10对联体双胎分离术后的远期结果

背景／目的：从1978-2000年，有10对联体双胞胎成功接受了手术分离，结果14人存活。其中6对是因为他们的联体同胞死亡或濒临死亡而接受紧急分离手术。剩余的4对，每对至少一个同胞接受至少一次的急诊手术后，才接受联体分离手术。方法：对这一独特的队列进行包含6个项目的问卷调查。问题的设计是开放式的，为父母／家庭提供有关信息的最大机会。对每个家庭就相同的问题也进行了当面的问卷调查。医师询问了与身体健康和疾病相关的一些问题。而社会工作询问了与发育、教育、社会心理和家庭功能相关的问题。结果：前面提及的14人存活的双胞胎中，4人大学毕业，1人于1981年完成中学学业，剩余9人正在上学。14人在最初的手术后，还需要接受再次手术，尤其是需要矫正泌尿系统、畸形、神经外科和小儿外科的问题。结论：通过超声检查对联体双胎进行宫内诊断，可以使医师和胎儿父母在孕早期决定在终止妊娠时是进行双胎分离还是保全其中一个。这些病例报道通过描述14例手术存活的长期体格检查和心理学检查结果，为医师和父母在关键时刻做出决定提供了另外有助的依据。     

Increasing Perceptions of Self-Worth in Preadolescents Diagnosed with ADHD

Purpose: To test the effectiveness of a school-based, nurse-facilitated support group in increasing perceptions of scholastic competence, social acceptance, behavioral conduct, perceived athletic competence, perceived physical appearance, and perceived global self-worth in preadolescents diagnosed with attention deficit disorder (ADD) or attention deficit hyperactivity disorder (ADHD).
Design: A quasi-experimental design with 65 preadolescents diagnosed with ADD or ADHD in an upper-middle class community in the United States.
Methods: Participants randomly assigned to either the control or experimental group completed Harter's Self-Perception Profile for Children instrument at the beginning of the study and 4 weeks later. Students in the experimental condition participated in a school-nurse facilitated support group which met twice weekly for 4 weeks.
Findings: Participants in the support group had increased scores on each of the six subscales, with significant increases on four of the subscales, including perceived social acceptance, perceived athletic competence, perceived physical appearance, and perceived global self-worth.
Conclusion: Participation in a school-based, nurse-led support group was positively associated with perception of self-worth in preadolescents diagnosed with ADD or ADHD.     

Isolated Donor Specific Alloantibody-Mediated Rejection after ABO Compatible Liver Transplantation

Antibody-mediated rejection (AMR) after liver transplantation is recognized in ABO incompatible and xeno-transplantation, but its role after ABO compatible liver transplantation is controversial. We report a case of ABO compatible liver transplantation that demonstrated clinical, serological and histological signs of AMR without evidence of concurrent acute cellular rejection. AMR with persistently high titers of circulating donor specific antibodies resulted in graft injury with initial centrilobular hepatocyte necrosis, fibroedematous portal expansion mimicking biliary tract outflow obstruction, ultimately resulting in extensive bridging fibrosis. Immunofluorescence microscopy demonstrated persistent, diffuse linear C4d deposits along sinusoids and central veins. Despite intense therapeutic intervention including plasmapheresis, IVIG and rituximab, AMR led to graft failure. We present evidence that an antibody-mediated alloresponse to an ABO compatible liver graft can cause significant graft injury independent of acute cellular rejection. AMR shows distinct histologic changes including a characteristic staining profile for C4d.     

The need for a revival of psychoanalytic investigations into premature ejaculation

Although psychoanalysis was the first-choice treatment for premature ejaculation (PE) between 1920 and 1960, hardly any reports on its efficacy have been published. Moreover, a scientific debate about its findings has never been fully developed. The recent progress that has been made in the classification of three different PE syndromes creates a new opportunity for psychoanalytic investigations of men with complaints of PE, distinguished by the actual duration of their intravaginal ejaculation latency time (IELT). The term premature-like ejaculatory dysfunction has been introduced to distinguish men with self-perceived PE at normal and long IELT durations from those men with lifelong, acquired and normal variable PE. Psychoanalytic research may contribute to a better understanding of the consequences of objective early ejaculations on the unconscious mental life of men with the four forms of PE. By integrating neurobiological, clinical and epidemiological data of ejaculatory performance, a revival of psychoanalytic research of PE in the four distinct, classified PE groups, will probably contribute to a deeper insight in to the unconscious mental life of men affected by PE.     

