透析相关性淀粉样变

透析相关性淀粉样变 透 析 相 关 性 淀 粉 样 变 的 临 床 报 告 傅芳婷 赵 丽 卞维静 张 凌 王 武 王 瑛 透析相关性淀粉样变(DRA)是血液透析患者远期致残性严重并发症。国外报导透析5年DRA发生率低于5%，10年后可高达65%，15年后可达 [1]75%—100%。发病率可随年龄及透析龄增长而增加，男、女患病大致相似。随着透析疗法的普及，长期生存数量不断增加，DRA将成为影响长期透析患者生活质量和康复率的重要因素。 材料和 一、观察对象:我院透析中心长期血液透析超过10年以上患者共30例，男14例，女16例，年龄58.8?8.43岁，透析时间10—18年，原发病为慢性肾小球肾炎15人，多囊肾及良性小动脉硬化各4人、结缔组织病2人、慢性肾盂肾炎5人。 二、研究: 30例透析患者进行B超检查,型号ATL3000，探头频率5-12MH2;惠普尖端影像，探头频率7.5-10MH2，横、纵、斜向扫描双侧肩关节，横向测定冈上肌处肩袖(RC)的厚度，并观察骨关节面的平整性、絮状物、关节周围有无增强回声(EP)，关节腔有无积液。X线平片14人与CT检查14人(CT机为PQ6000)和MRI5人(MRI机为GE Signa0.5T)对骨的病变进行比较。 ?S示 三、统计:本文数据以X ____________________ 作者单位:100029中日友好医院 肾病中心(傅芳婷 赵丽 卞维静 张 凌) CT室(王 武) B超室(王 瑛) for computer software engineering GB/T50314-2000 of electrical installations code for construction and acceptance of GBJ232- 92 intelligent building weak design construction figure set GJBT-471 2. system description this times engineering weak design including: hours a Kok information facilities system integrated wiring system (containing room network, communications part) wired TV system information guide and the released system hours II Kok public security system fire automatically alarm system (containing public broadcast part) security technology prevention system security monitoring system out entrance control system car library management system hours three Kok building equipment monitoring system hours four Kok information application system queued station ICU visitation system operation the system monitoring and training system call intercom system hours, five hours Kok Kok intelligent integrated system of information system (1) cabling systems (including computer room network, communication component) computer network systems and voice communications systems Center of information on medical floor computer room located in the engine room. Voice communication system using a local telecommunications operator providing voice service, configuration-independent integrated services digital program-controlled switchboards. Operators providing communications ... If any compatibility requirements, by way of increased access to the Communications Board within the chassis to meet 结果 长期血液透析患者超过10年以上共30例，均接受B超检查,其中25例超声可见冈上肌腱为主的RC增厚,为0.86?0.12mm(正常参考值<0.8mm),占83.3%，在肩袖的肌腱之间有片状PE阳性者6例,占20%，膝关节积液者20例,占66.6%。X线检查(颈部、手、膝关节)共14例,可见囊性骨损害者10例，此10例均存在二个或二个以上部位有意义的骨损害，占71.4%，2例有颈椎间隙变窄占14.3%，此二例患者透析时间均超过16年。 CT扫描和MRI检查:CT扫描14例，可见囊性骨损害14例，占100%，其中一例肱骨头非持重囊肿大于10mm，与文献报道相符。上述X线检查发现2例颈椎间隙变窄的患者经CT扫描和MRI检查发现齿状突膨大，项韧带，黄韧带增厚，被软组织取代，压迫颈端脊髓呈骨破坏关节病变的牙样侵蚀过程2例，占14.3 %。 下面对几例典型病例加以讨论: 例1 DRA的脊柱关节病 男性患者，70岁，1984年2月开始腹膜透析，1986年改为血液透析。1998年2月出现双肩不定时痛，以夜间和透析时加重伴双手指麻木、颈部活动受限，症状进行性加重。1998年4月颈部X线检查:颈椎间隙变窄，邻近椎板无明显 改变;CT及MRI检查:齿状突膨大， 项韧带、黄韧带增厚，被软组织取代， 压迫颈端脊髓呈骨破坏性关节病变的 牙样侵蚀过程，同时CT及MRI检查指 骨、胫骨发现多发性小囊肿。如图1。 