Homozygosity for the transthyretin-Met30-gene in seven individuals with familial amyloidosis with polyneuropathy detected by restriction enzyme analysis of amplified genomic DNA sequences

Familial amyloidotic polyneuropathy (FAP) with a mutation in position 30 of transthyretin (TTR) (previously called prealbumin) is an autosomal dominant inherited disorder characterized by varying degrees of peripheral neuropathy, nephropathy, gastrointestinal problems, and vitreous amyloid. We have earlier diagnosed homozygosity for the TTR-Met30-gene using Southern analysis in four Swedish individuals. We have found it possible to detect homozygosity for the Met-30 mutation by amplifying discrete regions of the TTR-gene using polymerase chain reaction (PCR), and the amplification products restricted with NsiI analysed by gel electrophoresis. Clinical data on seven homozygous individuals, including three new cases, are presented.     

全植入式药泵行肝血管灌注治疗晚期肝癌

对２４例不能切除的晚期肝癌采用完全植入式药泵行肝动脉和门静脉双灌注化疗。结果：部分缓解（ＰＲ）１５例（６２．５％），稳定（Ｓ）８例（３３．３％），进展（Ｐ）１例。平均生存期为９．１７月，半年生存率为６６．７％，１年为２５％。在ＡＦＰ＞４００μｇ／Ｌ的１０例中，５例下降。     

联合断流术治疗食管静脉曲张出血的临床研究及远期疗效观察

自1981年1月以来,行联合断流术治疗门脉高压症食管静脉曲张出血102例,手术死亡率为6.9%。随访1～8年的生存率为85.7%,食管静脉曲张减轻或消失者为90%,未发现肝性脑病症状。远期死亡9例(14.3%),再出血4例(6.3%)。资料表明,这种手术既阻断了胃外又阻断了胃壁内的门-奇静脉交通血流,止血效果肯定而持久。由于未降低门静脉压力,故有利于维持肝脏的血流灌注。肝功能状况是决定治疗效果的主要因素。文章提出了选择手术适应证的条件。     

Die verwendung der autologen vena femoralis bei vena-cava-superior-thrombose

We report on a 52-year old male patient who underwent implantation of an insulin pump because he had diabetes and in whom superior vena cava syndrome developed as a long-term complication. After unsuccessful lysis therapy superficial femoral vein was implanted to form a bypass from the internal jugular vein to the right atrium. The postoperative course was uneventful from the aspects of both the bypass and the leg after explantation of the superficial femoral vein. The bypass is still patent 7 months after the operation, and the patient has no symptoms. Autogenous superficial femoral vein can be used successfully in the reconstruction of large venous vessels. It should be the graft of choice for young patients with benign diseases. We compare our result and those obtained with different substitutes described in the literature that have been used for reconstruction in superior vena cava syndrome.

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Die verwendung der autologen vena femoralis bei vena-cava-superior-thromboseFallbericht mit literaturübersicht

Zusammenfassung Bei einem 52jährigen, männlichen Patienten trat nach Implantation einer Insulinpumpe als Spätkomplikation eine Thrombose der Vena cava superior auf. Nach erfolgloser Lysetherapie wurde ein Venajugularis-rechtsatrialer-Venenbypass mit autologer Vena femoralis durchgeführt. Der postoperative Heilungsverlauf war sowohl von Seiten des gefäßrekonstruktiven Eingriffs, als auch von Seiten der Venenentnahmestelle komplikationslos. Der Bypass ist 7 Monate postoperativ offen und der Patient beschwerdefrei. Die autologe Vena femoralis eignet sich zur Rekonstruktion großer venöser Gefäße ausgezeichnet. Sie sollte v. a. bei benignen Prozessen und jüngeren Patienten als autologes Bypassmaterial dem Kunststoff vorgezogen werden. Das Ergebnis wird mit verschiedenen Gefäßsubstituten, die bei Verschluß der oberen Hohlvene verwendet werden, verglichen.

     

A Decrease in Pulmonary Vein Diameter After Radiofrequency Ablation Predicts the Development of Severe Stenosis

A decrease in ostial pulmonary vein (PV) diameter was observed in patients on the day after radiofrequency ablation of atrial fibrillation (AF). This study examined whether a relative reduction in PV diameter on day 1 (RRPVD1) after the procedure predicts the late development of severe PV stenosis (PVS). The study included 104 consecutive patients (mean age = 55 years, range 46–61, 34 women) with drug refractory AF. Pulmonary vein diameter was measured using MR angiography (MRA) on the day before and on day 1 after the ablation procedure. The MRA was repeated every 3 months after the procedure. Severe PVS was defined as a >70% diameter reduction from the initial ostial diameter. The cut-off of RRPVD1 was prespecified as 25% decrease in initial diameter. The data are presented as medians and interquartile range. A total of 357 PV were treated. The RRPVD1 was 0.0% (0.0–11.1%). Severe PVS was found in 18 PV during a follow-up of 12 months (range 6–13). The log-rank analysis confirmed a strong association between a RRPVD1 ≥25% and the development of PVS (hazard ratio: 7.1; 95% confidence interval 3.8–13.5, P < 0.0001). By multivariate Cox regression model, after adjustment of procedure variables, RRPVD1 was the strongest predictor of development of severe PVS. RRPVD1 ≥25% was a strong independent predictor of development of severe PVS.     

