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31.
The De Sanctis-Cacchione Syndrome is the rarest and most severe kind of xeroderma pigmentosum, characterized by microcephaly, hypogonadism, neurological disorders, mental and growth retardation, with very few cases published. The clinical findings compatible with De Sanctis-Cacchione Syndrome and the therapeutic approach used to treat a one year and nine months old child, with previous diagnosis of xeroderma pigmentosum, are reported.  相似文献   
32.

Purpose  

Cancer in pregnancy is a rare circumstance. However, the coincidence of pregnancy and malignancy is supposed to increase due to a general tendency of postponing childbearing to older age. To date, clinical guidelines are scarce and experience regarding therapeutic management is limited to case reports.  相似文献   
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Science has played an influential role for at least the past 70 years in helping us to frame public policy in many areas of concern. But in recent years science itself has become an area of concern. This is partly because scientific theories can be difficult to understand, and partly because the evidence for evaluating them is rarely as definitive as we might hope. But it is also because scientific inquiry has increasingly come under the influence of a variety of factors that many people regard as non-scientific. Our society today is straddling several different ideas about what science is and what its primary goals should be-and the time has come to begin a public discussion to explore the different concerns that people might have about science and the different governance possibilities for addressing them. This editorial presents the main results of two panels that met on a monthly basis in Washington, DC from August 2005 through March 2007 to explore their concerns about science and to develop conceptual governance possibilities for public policy pertaining to it. The eight possibilities described below are not intended to be planks in a consistent policy platform for science. It would, indeed, be impossible to consistently adopt them all at once. But each possibility reflects serious concerns about science, and each of them merits further public discussion.  相似文献   
35.

Introduction  

To evaluate the operative outcomes of patients managed by laparoscopic-assisted vaginal hysterectomy (LAVH) with and without laparoscopic transsection of the uterine artery for benign gynaecologic diseases.  相似文献   
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Objective

To understand the complex effects of interruption in healthcare.

Materials and methods

As interruptions have been well studied in other domains, the authors undertook a systematic review of experimental studies in psychology and human–computer interaction to identify the task types and variables influencing interruption effects.

Results

63 studies were identified from 812 articles retrieved by systematic searches. On the basis of interruption profiles for generic tasks, it was found that clinical tasks can be distinguished into three broad types: procedural, problem-solving, and decision-making. Twelve experimental variables that influence interruption effects were identified. Of these, six are the most important, based on the number of studies and because of their centrality to interruption effects, including working memory load, interruption position, similarity, modality, handling strategies, and practice effect. The variables are explained by three main theoretical frameworks: the activation-based goal memory model, prospective memory, and multiple resource theory.

Discussion

This review provides a useful starting point for a more comprehensive examination of interruptions potentially leading to an improved understanding about the impact of this phenomenon on patient safety and task efficiency. The authors provide some recommendations to counter interruption effects.

Conclusion

The effects of interruption are the outcome of a complex set of variables and should not be considered as uniformly predictable or bad. The task types, variables, and theories should help us better to identify which clinical tasks and contexts are most susceptible and assist in the design of information systems and processes that are resilient to interruption.  相似文献   
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Aim

To evaluate postoperative sexual functioning and the influence of patients’ expectations on the change in sexuality following laparoscopic total (TLH) versus subtotal hysterectomy (LASH).

Methods

A total of 120 women undergoing laparoscopic hysterectomy were preoperatively enrolled in this bicentric prospective study. Sexual functioning (SF) was evaluated using the female sexual function index (FSFI). Additionally, participants filled in a standardised questionnaire concerning expected changes in SF after surgery. At 3, 6 and 12 months following surgery, women were asked again to assess their level of SF (FSFI). Data of women who participated in at least one FSFI follow-up assessment were analysed (n?=?92). We compared the change in SF after surgery between patients with TLH (n?=?46) and LASH (n?=?46). Additionally, we calculated regression analyses with the patients’ expectations as a predictor for change in FSFI scores.

Results

Comparing the change of FSFI scores after surgery in both collectives revealed differences only 3 months after surgery, as improvement was stronger for the LASH collective compared to the THL group (p?=?0.006). There were no changes comparing collectives after 6 (p?=?0.663) and 12 (p?=?0.326) months. Concerning patients? expectations, for the LASH group baseline SF (p?<?0.001), but not expectations (p?=?0.567) predicted the strength of change at each of the follow ups: a lower level of baseline SF was linked to a stronger improvement after surgery. For the THL collective, both baseline SF (p?<?0.001) as well as patients’ expectations (3 months: p?=?0.077, 6 months: p?=?0.37, 12 months: p?=?0.024) predicted the strength of change: both, a lower level of baseline SF and higher expectations towards an improvement predicted a stronger improvement.

Conclusion

The preservation of the cervix does not show an advantage in improving SF after surgery. Both methods induce a comparable improvement in long-time SF, especially in patients with an impaired sexuality pre-surgery. Furthermore, patients’ expectations concerning this matter seem to have an impact on the postoperative outcome; therefore, this circumstance should be considered in future projects.
  相似文献   
40.
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