1,225 research outputs found

    Adam and Eve had a Wonderful Time

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    https://digitalcommons.library.umaine.edu/mmb-vp/1035/thumbnail.jp

    Liver transplantation for type I and type IV glycogen storage disease

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    Progressive liver failure or hepatic complications of the primary disease led to orthotopic liver transplantation in eight children with glycogen storage disease over a 9-year period. One patient had glycogen storage disease (GSD) type I (von Gierke disease) and seven patients had type IV GSD (Andersen disease). As previously reported [19], a 16.5-year-old-girl with GSD type I was successfully treated in 1982 by orthotopic liver transplantation under cyclosporine and steroid immunosuppression. The metabolic consequences of the disease have been eliminated, the renal function and size have remained normal, and the patient has lived a normal young adult life. A late portal venous thrombosis was treated successfully with a distal splenorenal shunt. Orthotopic liver transplantation was performed in seven children with type N GSD who had progressive hepatic failure. Two patients died early from technical complications. The other five have no evidence of recurrent hepatic amylopectinosis after 1.1–5.8 postoperative years. They have had good physical and intellectual maturation. Amylopectin was found in many extrahepatic tissues prior to surgery, but cardiopathy and skeletal myopathy have not developed after transplantation. Postoperative heart biopsies from patients showed either minimal amylopectin deposits as long as 4.5 years following transplantation or a dramatic reduction in sequential biopsies from one patient who initially had dense myocardial deposits. Serious hepatic derangement is seen most commonly in types T and IV GSD. Liver transplantation cures the hepatic manifestations of both types. The extrahepatic deposition of abnormal glycogen appears not to be problematic in type I disease, and while potentially more threatening in type IV disease, may actually exhibit signs of regression after hepatic allografting

    NGO Legitimacy: Four Models

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    The aim of this paper is to examine NGOs’ legitimacy in the context of global politics. In order to yield a better understanding of NGOs’ legitimacy at the international level it is important to examine how their legitimacy claims are evaluated. This paper proposes dividing the literature into four models based on the theoretical and analytical approaches to their legitimacy claims: the market model, social change model, new institutionalism model and the critical model. The legitimacy criteria generated by the models are significantly different in their analytical scope of how one is to assess the role of NGOs operating as political actors contributing to democracy. The paper argues that the models present incomplete, and sometimes conflicting, views of NGOs’ legitimacy and that this poses a legitimacy dilemma for those assessing the political agency of NGOs in world politics. The paper concludes that only by approaching their legitimacy holistically can the democratic role of NGOs be explored and analysed in the context of world politics

    Assessment of cochlear synaptopathy by electrocochleography to low frequencies in a preclinical model and human subjects

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    Cochlear synaptopathy is the loss of synapses between the inner hair cells and the auditory nerve despite survival of sensory hair cells. The findings of extensive cochlear synaptopathy in animals after moderate noise exposures challenged the long-held view that hair cells are the cochlear elements most sensitive to insults that lead to hearing loss. However, cochlear synaptopathy has been difficult to identify in humans. We applied novel algorithms to determine hair cell and neural contributions to electrocochleographic (ECochG) recordings from the round window of animal and human subjects. Gerbils with normal hearing provided training and test sets for a deep learning algorithm to detect the presence of neural responses to low frequency sounds, and an analytic model was used to quantify the proportion of neural and hair cell contributions to the ECochG response. The capacity to detect cochlear synaptopathy was validated in normal hearing and noise-exposed animals by using neurotoxins to reduce or eliminate the neural contributions. When the analytical methods were applied to human surgical subjects with access to the round window, the neural contribution resembled the partial cochlear synaptopathy present after neurotoxin application in animals. This result demonstrates the presence of viable hair cells not connected to auditory nerve fibers in human subjects with substantial hearing loss and indicates that efforts to regenerate nerve fibers may find a ready cochlear substrate for innervation and resumption of function

    A prospective, multicentre, observational cohort study of analgesia and outcome after pneumonectomy

