四肢恶性骨肿瘤保肢手术失败的原因分析

目的 探讨四肢恶性骨肿瘤保肢手术存在的诊疗失误,分析保肢手术失败的原因.方法 回顾性分析2005 年4 月至2010 年6 月新疆医科大学附属肿瘤医院收治的16 例四肢恶性骨肿瘤患者保肢手术时存在的诊断和治疗缺陷.结果 16 例患者保肢手术失败的原因包括：（1）对四肢恶性骨肿瘤疾病缺乏认识;（2）病理诊断困难;（3）对新辅助化疗及术后化疗认识不足;（4）对四肢恶性骨肿瘤保肢手术适应证把握不恰当;（5）肿瘤切除的手术边界不足;（6）肿瘤切除后重建方式选择不当;（7）活检通道选择错误.结论 对四肢恶性骨肿瘤患者实施保肢手术应尽量避免上述诊疗缺陷,提高保肢手术的成功率.     

恶性骨肿瘤保肢治疗中存在的问题

 恶性骨肿瘤的治疗已进入一个比较成熟的阶段,新辅助化疗及广泛性切除的理念已取得广泛的共识。保肢手术已成为治疗肢体恶性骨肿瘤的经典方法。新辅助化疗能消灭微小转移灶并缩小原发肿瘤,有利于随后的保肢治疗,还可能通过评估肿瘤对化疗的反应,提供肿瘤对化疗敏感性的信息。恶性骨肿瘤的治疗目标是既要提高患者的生存率,又要保存良好的肢体功能。保肢手术首先要避免局部复发,其次是尽可能多地保留肢体功能。国内近20年来骨肿瘤的治疗已取得很大的进步,新辅助化疗的概念、广泛性切除的原则和方法已得到广泛应用,恶性骨肿瘤患者的生存率有了显著地提高。 误诊误治仍然是目前存在的一个严重问题。其主要原因：(1)骨与软组织肿瘤发病率较低,部分医生认识不足,缺乏足够的临床诊治经验;(2)医生没有按照已有的诊疗规范进行临床工作,如术前未进行肿瘤组织活检,将恶性肿瘤当作良性肿瘤进行病灶内刮除手术,造成肿瘤的局部污染及扩散, 使再次手术很难达到广泛性切除的要求,患者治愈的可能性大大减小,甚至不得不实施截肢手术。 对恶性骨肿瘤实施广泛性切除包含两个层面的含义：(1)对广泛性切除概念的理解,(2)广泛性切除手术的具体操作,由于不同医生手术操作技巧的差异而出现不同的结果。根据国际保肢学会(ISOLS)的要求,保肢手术的局部复发率应控制在8%以下。因而,保肢手术应由熟练的专科医生完成。目前,国内存在的问题是许多保肢手术是由非专业医生进行,局部复发率很高,由此导致生存率的下降。另外,由于对安全边界概念、外科分期及外科边界在术前计划中的重要性理解不足,导致术中切除范围不够。     

椎间盘镜下肢体骨肿瘤活检的初步分析

[目的]对椎间盘镜下肢体骨肿瘤活检进行疗效评估、[方法]以2008年10月～2011年8月来本院治疗骨肿瘤的患者总共80例为研究对象,所有患者均行择期手术,行椎间盘镜下骨肿瘤活榆手术.[结果]所有患者术前均行椎间盘镜下活检手术,取材成功率100％.活检准确率达96.25％,其中良性肿瘤准确率为96.26％；恶性肿瘤为97.22％ . 活检手术均未发生并发症.[结论]椎间盘镜下骨肿瘤活检术对骨肿瘤术前的诊断及治疗方法的选择有重要作用,且具有操作简便、并发症少、准确率高的特点.     

肩胛部恶性肿瘤保肢治疗

目的探讨肩部骨肿瘤保肢治疗的手术方法。方法对2例肩部恶性肿瘤实施保肢术，肱骨近端成骨肉瘤1例，行肱骨瘤段切除，锁骨翻转重建骨缺损，肩胛骨恶性纤维组织细胞瘤1例，行肿瘤切除、肱骨肩峰悬吊术。结果术后短期随访，肩关节被动活动有一定的功能。结论肩部恶性肿瘤应根据肿瘤发生的不同部位，患者的经济实力，选择不同保肢手术，目的是使肩部保留一定的功能和外形，提高患者生活的质量。     

