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We report on 2 sisters with an autosomal-recessive multiple pterygium syndrome, type Escobar, consisting of multiple pterygia with severe contractures, short stature, and minor facial and external genital anomalies. The striking finding was severe muscular atrophy. We speculate that a neu-romuscular disorder is the underlying pathogenesis of Escobar syndrome. © 1995 Wiley-Liss, Inc.  相似文献   
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Background: Postoperative pain and severe side effects of opioid analgesics present a clinical challenge after cardio-thoracic surgery. In this study, the impact of Kinesio® taping on postoperative morbidity after median sternotomy was observed. Methods: Thirty-nine patients (mean age 66 ± 9 years, CI: 63.28; 68.98) who underwent median sternotomy between 09/2014 and 11/2014 participated in this prospective randomized controlled trial. Patients were assigned into a treatment on a non-treatment group. Patients in the treatment group were taped after leaving the intensive care unit. We assessed, pain, consumption of pain medication, the subjective estimation of patients’ ability to breathe, radiologic and microbial abnormalities as well as adverse effects resulting from the tape use daily until discharge. To determine the patients’ satisfaction a discharge questionnaire was offered after completion of data. Results: Patients who were treated with tape report significantly less pain (2.14 ± 0.5, CI: 1.1; 3.13) than patients from the control group (4.16 ± 0.6, CI: 2.92; 5.41, p = 0.01). The need for opioid pain medication, as assessed by total analgesic consumption per patient, was significantly less in the treatment group (1.2 ml ± 0.4 ml, CI: 0.40 ml; 2.01 ml) versus (3.1 ml ± 0.5 ml, CI: 2.0 ml; 4.2 ml, p = 0.01). The subjective estimation of patients’ ability to breathe was significantly better (p < 0.001) and the satisfaction was higher in the Kinesio® tape group compared to the control group. Taped patients had a mean hospitalization of 10 ± 1 day (CI: 8.74 days; 11.78 days) untapped patients stayed for 11 ± 1 days (CI: 9.17 days; 11.83 days). Adverse effects from the tape treatment were not observed. Conclusions: Kinesio® taping after median sternotomy is a low-risk, non-pharmacologic, cost effective, and promising method for improving patients’ breathing conditions, reducing postoperative pain, pain medication consumption, and thus, potential adverse effects of analgesics.  相似文献   
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With support from the Institutes and Centers forming the NIH Blueprint for Neuroscience Research, we have designed and implemented a new initiative for integrating access to and use of Web-based neuroscience resources: the Neuroscience Information Framework. The Framework arises from the expressed need of the neuroscience community for neuroinformatic tools and resources to aid scientific inquiry, builds upon prior development of neuroinformatics by the Human Brain Project and others, and directly derives from the Society for Neuroscience’s Neuroscience Database Gateway. Partnered with the Society, its Neuroinformatics Committee, and volunteer consultant-collaborators, our multi-site consortium has developed: (1) a comprehensive, dynamic, inventory of Web-accessible neuroscience resources, (2) an extended and integrated terminology describing resources and contents, and (3) a framework accepting and aiding concept-based queries. Evolving instantiations of the Framework may be viewed at , , and other sites as they come on line.  相似文献   
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Closed rupture of the tibial anterior tendon is a rare clinical entity. Case reports in the literature reveal a total of only 49 cases up to the year 2000. According to these reports, the age group affected is 50 to 70 years old, and there are more men than women affected. Although the functional limitation is quite considerable, late diagnosis is common. An appropriate clinical examination, including an exact history taking, should lead to the right diagnosis. Ultrasound examination and magnetic resonance imaging (MRI) may be helpful. 'Restitutio ad integrum' can only be achieved by operative treatment. If technically possible, reinsertion of the tendon directly into bone or direct tendon repair is preferred. After delayed diagnosis, a secondary reconstruction through tendon transfer or transplantation is often necessary. A 64-year-old woman presented with pain and swelling in the area of the ankle joint 5 months after falling. She showed insecurity in walking, and the heel-walk could not be demonstrated. The distal neurovascular function was intact. The area of the retinaculum showed a swelling, and the tendon was not palpable in comparison with the other forefoot. An intact tendon could not be seen by ultrasound, and MRI confirmed these findings. A complete rupture was noted during the operative revision. The proximal and the distal tendon stumps were found to be thickened and knotted, the proximal stump was also atrophic. An augmented tenoplasty was performed. Afterwards, the tendon was tense in the neutral position. The lower leg was put in a plaster cast for 6 weeks, followed by physiotherapy. Ten months after the operation, the tendon was palpable in the correct position, the dorsal extension was powerful, and the patient did not experience any difficulty. Rupture of the anterior tibial tendon is a rare clinical entity and should be considered in the differential diagnosis of pain in the area of the ankle joint. An early operative treatment is advantageous.  相似文献   
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Obesity is associated with numerous inflammatory conditions including atherosclerosis, autoimmune disease and cancer. Although the precise mechanisms are unknown, obesity‐associated rises in TNF‐α, IL‐6 and TGF‐β are believed to contribute. Here we demonstrate that obesity selectively promotes an expansion of the Th17 T‐cell sublineage, a subset with prominent pro‐inflammatory roles. T‐cells from diet‐induced obese mice expand Th17 cell pools and produce progressively more IL‐17 than lean littermates in an IL‐6‐dependent process. The increased Th17 bias was associated with more pronounced autoimmune disease as confirmed in two disease models, EAE and trinitrobenzene sulfonic acid colitis. In both, diet‐induced obese mice developed more severe early disease and histopathology with increased IL‐17+ T‐cell pools in target tissues. The well‐described association of obesity with inflammatory and autoimmune disease is mechanistically linked to a Th17 bias.  相似文献   
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