Costimulation and Autoimmune Diabetes in BB Rats

Costimulatory signals regulate T-cell activation. To investigate the role of costimulation in autoimmunity and transplantation, we studied the BB rat model of type 1 diabetes. Diabetes-prone BB (BBDP) rats spontaneously develop disease when 55–120 days of age. We observed that two anti-CD28 monoclonal antibodies (mAb) with different functional activities completely prevented diabetes in BBDP rats. Anti-CD154 mAb delayed diabetes, whereas treatment with CTLA4-Ig or anti-CD80 mAb accelerated disease. Anti-CD86 or anti-CD134L mAbs had no effect. Diabetes resistant BB (BBDR) rats are disease-free, but >95% of them develop diabetes after treatment with polyinosinic-polycytidylic acid and an mAb that depletes Treg cells. In the induced BBDR model, anti-CD154 mAb delayed onset of diabetes, whereas CTLA4-Ig, anti-CD134L or either of the anti-CD28 mAbs had little or no effect. In contrast, blockade of the CD134-CD134L pathway was highly effective for preventing autoimmune recurrence against syngeneic islet grafts in diabetic BBDR hosts. Blockade of the CD40-CD154 pathway was also effective, but less so. These data suggest that the effectiveness of costimulation blockade in the treatment of type 1 diabetes is dependent on both the costimulatory pathway targeted and the mechanism of induction, stage, intensity and duration of the pathogenic process.     

复合杂合parkin基因突变家族的不同表型

背景：Parkin基因（PRKN）突变可导致常染色体隐性遗传性早发帕金森病（EOPD）。目的：探讨EOPD白人家族PRKN突变的表现和基因型-表型关系。设计：对EOPD家族的3代20例成员进行基因分析，该家族有4例患者。应用直接基因组DNA测序、半定量聚合酶链反应、实时定量聚合酶链反应以及逆转录酶聚合酶链反应分析以确定PRKN突变。结果：4例早发患者（年龄30—38岁）被确定有PRKN复合杂合突变（T240M和EX5_6缺失），虽然PRKN的杂合T240M和纯合EX5_6缺失突变已有描述，但是据悉，本文为上述复合杂合突变的首次报道。患者的表型为典型常染色体隐性遗传性EOPD的表现，其特征是对左旋多巴治疗有效、相对缓慢的进展和运动障碍。所有杂合突变的基因携带者（T240M或EX5_6缺失）和1例56岁的复合杂合突变女性携带者（T240M和EX5_6缺失）无任何神经系统症状。结论：研究发现，PRKN基因复合杂合突变（T240M和EX5_6缺失）导致一个大的白人家族中4例成员发生常染色体隐性遗传性EOPD。另外1例成员具有相同的突变，比4例患者的平均发病年龄大10岁，并且无本病的临床表现。不完全的外显率对遗传咨询具有暗示作用，并且提示复杂的基因一环境交互作用在PRKN相关EOPD的发病机制中发挥作用。     

A simple classification for standardisation of nomenclature in free flap outcome.

The numbers of free flap donor site as well as their indications are constantly increasing. Despite increasing popularity of microvascular reconstructive procedures, literature lacks clear and objective outcome criteria. This paper reports on a simple outcome classification that has become a routine part of the unit's large workload of microvascular outcome recording. The classification was formed through a retrospective analysis of 241 consecutive cases from 2000 to 2001 and is a five graded numerical classification. Grade 1 equates to total success without co-morbidity and grade 5 to a major complication such as amputation, etc., whatever the status of the flap itself. From 2002 to 2005 the classification was prospectively used on 527 consecutive cases with ease of integration into routine clinical practice. The Classification would enable a more objective record keeping thus analysis of the outcome. It would allow a more realistic comparison of different techniques or donor types as well set a benchmarking level for further improvement of the results.