骨的恶性纤维组织细胞瘤诊治分析

[目的]探讨骨的恶性纤维组织细胞瘤的临床特点、诊断及治疗方法。[方法]回顾性分析52例骨的恶性纤维组织细胞瘤患者的临床资料,包括发病部位、术前影像学检查、穿刺活检、肿瘤外科分期、治疗方法以及随访结果等。[结果]52例患者肿瘤位于膝关节周围32例、肩关节周围14例、股骨近端3例,跟骨、腰椎、髂骨各1例。术前常规行X线片、CT及MRI,并行穿刺活检。根据Enneking肿瘤外科分期,20例属于ⅡA期,32例属于ⅡB期。所有病例除1例保守治疗外,其余均行手术治疗,以保肢术为主,保肢率74.5%(38/51),同时进行术前、术后化疗及术后放疗。36例患者经过长期随访,随访率69.2%(36/52),其中局部复发9例,远处转移6例,5年生存率61.1%(22/36)。[结论]术前穿刺活检结合影像学检查对恶性纤维组织细胞瘤的诊断有很大帮助,治疗以保肢手术为主,术前、术后化疗以及术后放疗可以防止肿瘤复发,肿瘤的彻底切除是骨的恶性纤维组织细胞瘤治愈的关键。     

骨关节假体在四肢恶性骨肿瘤保肢手术中的应用

随着有效的新辅助化疗在临床治疗中的广泛应用，大大减少了恶性骨肿瘤的转移，提高了患者的5年生存率，从而为保肢手术提供了强有力的保证，保肢手术已经成为四肢恶性骨肿瘤的规范治疗方式。保肢手术应确保局部复发率不比截肢手术高，并且可以产生良好的功能结果。因此，术中必须对肿瘤进行广泛切除，但这样往往会造成严重的骨关节缺损。人工骨关节假体置换术是目前保肢术中重建骨关节的完整性，保留肢体功能的最有效的方法之一。     

恶性骨肿瘤保肢术中应注意的几个问题

探讨恶性骨肿瘤保肢术中应的几个问题。方法：自１９９１年６月，对６４例恶性骨肿瘤行保肢手术，男４１例，女２３例，年龄平均２９．９岁，ⅠＡ２型，ⅠＢ５例，ⅡＡ１２例，ⅡＢ４０例，ⅢＢ５例。５８例获得平均２１．９个月的随访。结果：５例发生肺转移，５例死亡，６例复发。保持手术成功率为７９．３％，肢体功能优良率为７６．７％。．     

脾脏占位性病变29例的临床分析

目的探讨脾脏占位性病变临床诊断和治疗。方法回顾分析1996年1月至2006年12月浙江金华中心医院肝胆外科手术治疗29例脾脏占位的临床资料。结果29例病人中良性19例，恶性10例。19例良性肿瘤中15例行脾切除术1例行脾部分切除术，3例腹腔镜下脾囊肿开窗术。10例恶性肿瘤中8例行脾切除术，1例累及肝左外叶及胃，同时行胃部分切除和肝左外叶切除术。1例脾血管肉瘤因肿瘤巨大并与周围脏器粘连严重，且肝脏有转移结节，无法切除而取活检。结论脾脏占位术前定位诊断较容易，定性较为困难。脾良性肿瘤可根据情况行脾切除或脾部分切除术，恶性肿瘤应采用以手术为主的综合治疗。     

微波治疗恶性骨肿瘤保肢手术病人的护理

对16例恶性或侵袭性骨肿瘤病人,用植入微波天线阵灭活骨肿瘤的方法进行保肢手术,配合精心护理,1年生存率和3年生存率分别为81.2%和43.8%,病人肢体功能恢复良好.提出术前需做好心理护理及化疗护理,术后加强病情观察及功能锻炼.     

恶性骨肿瘤保肢治疗的原则及问题

保肢手术已成为治疗肢体恶性骨肿瘤的经典方法。保肢治疗的目的是不但要提高患者的生存率，而且要保存良好的肢体功能。保肢手术的第一个目的是避免局部复发，第二个目的是尽可能多地保留功能。治疗格言为：“肿瘤第一，功能第二”。国内近20年来骨肿瘤的治疗已经取得了很大的进步，     

The following abstracts were submitted for the 2nd Congress of the International Society for Rotary Blood Pumps, held September 23–25, 1994, Vienna, Austria, but papers were not submitted.

