs 8.70(5.98,10.23)mm、(5.75±1.63)mm vs (6.58±1.58)mm,(5.82±1.50)mm vs 6.30(5.73,7.05)mm］均较对照组明显变薄。治疗后观察组患儿Lysholm评分［（77.20±6.83)分 vs 51.00（48.00,53.00分）］、FISH评分［29.00（27.00,29.75）分 vs (19.85±1.42）分］均较对照组显著增加(P＜0.05)。 结论 在常规运动干预基础上辅以Mulligan手法治疗能显著减小血友病膝关节炎患儿关节滑膜厚度,改善膝关节功能及日常独立生活能力,该联合疗法值得临床推广、应用。     

本体感觉训练配合关节粘连传统松解术在踝关节康复治疗中的应用

目的 观察本体感觉训练配合关节粘连传统松解术在踝关节骨折后康复治疗中的临床疗效。 方法 选择踝关节骨折后功能障碍患者30例（共47个关节）作为研究对象,分别进行中医关节粘连传统松解术、本体感觉训练、踝关节功能训练及冷疗等干预措施,连续治疗4周;然后采用美国足与踝关节协会踝与后足功能评分系统（AOFAS）评分、日常生活能力（ADL）评分、踝关节活动度、本体感觉训练后的位移差（包括角度位移和弧度位移）等指标进行康复评定。 结果 治疗后,患者关节活动度较治疗前有明显改善,AOFAS评分、本体感觉测试位移差、ADL评分均较治疗前有改善。治疗前后关节活动度分别为（29.07±12.88）°和（49.17±8.52）°,差异有统计学意义（P<0.05）;治疗前后AOFAS评分（50.52±19.30）分和（63.00±14.06）分,治疗前后ADL评分分别为63.23±19.90）分和（72.74±19.83）分,差异均有统计学意义（P<0.05）。治疗前后本体感觉测试指标的弧度位移差值（10.03±1.65）mm、（2.53±0.44）mm,角度位移差值（9.37±1.67）°、（2.18±0.45）°,差异亦均有统计学意义（P<0.01）。 结论 本体感觉训练配合关节粘连传统松解术治疗踝关节损伤后功能障碍有协同作用。     

Determination of creatine kinase isoenzyme MB activity in serum using immunological inhibition of creatine kinase M subunit activity. Activity kinetics and diagnostic significance in myocardial infarction.

This is a new method for the determination of creatine kinase isoenzyme MB activity in serum. The method uses direct activity measurement of creatine kinase B subunit activity after blocking of CK-M subunit activity by inhibiting antibodies. The test takes no longer than 15 min. The method yields an intra-serial C.V. of 2.0-12.9%, and a C.V. from day to day of 5.5%. The detection limit is 3.4 U/l creatine kinase MB. In the 95 cases with proven myocardial infarction several types of creatine kinase MB activity kinetics could be determined. The percentage of creatine kinase MB of peak CK-total is 6-25%, with a mean of 11.1%. The amount of creatine kinase MB with respect to total CK activity after reinfarction is higher than the amount after initial infarction.     

Dissociable correlates of two classes of retrieval processing in prefrontal cortex

Although substantial evidence suggests that the prefrontal cortex (PFC) implements processes that are critical for accurate episodic memory judgments, the specific roles of different PFC subregions remain unclear. Here, we used event-related functional magnetic resonance imaging to distinguish between prefrontal activity related to operations that (1) influence processing of retrieval cues based on current task demands, or (2) are involved in monitoring the outputs of retrieval. Fourteen participants studied auditory words spoken by a male or female speaker and completed memory tests in which the stimuli were unstudied foil words and studied words spoken by either the same speaker at study, or the alternate speaker. On "general" test trials, participants were to determine whether each word was studied, regardless of the voice of the speaker, whereas on "specific" test trials, participants were to additionally distinguish between studied words that were spoken in the same voice or a different voice at study. Thus, on specific test trials, participants were explicitly required to attend to voice information in order to evaluate each test item. Anterior (right BA 10), dorsolateral prefrontal (right BA 46), and inferior frontal (bilateral BA 47/12) regions were more active during specific than during general trials. Activation in anterior and dorsolateral PFC was enhanced during specific test trials even in response to unstudied items, suggesting that activation in these regions was related to the differential processing of retrieval cues in the two tasks. In contrast, differences between specific and general test trials in inferior frontal regions (bilateral BA 47/12) were seen only for studied items, suggesting a role for these regions in post-retrieval monitoring processes. Results from this study are consistent with the idea that different PFC subregions implement distinct, but complementary processes that collectively support accurate episodic memory judgments.     

