化妆品皮肤损伤989例分析

目的 探讨化妆品皮肤损伤状况和病因及发生规律。方法 对989例化妆品皮肤损伤进行了临床观察，并用辨敏牌标准筛选斑贴试验试剂盒对患者做斑贴试验。结果 临床表现主要为接触性皮炎(70．58％)，其次是色素沉着(11．02％)、痤疮样损害(7．99％)、光感性皮炎(1．01％)、混合性皮损(9．40％)。可疑致病化妆品种类189种。989例患者中阳性者653例，阳性率66．03％。结论 斑贴试验对接触性皮炎、色素沉着类化妆品皮肤损伤有确定的指导、防治意义，化妆品皮肤损伤应进行斑贴试验。     

化妆品皮肤损伤87例临床分析

化妆品皮肤损伤８７例临床分析蔡瑞康刘玮李鸿俞时玲近年来，有关化妆品引起不良反应的报道日渐增加。本文对１９９２年下半年我院门诊所见化妆品皮肤损伤８７例加以分析，报告如下：一、临床资料和分析一般资料：根据病史、临床表现及皮肤斑贴试验诊断为化妆品皮肤损伤者...     

2010年全国医学护肤品暨皮肤无创性测量讲习班通知

主办单位：中国医师学会皮肤科分会皮肤美容培训基地；四川大学华西医院。 办班宗旨：通过讲授、演示、实习皮肤外用制剂和护肤品常用剂型的配制，了解国内外皮肤外用制剂和化妆品新原料、新配方，提高研制水平。通过皮肤无创性检测技术理论学习和现场演示，了解皮肤颜色、水分、油脂、弹性、鳞屑、皱纹等指标的检测方法和皮肤微距摄影技巧，客观、科学评估皮肤外用制剂、护肤品的安全性和功效性，提高医学护肤品咨询能力。了解化妆品上市前后监管法规，掌握化妆品不良反应的诊断和处理原则。丰富和提升皮肤科临床医师、美容师以及化妆品研发人员、销售人员的技术水平和应用能力。     

化妆品使用中的阴差阳错

目前化妆品已成为人们增香添美的生活必用品。很多人为了保护皮肤的健康,预防皮肤的衰老,每日都在使用它。但是,由于种种原因,不少人在使用化妆品时都有一些小差错。不按照化妆品的使用程序有些人买了化妆品或者拿来别人的化妆品就往脸上涂,这是很危险的,因为未经清洗的皮肤上有死皮细胞、代谢产物,以及外界污染的尘埃、微生物、残存的化妆品等。此时涂用营养类或修饰类化妆品,不但不能延缓皮肤的衰老,描绘出动人的仪容,反而会影响皮肤的正常代谢,损害皮肤的健康。因此,化妆品的正确使用方法应该是先使用洁肤类化妆品,从皮肤清洁开始。然后才可使用诸如水、霜、粉底、胭脂、眼影等营养类和修饰类化妆品。     

2011全国护肤品研发应用暨皮肤无创性测量技术讲习班

中国皮肤科医师协会皮肤美容培训基地、四川大学华西医院主办的国家继续医学教育I类项目“全国护肤品研发应用暨皮肤无创性测量技术讲习班”由全国皮肤美容和化妆品学界的专家授课，将化妆品研发制备和皮肤科学的前缘技术结合起来。通过讲授和实习，熟悉外用药物和化妆品常见剂型的配制，掌握皮肤屏障、颜色、皮脂、皱纹、     

2010全国医学护肤品暨皮肤无创性测量讲习班通知

全国皮肤科医师学会皮肤美容培训基地／四川大学华西医院主办的国家继续医学教育Ⅰ类项目“医学护肤品暨皮肤无创性测量讲习班”将于2010年9月24～30日举行。由全国皮肤美容和化妆品学界的专家授课，将化妆品研发制备和皮肤科学的前沿技术结合起来。通过讲授和实习，熟悉外用药物和化妆品常见剂型的配制，掌握皮肤屏障、颜色、皮脂、皱纹、     

2009年全国医学护肤品暨皮肤无创性测量讲习班通知

全国皮肤科医师学会皮肤美容培训基地／四川大学华西医院主办的国家继续医学教育Ⅰ类项目（2009—04—12—022）“医学护肤品暨皮肤无创性测量讲习班”将于2009年7月3～9日举行。由全国皮肤美容和化妆品学界的专家授课，将化妆品研发制备和皮肤科学的前缘技术结合起来。通过讲授和实习，熟悉外用药物和化妆品常见剂型的配制，掌握皮肤表面生物学、皮肤颜色和皱纹、皮肤微循环等测量方法和仪器使用；了解国内外化妆品新原料、新配方、中药护肤品研发进展，提高护肤品、     

2009年全国医学护肤品暨皮肤无创性测量讲习班通知

全国皮肤科医师学会皮肤美容培训基地／四川大学华西医院主办的国家继续医学教育Ⅰ类项目（2009—04—12—022）“医学护肤品暨皮肤无创性测量讲习班”将于2009年7月3～9日举行。由全国皮肤美容和化妆品学界的专家授课，将化妆品研发制备和皮肤科学的前沿技术结合起来。通过讲授和实习，熟悉外用药物和化妆品常见剂型的配制，掌握皮肤表面生物学、     

美白祛斑添加剂安全性分析

长期以来，色斑一直是困扰人类皮肤的一大问题，世界化妆品专家一直致力于开发各种美白祛斑类化妆品。目前市面上销售的该类化妆品近千种，在一定程度上解决了皮肤色斑的问题。但由于科技水平所限，许多美白祛斑剂的安全性只有经过长期、广泛的使用才能逐步被确定。因此美白祛斑产品在带给人们美的同时，也带来许多不安全隐患。     

色素性化妆品皮炎48例报告分析

目的:探讨色素性化妆品皮炎的病因、发病特点及致病化妆品种类等。方法:采用笔者医院门诊2005年～2011年期间的48例色素性化妆品皮炎临床资料进行分析。结果:48例患者均为女性,年龄19～55岁,平均36.5岁;共使用化妆品种类11类,共计152种;化妆品以美白祛斑类和保湿类为主,共有75种产品(占49.34%);美容院自制产品造成的化妆品皮炎比例较高,23例(占47.92%)。其中44例患者经随访自觉症状好转,但色素沉着无1例完全好转。结论:色素性化妆品皮炎主要以美白祛斑类所致,美容院自制产品占较高比例,色素性化妆品皮炎难恢复,需引起重视。     

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.     

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。     

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.

---

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.

---

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.

---

---

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.

---

See Acknowledgment for members of the Autotranfusion Team.

---

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

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)     

恶性骨肿瘤的误诊误治分析

作者于１９９１～１９９４年，共收治曾有过不同程度误诊、误治的恶性骨肿瘤病人２５例，其中７例误诊为良性肿瘤行刮除植骨术或误诊为炎性病变行病灶清除术，５例活检失当，余病人虽诊断明确但未采取系统、有效的方法治疗以至延误保肢手术时机。我们认为：骨肿瘤的诊断应以临床、放射、病理三者结合，避免将一些缺乏典型Ｘ线特点的恶性骨肿瘤误诊为良性肿瘤。缺乏经验的诊断性活检易造成切口肿瘤细胞种植，导致保肢手术困难，应尽量避免术前活检。对一些诊断困难的病例，应由有经验的医生从事慎重、细致的术前活检。在治疗上以手术治疗为主，辅以放疗、化疗及免疫治疗等。为避免恶性骨肿瘤的误诊误治，应尽快在国内建立数个骨肿瘤治疗中心及转诊作业系统