1 AXIAL FLOW BLOOD PUMP FOR CHRONIC IMPLANT USE. K. Butler. T. Maher, H. Borovetz,* P. Litwak,* and R. Kormos,* Nimbus, Inc., U.S.A., and *University of Pittsburgh, U.S.A. 2 A NEW APPLICATION OF A ROTARY PUMP IN A SIMULTANEOUS ADSORPTION/FILTRATION PROCESS. D. Falkenhagen, C. Weber, Ch. Rajnoch, H. Schima,* F. Loth. ?Interdisciplinary Institute of Bioengi-neering, Danube University, Krems, Austria, *Centre of Biomedical Research, University of Vienna, Austria, and ?Fraunhoferinstitute of Polymerresearch, Teltow, Germany. 3 TESTING AND MEASUREMENT OF THE PERISTALTIC PUMP FOR THE EXTRACORPOREAL CIRCULATION. Z. Kratochvil and P. Fleischner, Department of Hydraulic Machines and Equipment, Technical University of Brno, Faculty of Mechanical Engineering, Czech Republic 4 SIMULATION OF THE CARDIOVASCULAR SYSTEM AND SOME POSSIBILITIES OF THE BLOOD PUMP SYSTEM OPTIMIZATION. J. Nevrlv . Technical University of Brno, Czech Republic. 5 VIBRATORY ORBITING BLOOD PUMP. A.J. Sipin . Anatole J. Sipin Co., Inc. 6 MECHANICAL HEMOLYSIS DERIVED IN SEVERAL TYPES OF STENOTIC TUBES BY USING A CENTRIFUGAL PUMP. M. Umezu, Department of Mechanical Engineering, Waseda University, Tokyo, Japan. 7 BASIC PERFORMANCE OF A MINIATURE INTRA-VENTRICULAR AXIAL PUMP. M. Umezu . Y. Otake, K. Sakata, T. Fujimoto, K. Yamazaki,* H. Koyanagi,* H. Iiyama.? T. Mori,? K. Higuchi.? Department of Mechanical Engineering, Waseda University, Tokyo, Japan, *Heart Institute of Japan, Tokyo, Japan, and ?Sun Medical Technology Research Corp., Nagano, Japan. 8 A FLUID DYNAMIC ANALYSIS OF THE BAYLOR/ NASA AXIAL FLOW BLOOD PUMP FOR DESIGN IMPROVEMENT. J.-T. Wernicke . D. Meier, K. Mizugu-chi, G. Damm, G. Aber,* B. Benkowski, Y. Nose, G. P. Noon, and M. E. DeBakey, Department of Surgery, Baylor College of Medicine, Houston, TX, U.S.A. and *NASA/Johnson Space Center, Houston, TX, U.S.A. 9 USE OF HEPARIN-COATED DEVICES: IS HEPA-RINIZATION STILL NECESSARY?: A CASE REPORT. G. Wieselthaler . H. Schima, R. Seitelberger, D. Heilinger,* E. Donner, M. Hiesmayer,* and E. Wolner, Department of Cardiothoracic Surgery, LBI for Cardio-surgical Research, and *Department of Cardiothoracic Anesthesiology, University of Vienna. 10 PULSATILE VERSUS NONPULSATILE PERFU-SION USING A CENTRIFUGAL PUMP FOR CAR-DIOPULMONARY BYPASS DURING CABG EFFECTS ON HEMODYNAMICS, OXYGENATION, AND INFLAMMATORY RESPONSE. J. Driessen . H. Dhaese, L. Rondelez, G. Fransen, and L. Gevaert, St. Jans Hospital, Brugge, Belgium.     

激光直接心肌隧道术治疗缺血性心脏病实验研究

研究钬激光和高功率二氧化碳激光在急性缺血心肌上产生穿透室壁全层隧道对心肌缺血的保护作用。以２０ｋｇ体重Ｙｏｒｋｓｈｉｒｅ猪分为钬激光组（７只），ＣＯ２激光组（１０只）及对照组（８只）进行实验。结果表明：钬激光无法产生满意的激光隧道，但有诱发新生血管形成的可能；高功率二氧化碳激光能够产生理想的激光隧道，对急性缺血心肌提供血液灌注，并可避免室颤的发生。但远期通畅仍有待解决。     