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    Background Meta-analysis and systematic reviews of epidural compared with paravertebral blockade analgesia techniques for thoracotomy conclude that although the analgesia is comparable, paravertebral blockade has a better short-term side-effect profile. However, reduction in major complications including mortality has not been proven. Methods The UK pneumonectomy study was a prospective observational cohort study in which all UK thoracic surgical centres were invited to participate. Data presented here relate to the mode of analgesia and outcome. Data were analysed for 312 patients having pneumonectomy at 24 UK thoracic surgical centres in 2005. The primary endpoint was a major complication. Results The most common type of analgesia used was epidural (61.1%) followed by paravertebral infusion (31%). Epidural catheter use was associated with major complications (odds ratio 2.2, 95% confidence interval 1.1–3.8; P=0.02) by stepwise logistic regression analysis. Conclusions An increased incidence of clinically important major post-pneumonectomy complications was associated with thoracic epidural compared with paravertebral blockade analgesia. However, this study is unable to provide robust evidence to change clinical practice for a better clinical outcome. A large multicentre randomized controlled trial is now needed to compare the efficacy, complications, and cost-effectiveness of epidural and paravertebral blockade analgesia after major lung resection with the primary outcome of clinically important major morbidity

    Catastrophic wildfire and number of populations as factors influencing risk of extinction for Gila trout (Oncorhynchus gilae)

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    We used the computer program RAMAS to explore the sensitivity of an extinction-risk model for the Gila trout (Oncorhynchus gilae) to management of wildfires and number of populations of the species. The Gila trout is an endangered salmonid presently restricted to very few headwaters of the Gila and San Francisco river tributaries in southwestern New Mexico. Life history data for 10 extant populations were used to examine sensitivity of the species' viability to changes in a variety of factors including population size, fecundity, life stage structure, number of populations, severity and probability of forest fires, and a regulated fishery. The probability and severity of forest fires and number of populations had the greatest effect on viability. Results indicate that successful conservation of Gila trout requires establishment of additional populations and reduction of the severity of forest fires through a program incorporating more frequent, but less severe, fires.Peer reviewedZoolog

    Moderating Readers and Reading Online

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    Despite the proliferation of online forums for the discussion of literary texts, very little has been written to date on the management of these spaces and how this helps frame the kinds of discussion and interpretative work that take place. This article draws on a series of interviews with moderators of online book-related sites, alongside close analysis of online interactions between moderators and users to consider issues of authority, hierarchy, power and control, asking how these act to structure or facilitate acts of interpretation taking place online. We begin by outlining the moderator's role before conducting a brief review of existing scholarship on offline reading groups and online communities, to identify how social infrastructures are established and negotiated. The main body of the article draws upon interviews with moderators of two online literary forums – The Republic of Pemberley and The Guardian’s online Reading Group – to explore the ways in which each of the respective moderators frames his or her role. This is accompanied by an in-depth exploration of how the forms of interpretation we find on the two sites are shaped and directed by the moderators. The article concludes by reflecting upon some of the issues raised by this study and its methodology, particularly with regards to digital dualism and the blurring of the boundaries between the public and the private in online spaces

    Desorption of hot molecules from photon irradiated interstellar ices

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    We present experimental measurements of photodesorption from ices of astrophysical relevance. Layers of benzene and water ice were irradiated with a laser tuned to an electronic transition in the benzene molecule. The translational energy of desorbed molecules was measured by time-of-flight (ToF) mass spectrometry. Three distinct photodesorption processes were identified - a direct adsorbate-mediated desorption producing benzene molecules with a translational temperature of around 1200 K, an indirect adsorbate-mediated desorption resulting in water molecules with a translational temperature of around 450 K, and a substrate-mediated desorption of both benzene and water producing molecules with translational temperatures of around 530 K and 450 K respectively. The translational temperature of each population of desorbed molecules is well above the temperature of the ice matrix. The implications for gas-phase chemistry in the interstellar medium are discussed.Comment: 23 pages, including 4 figures; submitted to Ap
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