搜索结果：Rundschau

Several types of molecular therapy have become a novel opportunity in the precision treatment of hereditary neuromuscular disorders. This cursive review of gene therapy in hereditary myopathies will focus on selected current phase 1 to 3 trials of common adult hereditary myopathies such as Becker muscular dystrophy, facioscapulohumeral muscular dystrophy, calpainopathy, and dysferlinopathy. The treatment options for Pompe disease serve as an example for hereditary metabolic myopathies. Unterschiedliche Formen der molekularen Therapie sind zu einer neuen Möglichkeit in der Präzisionsbehandlung erblicher neuromuskulärer Erkrankungen geworden. Dieser kursive Überblick über die molekularen Therapien bei hereditären Myopathien wird sich auf ausgewählte aktuelle Phase 1 bis 3 Studien zu häufigen hereditären Myopathien im Erwachsenenalter wie die Dystrophinopathie Becker-Kiener, die Fazioskapulohumerale Muskeldystrophie, Calpainopathie, und die Dysferlinopathie fokussieren. Die Therapieoptionen zum Morbus Pompe dienen als Beispiel für die hereditären metabolischen Myopathien.

The demand for surgery to treat the external female genital area has increased significantly in recent years. Since 2001, the author and his colleagues have performed more than 2,100 surgical procedures to enhance the shape and function of the female genital area. The majority of these procedures were aimed at reduction of the labia minora. Drawing on the technique for labia minora reduction (labiaplasty) that the author first described in 2007 (Gress S, Gynäkologisch-Geburtshilfliche Rundschau 47:23-32, 2007), the technique was advanced such that in addition to an even reduction of the labia over their entire length (i.e., not only the part below the clitoris but also the part of the clitoral hood and above), it currently is possible to achieve further tightening of the clitoral hood and correction of a protruding clitoris (clitoral protrusion). This technique creates separate labial segments, the composition of which allows for an optimal shaping and reduction of the labia minora. Since 2006, the author and his colleagues have managed 812 cases using this technique, which has been named "composite reduction labiaplasty." After removal of the excessive tissue in an S-shaped line along the internal and external aspects of the labium minus and after cutting of a cranial pedicle flap approximately 2-3 cm long (seen as the caudal extension of the clitoral hood), a crescent-shaped skin segment below the clitoris and a centrally pointed rectangular skin segment above the clitoral hood are removed. By joining the wound margins, a tightening and balanced reduction of the labia minora as well as a correction for the protruding tip of the clitoris (clitoral protrusion) is achieved. All the patients received postoperative care and follow-up assessments during a period of 6 months. Except for a few cases of wound dehiscence requiring surgical correction, wound healing was without complications, and the outcomes were both aesthetically and functionally very satisfactory. In contrast to most techniques published to date, the "composite reduction labiaplasty" technique ensures a balanced reduction and a tightening of all parts of the labia minora, especially in the region of the clitoral hood. In addition, this technique results in an optimal correction of clitoris positioning in cases of clitoral protrusion. Concerns regarding impairment of sexual sensation or the ability to be sexually stimulated are unfounded. Approximately 35 % of the patients even reported a postoperative increase in their ability to be sexually stimulated. This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

The heated public controversy about Freud and psychoanalysis that has been raging in America for a number of years has meanwhile found its way into major German dailies as well (Frankfurter Rundschau 5.1.1996; Frankfurter Allgemeine Zeitung 11.1.1996; Die Tageszeitung 17.1.1996). Bohleber outlines the latest case of Freud-bashing in the United States, sparked off by plans for a Freud exhibition in Washington. Closer inspection of the motives and roots of the attacks on Freud lead to the hardly surprising conclusion that the popular parlour game "Freud Bashing" has crested the wave in a social climate marked by fundamentalist conservatism, the new prudishness, and a species of scientific puritanism born of blind belief in the "exact sciences". The main representative of this attitude in connection with psychoanalysis is Adolf Grünbaum, while "revisionists" like Jeffrey Masson and Frederick Crews are to be regarded rather as exponents of the sexual counter-revolution. Bohleber's article is intended as an introductory commentary on the following essay by Jonathan Lear.