Acknowledgement

The Editor of Cerebral Cortex would like to thank the followingreviewers who have helped us in 2004. Abbott, Laurence Abraham, Wickliffe C. Aghajanian, George Aine, Cheryl Aizenstein, Howard Allen, John Allman, John Alloway, Kevin Alonso, Jose Manuel Amit, Daniel Andersen, Richard Anderson, Charles H. Andrews, Sally Ang, Eugenius Anton, Eva Aoki, Chiye Apkarian, Apkar Arieli, Amos Ashburner, John Ashe, James Astafiev, Serguei V. Averbeck, Bruno B. Ayoub, Albert Baciu, Monica Baker, Curtis Balaban, Evan Banich, Marie Bao, Shaowen Barash, Shabtai Barbas, Helen Barnes, Carol Barone, Pascal Barrionuevo, German Barth, Daniel Barto, Andrew G. Basar, Erol Basso, Michele A. Baxter, Mark Behar, Toby Belger, Aysenil Belin, Pascal Benson, Deanna L. Benveniste, Helene Berman, Karen Bernstein, Lynne Binder, Jeffrey Binkofski, Ferdinand Birbaumer, Niels Black, Sandra Blakemore, Sarah-Jane Blankenburg, Felix Bliss, Timothy Blood, Anne Blumenfeld, Hal Blumstein, Sheila Blusztajn,     

Health-related quality of life in patients with adolescent idiopathic scoliosis after treatment: short-term effects after brace or surgical treatment

For treatment of teenagers with progressive adolescent idiopathic scoliosis in an early stage, two options are generally considered: treatment with a brace or observation followed by surgery if necessary. Many doctors and patients prefer conservative treatment (i.e. brace treatment) to surgical treatment, because surgery of the spine is generally considered a drastic intervention. Because potential differences in health-related quality of life (HRQoL) after treatment between braced and surgically treated patients are not well explored, this study aimed to determine whether short-term differences exist in HRQoL between adolescents treated with a brace or treated surgically. A cross-sectional analysis of HRQoL was made of 109 patients with adolescent idiopathic scoliosis who, after completing treatment, filled out the Dutch SRS-22 Patient Questionnaire. All patients had been treated either with a brace or surgery, or with a brace followed by surgery. Patients treated surgically had significantly higher mean scores in the satisfaction with management domain than those treated with a brace. No other consistent differences in HRQoL were found between patients treated with a brace and patients treated surgically. Gender, curve type and curve size had no relevant effect on HRQoL. We conclude that short-term differences in HRQoL after treatment in adolescent patients with idiopathic scoliosis are negligible and cannot support preference of one treatment above the other. The NESCIO group: H.D. Been, MD, PhD, L.N.J.E.M. Coene, MD, PhD, H. Creemers, MD, A.J. de Gruijter, MD, PhD, A.A.J.M. Hazebroek-Kampschreur, MD, PhD, P. Klop, MD, H.J.A. Kruls, MD, PhD, P.J.M. van Loon, MD, L.C.F. Luttmer, MD, F. de Nies, MD, J.E.H. Pruijs, MD, PhD, L.W. van Rhijn, MD, PhD, M.P. Teeuwen, MD, P.A. Wiegersma, MD, PhD.     

Perioperative mortality and morbidity in the year 2000 in 502 Japanese certified anesthesia-training hospitals: with a special reference to ASA-physical status--report of the Japan Society of Anesthesiologists Committee on Operating Room Safety

Perioperative mortality and morbidity in Japan from Jan. 1 to Dec. 31, 2000 were studied retrospectively. Committee on Operating Room Safety in Japanese Society of Anesthesiologists (JSA) sent confidential questionnaires to 794 certified training hospitals of JSA and received answers from 67.6% of the hospitals. We analyzed their answers with a special reference to ASA physical status (ASA-PS). The total number of anesthesia available for this analysis was 897,733. The percentages of patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E are 38.0, 40.3, 8.5, 0.4, 4.3, 5.3, 2.5, and 0.7%, respectively. Mortality and morbidity from all kinds of causes including anesthetic management, intraoperative events, co-existing diseases, and surgical problems were as follows. The incidences of cardiac arrest (per 10,000 cases of anesthesia) were 1.11, 3.26, 12.25, 54.60, 0.77, 4.46, 21.08 and 217.75 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The incidences of critical events including cardiac arrest, severe hypotension, and severe hypoxemia were 6.89, 20.22, 62.18, 148.21, 6.71, 20.38, 106.72 and 592.21 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The mortality rates (death during anesthesia and within 7 postoperative days) after cardiac arrest were 0.26, 0.77, 3.69, 41.60, 0.00, 1.06, 9.42 and 163.31 per 10,000 cases of anesthesia in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The overall mortality rates were 0.32, 1.38, 9.75, 70.20, 0.26, 2.12, 29.15 and 353.02 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. Overall mortality and morbidity were higher in emergency anesthesia than in elective anesthesia. ASA-PS correlated well with overall mortality and morbidity, regardless of etiology. The incidences of cardiac arrest totally attributable to anesthesia were 0.23, 0.50, 1.32, 0.00, 0.00, 0.85, 2.69 and 4.95 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The incidences of all critical events totally attributable to anesthesia were 3.13, 5.56, 11.46, 5.20, 3.87, 5.94, 13.90 and 14.85 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The mortality rates after cardiac arrest totally attributable to anesthesia were 0.03, 0.03, 0.00, 0.00, 0.00, 0.21, 0.45 and 3.30 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The overall mortality rates totally attributable to anesthesia were 0.03, 0.06, 0.00, 0.00, 0.00, 0.21, 0.45 and 6.60 in patients with ASA-PS of I, II, III, IV, I E, II E, III E, and IV E, respectively. The overall mortality rate totally attributable to anesthesia among patients with good physical status (ASA-PS of I, II, I E, II E) was 0.05. Anesthetic management was mainly responsible for critical events in patients with good physical status, while coexisting diseases were in those with poor physical status. Surgical problems including procedures and massive hemorrhage were the leading causes of mortality in patients with good physical status. We reconfirmed that ASA-PS is useful to predict perioperative mortality and morbidity. It also seems likely that we should make much more efforts to reduce anesthetic morbidity in patients with good physical status, and to improve preanesthetic assessment and preparation in those with poor physical status. Reducing mortality and morbidity from surgical problems is also required for improving perioperative mortality.     