门静脉高压经肾静脉自发性分流的多层CT诊断

目的：探讨肝硬化门静脉高压时经肾静脉自发分流的CT表现与诊断。方法：220例临床确诊为肝硬化门静脉高压的患者行多层螺旋CT检查，扫描参数：准直／有效层厚／重建间隔为5mm／6．5mm／2．0mm(180例)或2．5mm／3．2mm／1．6mm(40例)。双期增强扫描：动脉期延迟26～28s，门脉期延迟55～60s。对图像进行电影显示，对有异常血管的病例分别完成薄层块最大强度投影(TS-MIP)和容积显示(VR)三维血管成像。结果：经肾静脉分流26例(11．8％)，其中脾左肾静脉分流20例，胃左肾静脉分流16例(同时有此2种分流12例)，门脉右肾静脉分流2例。肾门周围静脉增多、增粗7例。结论：自发性门脉-肾静脉分流并不少见，多层螺旋CT增强扫描及血管造影可有效显示分流血管，有助于指导临床诊断和治疗。     

肝硬化门静脉高压症内毒素血症的治疗

目的　研究肝硬化门静脉高压症 (PHC)病人内毒素血症治疗方法及其对预后的影响。方法　选 3 6例 PHC择期手术病人 ,随机分为治疗组 (n=18)和对照组 (n=18) ;治疗组入院后除保肝、纠正低蛋白血症及贫血等术前一般准备外 ,另行每日服用中药茵陈小承气汤和稀生理盐水碘伏液灌肠术等 ,对照组行 PHC的常规术前准备。观察两组病人术前血浆内毒素(PE)水平变化和术后全身炎症反应综合症 (SIRS)、多脏器障碍综合症 (MODS)的发生率等。结果　入院时所有 PHC病人均存在不同程度的内毒素血症。经过术前准备 ,治疗组术前 PE水平由 0 .13 9± 0 .0 2 2 Eu/ m l降至 0 .12 2± 0 .0 2 4 Eu/ m l(P<0 .0 5 ) ,对照组则下降不明显 (P>0 .0 5 )。术后治疗组的 SIRS、MODS的发生率和死亡率分别为 :11.1%、5 .6%、0 % ;对照组则分别为 :4 4 .4 %、2 7.8%、5 .6%。术后胃肠功能的恢复时间 :治疗组 (78.3± 2 1.2 h)比对照组 (13 8.4± 4 2 .5 h)显著缩短 (P<0 .0 0 1)。结论　应用茵陈小承气汤结合稀生理盐水碘状液清洁胃肠道 ,可以降低 PHC病人由于细菌易位所致的内毒素血症以及其对机体的损害 ,从而减少 PHC病人术后 SIRS、MODS的发生率及死亡率     

下腔静脉滤器在治疗下肢深静脉血栓中的应用

目的：探讨下腔静脉滤器在治疗下肢深静脉血栓(DVT)中的应用价值。方法：选择10例DVT患，使用进口铁质滤器，经股静脉或颈静脉途径置人下腔静脉，随访8—20个月。结果：10例患中无一例发生肺动脉栓塞和其他严重并发症。结论：下腔静脉滤器置人方法简单，安全；可有效防止肺动脉栓塞的发生。     

Bilocular atrial malignant mesenchymoma causing mitral and localized pulmonary vein flow obstruction: diagnosis by transoesophageal echocardiography.

Cardiac malignant mesenchymoma is an extremely rare malignancy with poor prognosis. We report a patient presenting with a history and clinical findings typical of mitral stenosis. Transthoracic echocardiography showed a mass on the thickened posterior mitral leaflet. Transoesophageal echocardiography revealed two tumoural masses: one on the atrial side of the posterior mitral leaflet causing mitral obstruction, the other arising in the region of the right lower pulmonary vein orifice and obstructing inflow through this vein.     

Effect of water restrictions on the physiological parameters,psychological behavior and brain c-Fos expression in rat

1 Introduction Exposure to hostile stressors causes a series of coor- dinated responses in the body, such as alterations of neu- roendocrine secretion, immune reaction and behavioral manifestation to maintain homeostasis stability and sur-vival of the organisms. Stressors are divided into two main categories: physical, or systemic, and psychological, or emotional / processive. Each stressor might activate a spe- cific central pathway to induce a special neuroendocrine response, even cause stre…