The living environment of patients suffering from progressive supranuclear palsy (PSP) has attracted little interest so far. The aim of this study was to record environmental factors and patient care structures of PSP patients in Germany. In light of this aim 100 questionnaires consisting of 28 questions were distributed in the journal PSP-Rundschau (PSP Review) in February 2009. Up to August 2009, 69 completed questionnaires had been received for evaluation. The main results were a long period up to diagnosis (3.33 ± 2.5 years) and early clinical symptoms noted by many patients which could be used for the differential diagnosis between PSP and Parkinson's disease. In 87% of the cases the patients were cared for by relatives at home mostly without professional nursing home care.It is hoped that this investigation has shed more insight into the life and disease-related symptoms of patients with PSP and can provide valuable information for the understanding and treatment of this devastating disease.

This article deals with the cultural framing of the near sequencing of the human genome and its impact on the media coverage in Germany. It investigates in particular the way in which the weekly journal Die Zeit and the daily newspaper Frankfurter Rundschau reported this media event and its aftermath between June 2000 and June 2001. Both newspapers are quality papers that played an essential role in framing the human genome debate--alongside the Frankfurter Allgemeine Zeitung--which became the most prominent genomic forum. The decoding of the human genome prompted a huge controversy concerning the ethics of human engineering, research on stem cells and Preimplantation Genetic Diagnosis. The main aim of this article is to show how this controversy was structured by metaphor. The media coverage of the genome generated DNA-factishes--a neologism designating the ambivalence of something as fact (fait) and as a fetish (fetiche)--that mostly propagated images of a new DNA-scienticism or biological determinism. Mediated by cultural experiences, the human genome became a highly artificial and social construct of a 'NatureCulture'.

In 1894, the Polish neurologist Edward Flatau (1868-1932), working in Berlin, published an exquisite photographic atlas of the unfixed human brain, preceding by 2 years Das Menschenhirn, the reference work of Gustaf Retzius (1842-1919) in Stockholm. In his early career as a neuroanatomist and neurologist, Sigmund Freud (1856-1939) wrote a review of Flatau's atlas for the Internationale klinische Rundschau, which has not been included in the 'Standard Edition of the Complete Psychological Works'. The aim of the present paper is twofold: to document Freud's review, and to revive the largely forgotten atlas of Flatau. The full text of Freud is presented in translation. Further, one element Flatau, Retzius and Freud had in common is discussed: their early role as protagonists and firm supporters of Ramón y Cajal's neuron theory, the cornerstone of modern neuroscience.

The giant sporangiophore of the single-celled fungus, Phycomyces blakesleeanus, utilises light, gravity and gases (water and ethylene) as environmental cues for spatial orientation. Even though gravitropism is ubiquitous in fungi (Naturwissenschaftliche Rundschau, 1996, 49, 174), the underlying mechanisms of gravireception are far less understood than those operating in plants. The amenability of Phycomyces to classical genetics and the availability of its genome sequence makes it essential to fill this knowledge gap and serve as a paradigm for fungal gravireception. The physiological phenomena describing the gravitropism of plants, foremost adherence to the so-called sine law, hold even for Phycomyces. Additional phenomena pertaining to gravireception, specifically adherence to the novel exponential law and non-adherence to the classical resultant law of gravitropism, were for the first time investigated for Phycomyces. Sporangiophores possess a novel type of gravisusceptor, i.e. lipid globules that act by buoyancy rather than sedimentation and that are associated with a network of actin cables (Plant Biology, 2013). Gravitropic bending is associated with ion currents generated by directed Ca(2+) and H(+) transport in the growing zone (Annals of the New York Academy of Sciences, 2005, 1048, 487; Planta, 2012, 236, 1817). A set of behavioural mutants with specific defects in gravi- and/or photoreception allowed dissection of the respective transduction chains. The complex phenotypes of these mutants led to abandoning the concept of simple linear transduction chains in favour of interacting networks with molecular modules of physically interacting proteins.

暂无摘要（点击查看详情）