Perioperative mortality and morbidity in 1999 with a special reference to age in 466 certified training hospitals of Japanese Society of Anesthesiologists--report of Committee on Operating Room Safety of Japanese Society of Anesthesiologists

Perioperative mortality and morbidity in Japan from Jan. 1 to Dec. 31, were studied retrospectively. Committee on Operating Room Safety of Japanese Society of Anesthesiologists (JSA) sent confidential questionnaires to 774 Certified Training Hospitals of JSA and received answers from 60.2% of the hospitals. We analyzed their answers with a special reference to the age group. The total number of anesthetics available for this analysis was 732,788. All cases were divided in to 7 groups; group A(< 1 months), group B(< 12 months), group C(< 5 years), group D(< 18 years), group E (< 65 years), group F(< 85 years), and group G(> 85 years). The incidences of all critical events including cardiac arrest, severe hypotension, and severe hypoxemia were 168.14, 47.86, 24.63, 14.65, 28.43, 50.4, and 43.68 per 10,000 in patients with group A, B, C, D, E, F, and G, respectively. The overall mortality rate (death during anesthesia and within 7th postoperative day) were 74.10, 6.63, 3.30, 3.07, 4.82, 13.74, and 11.84 per 10,000 anesthetics in patients with group A, B, C, D, E, F, and G, respectively. The incidences of cardiac arrest were 54.15, 8.84, 5.08, 2.56, 4.84, 11.02, and 6.66 per 10,000 in patients with group A, B, C, D, E, F, and G, respectively. The mortality rates after cardiac arrest were 42.75, 2.95, 2.54, 1.70, 2.00, 6.56, and 5.18 in patients with group A, B, C, D, E, F, and G, respectively. The incidences of all critical events, the incidence of cardiac arrest, and the overall mortality rate were much higher in group A than other groups and lower in group D. Mortality and morbidity due to all kinds of causes including anesthetic management, intraoperative events, co-existing diseases, and operation were as follows. The incidence of all critical events attributable to co-existing disease were the highest in these four groups, and 94.04, 15.46, 7.87, 6.13, 7.26, 17.38, and 16.29 per 10,000 in patients with group A, B, C, D, E, F, and G, respectively. The incidences of all critical events attributable to anesthetic management were 31.35, 16.94, 4.60, 6.09, 10.77, and 14.07 per 10,000 in patients with group A, B, C, D, E, F, and G, respectively. The incidence of cardiac arrest in group A was much more attributable to co-existing disease and operation than other causes. The incidences of cardiac arrest attributable to anesthetic management were 0.00, 1.47, 0.25, 0.34, 0.83, 0.92, and 0.22 per 10,000 in patients with group A, B, C, D, E, F, and G, respectively. The mortality rates in these groups were 0.00, 0.00, 0.00, 0.17, 0.07, 0.05, and 1.48, and no death was found in cases under 5 years of age. The two cases of death in G group were due to too high anesthesia levels in spinal anesthesia. Other causes including overdose of anesthetics, toxic effect of local anesthetic, improper management of airway, and incompatible blood transfusion were preventable with the anesthesiologists' effort in protocol development and skilled assistance.     