诊断:颈椎关节DRA(破坏性骨关节病) 讨论:颈椎关节损害是DRA时十分严 重的一种致残性并发症，比较少见，通 常发生于透析超过10年以上的患者， 图1 DRA的脊柱关节病 for computer software engineering GB/T50314-2000 of electrical installations code for construction and acceptance of GBJ232- 92 intelligent building weak design construction figure set GJBT-471 2. system description this times engineering weak design including: hours a Kok information facilities system integrated wiring system (containing room network, communications part) wired TV system information guide and the released system hours II Kok public security system fire automatically alarm system (containing public broadcast part) security technology prevention system security monitoring system out entrance control system car library management system hours three Kok building equipment monitoring system hours four Kok information application system queued station ICU visitation system operation the system monitoring and training system call intercom system hours, five hours Kok Kok intelligent integrated system of information system (1) cabling systems (including computer room network, communication component) computer network systems and voice communications systems Center of information on medical floor computer room located in the engine room. Voice communication system using a local telecommunications operator providing voice service, configuration-independent integrated services digital program-controlled switchboards. Operators providing communications ... If any compatibility requirements, by way of increased access to the Communications Board within the chassis to meet [2,3]。本透析中心2例颈椎破坏性骨关节患者均透析16年以上，其发生率占14% [3]占14.3%。与国外近似。颈椎关节呈破坏性关节病变的牙样侵蚀过程，骨质破坏常为多发并大量呈对称性分布，但X线平片较难发现。CT和MRI有助于发现普通X线上不易显示的病变。如枕部环枢椎关节与颈结合部位的骨质破坏，椎体后弓骨的透亮区在CT或MRI下可清楚见到该病侵犯齿状突根部，造成骨 [2,3]折的危险性增加。少数椎突后弓受累的患者可发生脊椎移位，造成脊神经根甚至脊髓压迫，以致截瘫而造成恶性后果。由于病变发展迅速，早期诊断脊柱关节DRA至关重要。伴有脊髓或神经根压迫的脊椎移位患者往往需接受融合矫形术治疗。 例2 DRA的囊性骨损害 男性患者，68岁，1986年开始血液透析。1992年出现双下肢痛、麻木感。 血磷6.2mg/dl; 双下肢X线平片检查:见双下肢动脉血管钙化;化验血钙8.6mg/dl，iPTH>1000pg /ml，ALP 300IU/L。1999年，双手X线平片检查:见双腕部血管钙化，双手普遍性骨质疏松，骨皮质变薄，多处指骨端可见小囊状骨质吸收;查血:β -MG 60ug/dl、 ALP 500IU/L;CT可见:肱骨头非持重面囊肿大于2 10mm(如图2a)，距骨囊肿(如图2b)，胫骨平台囊肿(如图2c)。 a) 肱骨头囊肿 b) 距骨囊肿 c) 胫骨平台 图2 DRA的囊性骨损害的CT图象 ormation system (1) cabling systems (including computer room network, communication component) computer network systems and vthe system monitoring and training system call intercom system hours, five hours Kok Kok intelligent integrated system of inf rationk building equipment monitoring system hours four Kok information application system queued station ICU visitation system opehnology prevention system security monitoring system out entrance control system car library management system hours three Koty tecreleased system hours II Kok public security system fire automatically alarm system (containing public broadcast part) securiies system integrated wiring system (containing room network, communications part) wired TV system information guide and the 471 2. system description this times engineering weak design including: hours a Kok information facilit-92 intelligent building weak design construction figure set GJBT -2000 of electrical installations code for construction and acceptance of GBJ232-T50314for computer software engineering GB/3to the Communications Board within the chassis to meet controlled switchboards. Operators providing communications ... If any compatibility requirements, by way of increased access-ramindependent integrated services digital prog-ystem using a local telecommunications operator providing voice service, configurationcommunications systems Center of information on medical floor computer room located in the engine room. Voice communication soice 诊断:DRA的囊性骨损害 讨论:DRA致囊性骨损害是长期透析患者一种致残性并发症，根据我们的经验指骨、腕骨、胫骨、肱骨头、股骨头和髋骨是最长受累的部位，多是滑膜关节附近，病变呈多发的软骨下溶骨性改变，严重时如发生在股骨头或髋臼处则易导致病理性骨折，以股骨头多见。