俯卧位通气对高海拔地区肺复张治疗无效急性呼吸窘迫综合征患者氧合的影响

目的 探讨俯卧位通气对高海拔地区肺复张术(RM)治疗无效急性呼吸窘迫综合征(ARDS)患者的治疗作用.方法 从海拔2260m的地区医院筛选RM治疗无效的41例ARDS患者[平均氧合指数( PaO2/FiO2)较RM前升高＜20％视为RM无效],依不同病因分为肺内源性ARDS组(ARDSp组)和肺外源性ARDS组(ARDSexp组),每组再按信封法随机分为俯卧位组和仰卧位组,即ARDSp俯卧位组(11例)、ARDSp仰卧位组(9例)、ARDSexp俯卧位组(10例)、ARDSexp仰卧位组(11例).在通气前及通气1、2、3、4h监测动脉血氧分压( PaO2)、PaO2/FiO2、静态顺应性(Cst)、气道阻力(Raw)的变化.结果 通气lh时,ARDSexp俯卧位组PaO2/FiO2( mm Hg,l mm Hg=0.133 kPa)即较通气前显著升高(157.4±40.6比129.3±48.7,P＜0.05),并随通气时间延长呈持续增高趋势,4h达峰值(219.1 ±41.1);且ARDSexp俯卧位组通气3h内PaO2/FiO2较其他3组显著增高,另3组间则差异无统计学意义.ARDSp俯卧位组、ARDSexp俯卧位组通气4h时PaO2/FiO2均较相应仰卧位组显著增高(208.8±39.7比127.4±47.1,219.1±41.1比124.9±50.8,均P＜0.05).4组通气前后Cst无显著改变,各组间差异也无统计学意义.ARDSp俯卧位组通气4h时Raw(cmH2O·L-1·s-1)较通气前显著降低(6.8±1.7比10.7±1.8,P＜0.05),且明显低于其他3组;其他3组各时间点Raw组内及组间比较差异均无统计学意义.结论 俯卧位通气作为ARDS机械通气重要策略之一,可以改善RM无效高原ARDS患者的氧合,为抢救患者赢得宝贵的时间.     

Digital imaging among medical facilities

The Department of Veterans Affairs (VA) in the USA operates a network of 172 medical centres which all utilize a hospital information system (HIS) which has been developed and is currently maintained by the VA. During the past several years, an image management and communication module has been developed, installed and clinically utilized at the Washington DC and Maryland VA Medical Centres. This image management and communication system, referred to as the decentralized hospital computer program (DHCP) imaging system, is fully integrated with a commercial picture archiving and communication system (PACS). The system is utilized to capture, archive, and display all images generated within the hospital including radiology, nuclear medicine, pathology, endoscopy, bronchoscopy, and dermatology, intraoperative photographs, ECG data, and a limited number of paper documents. The ultimate goal of the project is to have all patient text and image data available at any clinical workstation to any authorized user anywhere within the network of medical centres. Clinical requirements for an imaging workstation include ease of use, rapid and reliable access to the complete set of patient information, and images which are of acceptable quality to meet the requirements of the user and the subspecialty. Patient confidentiality and data security must be safeguarded at all times. Integration of the images with the remainder of the patient's database was found to be critical to the success of the project. The experience at the Washington and Maryland facilities suggests that an imaging system that is successfully integrated with a hospital information system can provide substantial clinical and economic benefits both within and among medical centres. Clinical acceptance and utilization of the system has been excellent, particularly in diagnostic radiology where DHCP Imaging has been interfaced to a commercial PAC system. Based upon this initial experience, the VA has begun to deploy the system throughout its large network of medical centres.     

Myocardial elastography at both high temporal and spatial resolution for the detection of infarcts

Myocardial elastography is a novel method for noninvasively assessing regional myocardial function, with the advantages of high spatial and temporal resolution and high signal-to-noise ratio (SNR). In this paper, in-vivo experiments were performed in anesthetized normal and infarcted mice (one day after left anterior descending coronary artery [LAD] ligation) using a high-resolution (30 MHz) ultrasound system (Vevo 770, VisualSonics Inc., Toronto, ON, Canada). Radiofrequency (RF) signals of the left ventricle (LV) in longitudinal (long-axis) view and the associated electrocardiogram (ECG) were simultaneously acquired. Using a retrospective ECG gating technique, 2-D full field-of-view RF frames were acquired at an extremely high frame rate (8 kHz) that resulted in high-quality incremental displacement and strain estimation of the myocardium. The incremental results were further accumulated to obtain the cumulative displacements and strains. Two-dimensional and M-mode displacement images and strain images (elastograms), as well as displacement and strain profiles as a function of time, were compared between normal and infarcted mice. Incremental results clearly depicted cardiac events including LV contraction, LV relaxation and isovolumetric phases in both normal and infarcted mice, and also evidently indicated reduced motion and deformation in the infarcted myocardium. The elastograms indicated that the infarcted regions underwent thinning during systole rather than thickening, as in the normal case. The cumulative elastograms were found to have higher elastographic SNR (SNR(e)) than the incremental elastograms (e.g., 10.6 vs. 4.7 in a normal myocardium, and 6.0 vs. 2.4 in an infarcted myocardium). Finally, preliminary statistical results from nine normal (m = 9) and seven infarcted (n = 7) mice indicated the capability of the cumulative strain in differentiating infracted from normal myocardia. In conclusion, myocardial elastography could provide regional strain information at simultaneously high temporal (>/=0.125 ms) and spatial ( approximately 55 microm) resolution as well as high precision ( approximately 0.05 microm displacement). This technique was thus capable of accurately characterizing normal myocardial function throughout an entire cardiac cycle, at the same high resolution, and detecting and localizing myocardial infarction in vivo.     

Clinical Pharmacokinetics of Morphine

Morphine, the most widely used mu-opioid analgesic for acute and chronic pain, is the standard against which new analgesics are measured. A thorough understanding of the pharmacokinetics of morphine is required in order to safely and effectively use this analgesic in a wide variety of patients with different levels of organ function. A MEDLINE search was conducted to identify literature published between 1966 and January 2002 relevant to the pharmacokinetics of morphine. These publications were reviewed and the literature summarized regarding unique and clinically important elements of morphine disposition relative to its parenteral administration (including intravenous, intramuscular, subcutaneous, epidural and intrathecal administration), absorption profile (immediate release, controlled release, and sublingual/buccal, and rectal administration), distribution, and its metabolism/ excretion. Special populations, including infants, elderly, and those with renal/liver failure, have a unique morphine pharmacokinetic profile that must be taken into account in order to maximize analgesic efficacy and reduce the risk of adverse events.