Autologous blood predeposit for elective surgery: An Italian experience

A program of predeposit autotransfusion in elective surgery was implemented with the main purpose of decreasing the incidence of posttransfusion hepatitis and of conserving homologous blood. Specific procedures and computer programs were designed to monitor the transfusion practice by key indicators, and the incidence of posttransfusion hepatitis and HTLV-III infections. Arrangements were devised to ensure the proper management of autologous and homologous units. In 1985, autologous units accounted for 13.5% of all units transfused in elective surgery. While encouraging, our results indicate that efforts have to be made to improve organization and increase awareness of the benefits of autotransfusion in the medical and lay communities.

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Resumen Un programa de autotransfusión predepositada en cirugía electiva ha sido organizado con el propósito de rebajar la incidencia de hepatitis postransfusional y de conservar sangre homôloga. Se diseñaron procedimientos especiales y programas de computación para la monitoría de las prácticas de transfusión por medio de indicadores claves, y la incidencia de hepatitis postransfusional y de infección por HTLV-III. Se establecieron arreglos especiales para asegurar el debido manejo de las unidades autólogas y homologas. En 1985, las unidades autólogas representaron el 13.5% de la totalidad de las unidades transfundidas en cirugía electiva. Aunque promisorios, los resultados indican que deben emprenderse esfuerzos orientados a mejorar la organización y a incrementar el conocimiento de los beneficios de la autotransfusión tanto entre los médicos como en la comunidad general.

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Résumé Un programme de transfusion reposant sur l'emploi du propre sang du malade (sang autologue) prélevé avant l'intervention a été mis en oeuvre au cours de la chirurgie élective. Son but principal est de réduire la fréquence de l'hépatite posttransfusionnelle et l'utilisation de sang homologue. Des méthodes spécifiques et programmées sur ordinateur ont été mises au point de manière à contrôler la pratique de la transfusion en fonction d'éléments clefs et aussi de contrôler la fréquence de l'hépatite post-transfusionnelle et des infections HTLV-III. Des dispositions ont été établies pour assurer l'emploi adéquat d'unités de sang du malade ou de sang homologue. En 1985, la méthode a été employée dans 13.5% des cas au cours de la chirurgie élective. Bien qu'encourageants, les résultats obtenus indiquent que les efforts doivent être poursuivis pour améliorer l'organisation de ce mode d'auto-transfusion et aussi pour améliorer ses résultats.

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Members of the Autotransfusion Team, 1985 B. Andreoni, F. Annoni, A. Anselmi, C. Arienta, C. Bagni, M. Baiguini, L. Baldini, L. Beretta, S. Berra, G. Bevilacqua, R. Biffi, L. Bigatello, V. Buzzetti, G. Cantaluppi, G. Cantoni, L. Ceretti, D. Chiurazzi, M. Citterio, C. Confalonieri, E. Consonni, E. Contessini Avesani, A. Cortelezzi, C. Crosta, Jr., M. Cugnasca, G. Damia, C. De Luca, P. De Rai, A. De Sanctis, S. Doldi, M. Erba, C. Ferrari, O. Ferri, N. Fraschini, S.M. Gaini, P.L. Giorgetti, G. Giuffrida, G. Gonnella, G. Granata, A. Inzaghi, G.L. Legnani, T. Longo, A. Mandressi, A. Mantovani, R. Marconato, M. Marinoni, R. Massei, A. Mattioli, M. Marzotto, M. Meriggi, M. Mezzetti, S. Miani, G. Miserocchi, W. Montorsi, A. Morbidelli, L. Morelli, R. Mozzana, E. Mozzi, A. Nespoli, M. Nosotti, A. Odero, N. Olivari, G.F. Pellegrini, G. Petrini, G. Pezzuoli, E. Pisani, M.N. Pizzi, S. Poma, P. Rampini, R. Ravagnan, E. Ronchetti, R. Rosati, R. Rossi, U. Ruberti, R. Russo, P. Salvini, M.G. Sandri, L. Santambrogio, V. Scortecci, R. Scorza, P. Settembrini, P. Setti Carraro, E. Sibilla, G. Signoroni, V.A. Sironi, A. Tajana, L. Tarenzi, A.M. Taschieri, P. Tombolini, G. Tomei, M. Tos, R. Trazzi, A. Trimboli Cataldo, A. Trinchieri, L. Vicentini, R. Villani, G. Vincre, A. Vinci, C.P. Voci, and M. Zavannone.

