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PurposeThis study aimed to describe the spectrum of cancers observed in Bloom Syndrome and the observed survival and age of first cancer diagnosis in Bloom syndrome as these are not well-defined.MethodsData from the Bloom Syndrome Registry (BSR) was used for this study. Cancer history, ages of first cancer diagnosis, and ages of death were compiled from the BSR and analyzed.ResultsAmong the 290 individuals in the BSR, 155 (53%) participants developed 251 malignant neoplasms; 100 (65%) were diagnosed with 1 malignancy, whereas the remaining 55 (35%) developed multiple malignancies. Of the 251 neoplasms, 83 (33%) were hematologic and 168 (67%) were solid tumors. Hematologic malignancies (leukemia and lymphoma) were more common than any of the solid tumors. The most commonly observed solid tumors were colorectal, breast, and oropharyngeal. The cumulative incidence of any malignancy by age 40 was 83%. The median survival for all participants in the BSR was 36.2 years. There were no significant differences in time to first cancer diagnosis or survival by genotype among the study participants.ConclusionWe describe the spectrum of cancers observed in Bloom syndrome and the observed survival and age of first cancer diagnosis in Bloom syndrome. We also highlight the significant differences in survival and age of diagnosis seen among different tumor types and genotypes.  相似文献   
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ObjectivesSeveral implementation strategies can reduce potentially inappropriate medication (PIM) prescribing. Although use of PIMs has declined in recent years, it remains prevalent. Various strategies exist to improve the appropriateness of medication use. However, little is known about the processes of these different implementation strategies. This scoping review aims to investigate how the process evaluation of implementation strategies for reducing PIM prescribing in the older population has been studied.MethodsWe searched for process evaluations of implementation strategies for reducing PIM prescribing in PUBMED, SCOPUS and Web of Science published between January 2000 and November 2019 in English. We applied the following inclusion criteria: patients aged ≥65 years, validated PIM criteria, and implementation process evaluated. The review focuses on decision support for health care professionals. We described the findings of the process evaluations, and compared the authors’ concepts of process evaluation of the included publications to those of Proctor et al.( 2010).ResultOf 9131 publications screened, 29 met our inclusion criteria. Different process evaluation conceptualizations were identified. Most process evaluations took place in the initial stages of the process (acceptability, adoption, appropriateness, and feasibility) and sustainability and implementation costs were seldom evaluated. None of the included publications evaluated fidelity.Multifaceted interventions were the most studied implementation strategies. Medication review was more common in acceptability evaluations, multidisciplinary interventions in adoption evaluations, and computerized systems and educational interventions in feasibility evaluations. Process evaluations were studied from the health care professionals’ viewpoint in most of the included publications, but the management viewpoint was missing.DiscussionThe conceptualization of process evaluation in the field of PIM prescribing is indeterminate. There is also a current gap in the knowledge of sustainability and implementation costs. Clarifying the conceptualization of implementation process evaluation is essential in order to effectively translate research knowledge into practice.  相似文献   
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目的 研究脂肪肝对超声造影诊断肝脏炎性假瘤(IPL)的影响。方法 2016年1月~2021年1月华北理工大学附属开滦总医院诊治的IPL患者28例,其中伴有脂肪肝者12例,正常肝内IPL患者16例。所有患者均接受超声造影检查,比较两组血供状态、超声造影增强模式和超声造影定量参数的异同。结果 伴脂肪肝的IPL病灶动脉供血1例(8.3%)、静脉供血11例(91.7%)、富血供3例(25.0%)、乏血供9例(75.0%),IPL病灶动脉供血7例(43.8%)、静脉供血9例(56.3%)、富血供5例(31.3%)、乏血供11例(68.8%),两组供血类型存在显著性差异(P<0.05),而血供丰富程度无显著性差异(P>0.05);伴脂肪肝病灶呈无明显增强型1例、快速廓清型5例、等增强型3例和低增强型3例,IPL病灶呈无明显增强型2例、快速廓清型5例、等增强型7例和低增强型2例,两组超声造影增强模式无显著差异(P=0.661);伴脂肪肝病灶造影剂到达时间为(8.9±1.3)s,显著慢于IPL病灶【((8.0±0.9)s,P<0.05】,峰值强度为(53.2±7.8)dB,显著弱于IPL病灶【((61.1±9.7)dB,P<0.05】,而两组达峰时间、曲线尖度和曲线下面积无显著性差异(P>0.05)。结论 与正常肝背景下的IPL病灶比,合并脂肪肝的IPL病灶以静脉供血为主,其造影剂的到达时间较长,而峰值强度降低,在临床诊断时需要认真地甄别。  相似文献   
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目的探讨核磁共振动态增强扫描对前列腺癌的诊断价值。方法选取40例前列腺癌患者与40例参加癌症筛查的其他前列腺疾病患者,均接受核磁共振动态增强扫描与电子计算机断层扫描(CT)检查。比较两种检查方法对前列腺癌的诊断结果。结果核磁共振动态增强扫描与CT检查的诊断阳性率比较,差异无统计学意义(P>0.05)。核磁共振动态增强扫描检查呈阳性39例,阴性41例,灵敏度为92.50%(37/40)、特异度为95.00%(38/40)、准确率为93.75%(75/80)。CT检查呈阳性40例,阴性40例,灵敏度为75.00%(30/40)、特异度为75.00%(30/40)、准确率为75.00%(60/80)。核磁共振动态增强扫描检查的灵敏度、特异度、准确率均高于CT检查,差异有统计学意义(P<0.05)。结论对于前列腺癌患者通过核磁共振动态增强扫描进行诊断,具有较高的敏感性、特异性以及准确率,可以详细地掌握患者病情状况,提高患者的预后质量。  相似文献   
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This paper describes the diagnostic criteria for presbyvestibulopathy (PVP) of the Classification Committee of the Bárány Society. PVP is defined as a chronic vestibular syndrome characterized by unsteadiness, gait disturbance, and/or recurrent falls in the presence of mild bilateral vestibular deficits, with findings on laboratory tests that are between normal values and the thresholds established for bilateral vestibulopathy. The diagnosis of PVP is based on patient history, bedside examination, and laboratory evaluation. The diagnosis of PVP requires bilaterally reduced function of the vestibulo-ocular reflex (VOR). This can be diagnosed for the high frequency range of the VOR with video-HIT (vHIT), for the middle frequency range with rotary chair testing, and for the low frequency range with caloric testing. For the diagnosis of PVP, the horizontal angular VOR gain on both sides should be < .8 and > .6, and/or the sum of the maximal peak velocities of the slow phase caloric-induced nystagmus for stimulation with warm and cold water on each side should be < 25°/s and > 6°/s, and/or the horizontal angular VOR gain should be > .1 and < .3 upon sinusoidal stimulation on a rotatory chair. PVP typically occurs along with other age-related deficits of vision, proprioception, and/or cortical, cerebellar, and extrapyramidal function which also contribute to and might even be required for symptoms of unsteadiness, gait disturbance, and falls to manifest. These criteria simply consider the presence of these symptoms, along with documented impairment of vestibular function, in older adults.  相似文献   
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