文献报道骨骼 X线检查对骨的β -MG 淀粉样变2的特异性诊断:骨囊肿的大小在髋关节及肩关节要超过10mm，腕关节要超过5mm，骨囊肿往往位于髋臼的非持重面;受累骨临近的关节间隙正常，可与骨关节炎区别，当骨囊肿大小符合上述，而影响持重面或滑膜受侵时，若骨囊肿 [1]的直径每年递增30%，才有诊断意义。对于β -MG 淀粉样变的诊断，至少存2 在二个有意义的骨损害。CT扫描和MRI显示骨皮质破坏，有助于区别淀粉样囊性病变和其它原因造成的骨腔隙，并能发现普通X线不易显示的病变，如肱骨及股骨内囊性变，枕部环枢区椎关节与颈结合部位的骨质破坏,椎体后弓骨的透亮区可清楚看到。MRI对滑膜、韧带等软组织敏感度高，为判断骨囊性损害的程度提供了可靠的测量方法。当负重关节的破坏性关节病变严重时可行关节置换术。 例3 腕管综合征 女性患者，61岁，1990年开始血液透析。1998年出现双腕关节肿痛，夜间和透析时加重，以右腕肿痛明显。双手大小鱼际肌萎缩。查:β -MG 60.8 ug/dl。2 间断做过血液滤过，双手腕关节肿痛有所好转。双手X线平片检查:见双手腕骨、掌骨间隙变窄，骨皮质变薄，骨质疏松，多发性腕骨虫蚀样破坏并软组织肿胀，骨囊性变(如图3);CT检查:腕骨、指骨可见多发小囊肿;肌电图:神经原性损害(符合周围神经病变、右正中神经、胫神经)。 for computer software engineering GB/T50314-2000 of electrical installations code for construction and acceptance of GBJ232- 92 intelligent building weak design construction figure set GJBT-471 2. system description this times engineering weak design including: hours a Kok information facilities system integrated wiring system (containing room network, communications part) wired TV system information guide and the released system hours II Kok public security system fire automatically alarm system (containing public broadcast part) security technology prevention system security monitoring system out entrance control system car library management system hours three Kok building equipment monitoring system hours four Kok information application system queued station ICU visitation system operation the system monitoring and training system call intercom system hours, five hours Kok Kok intelligent integrated system of information system (1) cabling systems (including computer room network, communication component) computer network systems and voice communications systems Center of information on medical floor computer room located in the engine room. Voice communication system using a local telecommunications operator providing voice service, configuration-independent integrated services digital program-controlled switchboards. Operators providing communications ... If any compatibility requirements, by way of increased access to the Communications Board within the chassis to meet 诊断:腕管综合征(Carpal Tunnel Syndrome CTS ) 讨论: CTS是DRA的早期特征性表现，透析5年即 可出现，随透析时间的延长其发生率增加，透析15 [8]，其症状主要是由腕部横韧带或手年可高达100% 指屈肌滑膜的,-MG淀粉样沉积所致。由于,-MG淀22图3 腕骨虫蚀样破坏 粉样沉积于腕部的滑膜部位，造成腕管腔相对狭小， 腕管内压上升，出现压迫症状。早期表现为:双侧手部正中神经分布区的感觉异常、手痛、麻木，症状于夜间及透析时加重，伴手部活动障碍。如压迫未能及时解除，将会发展为掌部关节病变伴运动障碍，屈指肌腱淀粉样物沉积后粘连、断 [8]裂造成不可逆手指挛缩、鱼际肌萎缩，最后手功能丧失。X线检查:在腕关节囊肿要超过5mm和腕骨虫蚀样破坏;CT检查:腕骨、指骨有多发性囊肿样改变。关于CTS，一般主张早期手术，以免造成不可逆的神经肌肉损伤。目前，国内外对腕管综合征及肩关节病变均采用内窥镜切除局部韧带的治疗方法，但容易复发。 论 讨 血液透析是慢性肾功能衰竭患者得以维持生命的主要治疗方法。随着透析技术的普及和治疗水平的提高，透析患者的生存率逐年提高，人们也越来越注意患者的生活质量。DRA是长期透析患者的致残性严重并发症，重点在于预防，目前尚无特效治疗方法。主张采用综合治疗改善症状:如用非甾类消炎止疼药、激素 [3]可以缓解症状，但不能减少患者体内的,微球蛋白。高效透析器或血液透析滤2 过(HDF)能有效地清除血液中的β2-MG;联机HDF有可能成为防治β2-MG沉积的 [3]重要手段。采用B超检查可早期发现DRA的证据，CT或MRI检查有助于发现X线不易显示的颈椎关节DRA(破坏性骨关节病)。因此，及早发现DRA和及时治疗，对提高长期透析患者生存率和生活质量有着重要的临床意义。 