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See Acknowledgment for members of the Autotranfusion Team.

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Supported in part by a grant from Ministero della Sanità, Rome, Italy.     

多沙普仑,纳络酮对血流动力学影响的实验研究

观察了全麻催醒药多沙普仑（ｄｏｘａｐｒａｍ），纳络酮（ｎａｌｏｘｏｎｅ）对血流动力学的影响。犬分为多沙普仑组和纳络酮组，每组５只。采用Ｓｗａｎ－Ｇａｎｚ漂浮导管及心脏电脑监护仪等方法，观察动物用药前后血流动力学的变化。结果多沙普仑组（２ｍｇ／ｋｇ）用药后５分钟ＣＯ、ＣＩ、ＳＶ、ＬＶＳＷ、ＬＶＳＷＩ均明显高于用药前，外周阻力（ＳＶＲ）明显低于用药前，４５分钟后基本恢复至用药前水平。纳络酮组（０．０１５ｍｇ／ｋｇ）用药后５分钟ＣＯ、ＣＩ、ＳＶ、ＬＶＳＷ、ＬＶＳＷＩ明显低于用药前，ＳＶＲ则明显高于用药前，４５分钟尚未恢复至用药前水平。提示多沙普仑对血流动力学的影响优于纳络酮。     

No evidence for genotype/phenotype correlation in<Emphasis Type="Italic"> NPHS1</Emphasis> and<Emphasis Type="Italic"> NPHS2</Emphasis> mutations

Primary steroid-resistant nephrotic syndrome (SRNS) is characterized by childhood onset of proteinuria and progression to end-stage renal disease. In 26% of cases it is caused by recessive mutations in NPHS2 (podocin). Congenital nephrotic syndrome (CNS) is caused by mutations in NPHS1 (nephrin) or NPHS2. In three families mutations in NPHS1 and NPHS2 had been reported to occur together, and these tri-allelic mutations were implicated in genotype/phenotype correlations. To further test the hypothesis of tri-allelism, we examined a group of 62 unrelated patients for NPHS1 mutations, who were previously shown to have NPHS2 mutations; 15 of 62 patients had CNS. In addition, 12 CNS patients without NPHS2 mutation were examined for NPHS1 mutations. Mutational analysis yielded three different groups. (1) In 48 patients with two recessive NPHS2 mutations (11 with CNS), no NPHS1 mutation was detected, except for 1 patient, who had one NPHS1 mutation only. This patient was indistinguishable clinically and did not have CNS. (2) In 14 patients with one NPHS2 mutation only (4 with CNS), we detected two additional recessive NPHS1 mutations in the 4 patients with CNS. They all carried the R229Q variant of NPHS2. The CNS phenotype may be sufficiently explained by the presence of two NPHS1 mutations. (3) In 12 patients without NPHS2 mutation (all with CNS), we detected two recessive NPHS1 mutations in 11 patients, explaining their CNS phenotype. We report ten novel mutations in the nephrin gene. Our data do not suggest any genotype/phenotype correlation in the 5 patients with mutations in both the NPHS1 and the NPHS2 genes.Members of the Study Group of the Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN) participating in this study: J. Thaarup (Aalborg, Denmark); P. Henning (Adelaide, Australia); I. Attrach (Aleppo, Syria); A. Bakkaloglu (Ankara, Turkey); C. Rudin (Basel, Switzerland); R. Bogdanovic (Belgrade, Yugoslavia); S. Briese, J. Gellermann, T. Lennert, U. Querfeld, Sacherer, M. Schürmann, and M. Zimmering (Berlin, Germany); C. Roth, C. Schröter, and B. Utsch (Bonn, Germany); Matthes (Bremen, Germany); A. Heilmann and G. Kalvoda (Dresden, Germany); F. Wegner (Düren, Germany); V. Schumacher (Düsseldorf, Germany); Bär, B. Bosch, M. Kamm, S.M. Karle, K. Nüsken, C. Plank, W. Rascher, and B. Zimmermann (Erlangen, Germany); K. E. Bonzel, M. Bald, P. Hoyer, and U. Vester (Essen, Germany); U. Neyer (Feldkirch, Austria); Rippel (Frankfurt, Germany); M. Brandis, A. Fuchshuber, K. Häffner, A. Kirchhoff, and M. Pohl (Freiburg, Germany); J. Steiss (Giessen, Germany); J.P. Haas (Greifswald, Germany); L. Patzer (Halle, Germany); M. Kemper, H. Altrogge, D.E. Müller-Wiefel, U. Peters, and K. Timmermann (Hamburg, Germany); J.H.H. Ehrich, H. Haller, and C. Strehlau (Hannover, Germany); M. Daschner, S. Hessing, Janssen, D. Kiepe, S. Köpf, O. Mehls, and B. Tönshoff (Heidelberg, Germany); F. Prüfer and L.B. Zimmerhackl (Innsbruck, Austria); U. John, J. Misselwitz, G. Rönnefarth, and J. Seidel (Jena, Germany); D. Blowey and J. Scheinman (Kansas City, Mo., USA); B. Beck, K. Frankenbusch, B. Hoppe, C. Licht, D. Michalk, T. Ronda, and L. Stapenhorst (Cologne, Germany); D. Drozdz and A. Pogan (Krakau, Poland); Froster, E. Vogel and S. Wygoda (Leipzig, Germany); R. Hettenger (Los Angeles, Calif., USA); H. Schriewer and H.-P. Weber (Lüdenscheid, Germany); R. Beetz (Mainz, Germany); M. Konrad (Marburg, Germany); H. Fehrenbach (Memmingen, Germany); M. Griebel and B. Klare (München, Germany); M. Bulla, S. Fründ, E. Kuwertz-Bröking, A. Schulze-Everding and Yelbuz (Münster, Germany); L. Monnens (Nijmegen, The Netherlands); J. Janda and T. Seemann (Prag, Czech Republic); G. Adomssent, G. Krüger, Lorenzen, J. Muscheites, H.-J. Stolpe and M. Wigger (Rostock, Germany); W. Sperl (Salzburg, Austria); R. Egger (Schaffhausen, Switzerland); V. Tasic (Skopje, Macedonia); M. Bald and H.-E. Leichter (Stuttgart); O. Amon (Tübingen, Germany); T. Arbeiter, C. Aufricht and K. Müller (Vienna, Austria); D. Bockenhauer and N. Siegel (New Haven, Conn., USA); and T. Neuhaus and A. Staub (Zürich, Switzerland)     

