Physical Review a最新文献

Evans syndrome (ES) is a spectrum of diseases in which the combination of autoimmune hemolytic anemia and immune thrombocytopenia or sometimes neutropenia. ES has been accepted usually as an idiopathic condition, but it may be secondary. The coexistence of autoimmune cytopenias and Hodgkin lymphoma (HL) is rarely observed and the rate of ES in HL patients is not clear. Here we describe a 56-year-old male patient who presented with ES and was diagnosed with HL. After corticosteroids, intravenous immunoglobulin (IVIG) and ABVD (doxorubicin, bleomycin, vinblastine, dacarbazine) treatment, immune cytopenias were completely resolved. The literature is also reviewed and we found 16 cases in which HL and ES coexist. Although AIHA and immune thrombocytopenia usually develop simultaneously, they rarely occur at different times. Many aspects of the pathogenesis are unknown, but it is thought to be a complex immunological background. Corticosteroids and/or IVIG are the most commonly used first-choice drugs in the initial treatment of ES. Response rates to treatment are variable and response to treatment may be poor, particularly with underlying conditions. If detected, the underlying lymphoma should be treated.

This article uses three cities in the same Canadian province (Ontario): Toronto, Ottawa and Waterloo, to examine how regions compete in high-technology markets. We find that regions use civic capital to leverage new, technological windows of opportunity, but they do so in very different ways. Tracing Toronto's evolution from a marketing hub for foreign multinationals into a centre for entrepreneurship, we illustrate how weak ties and cross-sectoral buzz created a 'super connector', scaling high-technology firms in a wide variety of areas. In Ottawa, task-specific cooperation in R&D, education and specialised infrastructure enabled the region to overcome the disadvantages of its small size as a 'specialist' in a single, capital-intensive niche, telecommunications equipment. Finally, entrepreneurs in Waterloo eschewed task-specific cooperation for peer-to-peer mentoring. By diffusing generic knowledge about how to circumvent the liabilities of smallness, mentoring networks enabled this 'scrapper' city to support smaller start-ups in a broad range of niches.

Background: Glucose-6-phosphate dehydrogenase (G6PD) deficiency is an X-linked recessive Mendelian genetic disorder characterized by neonatal jaundice and hemolytic anemia, affecting more than 400 million people worldwide. The purpose of this research was to investigate prevalence rates of G6PD deficiency and to evaluate and establish specific cut-off values in early prediction of G6PD deficiency by regions (HeFei, FuYang, AnQing) on different seasons, as well as to investigate the frequencies of G6PD gene mutations among three regions mentioned above.

Methods: A total of 31,482 neonates (21,402, 7680, and 2340 for HeFei, FuYang, and AnQing cities, respectively) were recruited. Positive subjects were recalled to attend genetic tests for diagnosis. G6PD activity on the Genetic screening processor (GSP analyzer, 2021-0010) was measured following the manufactureržs protocol. The cut-off value was first set to 35 U/dL. The receiver operating characteristics (ROC) curve was employed to assess and compare the efficiency in predicting G6PD deficiency among HeFei, FuYang, and AnQing cities in different seasons.

In this paper, we discuss a response adaptive randomization method, and why it should be used in clinical trials for rare diseases compared to a randomized controlled trial with equal fixed randomization. The developed method uses a patient's biomarkers to alter the allocation probability to each treatment, in order to emphasize the benefit to the trial population. The method starts with an initial burn-in period of a small number of patients, who with equal probability, are allocated to each treatment. We then use a regression method to predict the best outcome of the next patient, using their biomarkers and the information from the previous patients. This estimated best treatment is assigned to the next patient with high probability. A completed clinical trial for the effect of catumaxomab on the survival of cancer patients is used as an example to demonstrate the use of the method and the differences to a controlled trial with equal allocation. Different regression procedures are investigated and compared to a randomized controlled trial, using efficacy and ethical measures.

Purpose of the review: Systemic sclerosis (SSc) is a condition of dermal and visceral scar formation characterized by immune dysregulation and inflammatory fibrosis. Approximately 90% of SSc patients develop interstitial lung disease (ILD), and it is the leading cause of morbidity and mortality. Further understanding of immune-mediated fibroproliferative mechanisms has the potential to catalyze novel treatment approaches in this difficult to treat disease.

Recent findings: Recent advances have demonstrated the critical role of aberrant innate immune activation mediated by mitochondrial DNA (mtDNA) through interactions with toll-like receptor 9 (TLR9) and cytosolic cyclic guanosine monophosphate-adenosine monophosphate synthase (cGAS).

Summary: In this review, we will discuss how the nature of the mtDNA, whether oxidized or mutated, and its mechanism of release, either intracellularly or extracellularly, can amplify fibrogenesis by activating TLR9 and cGAS, and the novel insights gained by interrogating these signaling pathways. Because the scope of this review is intended to generate hypotheses for future research, we conclude our discussion with several important unanswered questions.

Introduction: Proton beam therapy (PBT) is an effective treatment option for uveal melanomas. Following treatment, it may take many months for the tumour to respond and it may initially enlarge. We reviewed our PBT patients to determine when they showed a radiological response to treatment.

Methods: Patients undergoing PBT for ciliary body or choroidal melanomas between 2008 and 2018 were included. Data were collected on patient demographics, treatments before and after PBT and survival. All ultrasound investigations prior and since PBT were reviewed and tumour volume calculated using a validated formula for a half-ellipsoid shape.

Results: 193 patients were analysed, 169 with choroidal and 24 with ciliary body melanomas. 31.6% patients had other treatment prior to PBT. At a mean of 8 months post-PBT, 64.7% of patients had a reduced tumour volume with 20.2% having larger tumours. At a mean of 15 months post-treatment, these figures were 67.8% and 10.3%. 18.1% of patients had an enucleation during the study period. The earliest responses were seen at 2 months, the latest at 32 months post-treatment. 5-year melanoma-specific survival was 82.3%.

Conclusions: In our study, by 6 months post-PBT, a majority of patients show a reduction in tumour volume. Of those that do not, many respond in the next 6 months and a response may be seen up to 32 months after treatment. Patients may need to be monitored for up to 32 months to see a final response to PBT treatment.