参 考 文 献 the system monitoring and training system call intercom system hours, five hours Kok Kok intelligent integrated system of inf rationk building equipment monitoring system hours four Kok information application system queued station ICU visitation system opehnology prevention system security monitoring system out entrance control system car library management system hours three Koty tecreleased system hours II Kok public security system fire automatically alarm system (containing public broadcast part) securiies system integrated wiring system (containing room network, communications part) wired TV system information guide and the 471 2. system description this times engineering weak design including: hours a Kok information facilit-92 intelligent building weak design construction figure set GJBT -2000 of electrical installations code for construction and acceptance of GBJ232-T50314for computer software engineering GB/5to the Communications Board within the chassis to meet controlled switchboards. Operators providing communications ... If any compatibility requirements, by way of increased access-ramindependent integrated services digital prog-ystem using a local telecommunications operator providing voice service, configurationcommunications systems Center of information on medical floor computer room located in the engine room. Voice communication soice ormation system (1) cabling systems (including computer room network, communication component) computer network systems and v 1. 侯凡凡. 透析相关性淀粉样变诊断与治疗的进展. 中华内科杂志, 1997, 36: 135-137. 2. 杨燕. 超声诊断透析相关性淀粉样变. 中华超声影像学杂志, 2000, 9: 240-242. 3. Jadoul M,Carbar C,et al.hlc C, Pathological aspects of beta2-microglobulin amyloidosis.Semin Dial,2001,14(2):86-89. for computer software engineering GB/T50314-2000 of electrical installations code for construction and acceptance of GBJ232- 92 intelligent building weak design construction figure set GJBT-471 2. system description this times engineering weak design including: hours a Kok information facilities system integrated wiring system (containing room network, communications part) wired TV system information guide and the released system hours II Kok public security system fire automatically alarm system (containing public broadcast part) security technology prevention system security monitoring system out entrance control system car library management system hours three Kok building equipment monitoring system hours four Kok information application system queued station ICU visitation system operation the system monitoring and training system call intercom system hours, five hours Kok Kok intelligent integrated system of information system (1) cabling systems (including computer room network, communication component) computer network systems and voice communications systems Center of information on medical floor computer room located in the engine room. Voice communication system using a local telecommunications operator providing voice service, configuration-independent integrated services digital program-controlled switchboards. Operators providing communications ... If any compatibility requirements, by way of increased access to the Communications Board within the chassis to meet