Analysis of complications of radiofrequency pallidotomy

OBJECTIVE: To systematically report the complications of pallidotomy and to tentatively determine the incidences of complications of pallidotomy, possible influencing factors, and the acceptability of symptomatic hemorrhage rates for microelectrode-guided pallidotomy. METHODS: Clinical events were analyzed for 1116 patients with Parkinson's disease who underwent microelectrode-guided pallidotomies at our center. Complications included visual field deficits, weakness, fatigue, hypersomnia, drooling, dysphagia, speech disorders, hiccups, hemorrhage, seizures, apraxia, coma, infection, mental confusion, and impaired memory. Complication rates for bilateral pallidotomy and double-lesion groups were compared with those for unilateral pallidotomy and single-lesion groups, respectively. RESULTS: Among the total of 1116 patients, the incidences of visual field deficits, weakness, fatigue, hypersomnia, drooling, dysphagia, and speech disorders were 0.4, 4.2, 19.9, 12.4, 7.0, 3.7, and 11.9%, respectively. Symptomatic hemorrhage was observed for 17 patients, apraxia for 3 patients, coma for 2 patients, mental confusion for 24 patients, and impaired memory for 18 of the 1116 patients. The incidences of fatigue, speech disorders, drooling, dysphagia, and hypersomnia were 18.1, 10.3, 5.2, 2.4, and 11.6%, respectively, in the unilateral pallidotomy group and 34.9, 25.5, 22.6, 14.2, and 17.0%, respectively, in the staged pallidotomy group. Of the three patients who underwent simultaneous bilateral pallidotomies (all <50 yr of age), all developed severe fatigue and two exhibited drooling and dysphagia. The incidences of weakness, fatigue, speech disorders, drooling, dysphagia, and hypersomnia were 8.7, 30.4, 18.8, 7.2, 2.9, and 20.3%, respectively, in the double-lesion group and 3.2, 17.2, 9.7, 5.0, 2.3, and 11.5%, respectively, in the single-lesion group. CONCLUSION: Staged bilateral pallidotomy should be carefully evaluated before decision-making, whereas simultaneous bilateral pallidotomy is undesirable. Our study suggests that the size of the final lesion should be limited, to minimize the risks of complications. The incidence of symptomatic hemorrhage in microelectrode-guided pallidotomy is low and acceptable, because of the benefits of microelectrode-guided pallidotomy.