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排序方式: 共有1231条查询结果,搜索用时 250 毫秒
1.
Monica Poiani Myriam Labopin Giorgia Battipaglia Dietrich W. Beelen Johanna Tischer Jürgen Finke Arne Brecht Edouard Forcade Arnold Ganser Jakob R. Passweg Helene Labussiere‐Wallet Ibrahim Yakoub‐Agha Kerstin Schfer‐Eckart Nicolaus Kroeger Blandine Guffroy Annalisa Ruggeri Jordi Esteve Arnon Nagler Mohamad Mohty 《American journal of hematology》2021,96(1):40-50
Karyotypic analysis at time of diagnosis has an important value in determining initial response to treatment, remission duration and overall survival (OS) in acute myeloid leukemia (AML). Less is known about its value before allogeneic hematopoietic cell transplantation (allo‐HCT) in patients transplanted with active disease, either relapsed or primary refractory (Rel‐Ref) AML. We explored the impact of cytogenetic risk (stratification according to MRC‐UK) in 2089 patients with either Ref (n = 972) or Rel AML (n = 1117) transplanted during the period 2000‐2017. Overall, 154 patients had a favorable risk, 1283 had an intermediate risk and 652 had an adverse cytogenetic risk. Median follow‐up was 49 months. Compared to the favorable risk group, intermediate and adverse risk patients were associated with worse leukemia‐free survival and OS and also with a higher incidence of relapse. In a subgroup analysis of patients in the intermediate risk group harboring Fms‐like tyrosine kinase 3‐internal tandem duplication (FLT3‐ITD), this remained an important prognostic factor, being associated with worse outcomes. When analyzing patients according to the intensity of the conditioning regimen, no differences were observed for the main transplant outcomes. In conclusion, in patients diagnosed with AML and transplanted with active disease, karyotype remains an important prognostic factor, allowing splitting patients into different risk groups according to their cytogenetics. Similarly, FLT3‐ITD mutation also remains a negative prognostic factor in this population. 相似文献
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Vered Molho-Pessach MD Arnon Meltser BSc Adaia Kamshov MD Yuval Ramot MD MSc Abraham Zlotogorski MD 《Pediatric dermatology》2020,37(1):153-155
Heterozygous STAT1 gain-of-function (GOF) mutations result in a combined form of immunodeficiency which is the most common genetic cause of chronic mucocutaneous candidiasis (CMC). We present a pedigree with a GOF mutation in STAT1, manifesting with chronic demodicosis in the form of a facial papulopustular eruption, blepharitis, and chalazion. So far, demodicosis has been described in only one family with STAT1-GOF mutation. We suggest that chronic demodicosis is an under-recognized feature of the immune dysregulation disorder caused by STAT1 gain-of-function mutations. 相似文献
4.
Shema-Shiratzky Shirley Hillel Inbar Mirelman Anat Regev Keren Hsieh Katherine L. Karni Arnon Devos Hannes Sosnoff Jacob J. Hausdorff Jeffrey M. 《Journal of neurology》2020,267(7):1912-1921
Journal of Neurology - People with multiple sclerosis (pwMS) often suffer from gait impairments. These changes in gait have been well studied in laboratory and clinical settings. A thorough... 相似文献
5.
Marina Díaz-Beyá Myriam Labopin Johan Maertens Mahmoud Alijurf Jakob Passweg Beelen Dietrich Harry Schouten Gerard Socié Nicolaas Schaap Rainer Schwerdtfeger Liisa Volin Mauricette Michallet Emmanuelle Polge Jorge Sierra Mohamad Mohty Jordi Esteve Arnon Nagler the Acute Leukaemia Working Party of the European Society for Blood Marrow Transplantation 《British journal of haematology》2020,189(5):920-925
Acute myeloid leukaemia (AML) with t(6;9)(p23;q34) is a poor-risk entity, commonly associated with FLT3-ITD (internal tandem duplication). Allogeneic stem-cell tranplantation (allo-SCT) is recommended, although studies analysing the outcome of allo-SCT in this setting are lacking. We selected 195 patients with t(6;9) AML, who received a first allo-SCT between 2000 and 2016 from the EBMT (European Society for Blood and Marrow Transplantation) registry. Disease status at time of allo-SCT was the strongest independent prognostic factor, with a two-year leukaemia-free survival and relapse incidence of 57% and 19% in patients in CR1 (first complete remission), 34% and 33% in CR2 (second complete remission), and 24% and 49% in patients not in remission, respectively (P < 0·001). This study, which represents the largest one available in t(6;9) AML, supports the recommendation to submit these patients to allo-SCT in CR1. 相似文献
6.
Hyla Allouche‐Arnon Yonatan Hovav Lanette Friesen‐Waldner Jacob Sosna J. Moshe Gomori Shimon Vega Rachel Katz‐Brull 《NMR in biomedicine》2014,27(6):656-662
A kinetic model is provided to obtain reaction rate constants in successive enzymatic reactions that are monitored using NMR spectroscopy and hyperpolarized substrates. The model was applied for simulation and analysis of the successive oxidation of choline to betaine aldehyde, and further to betaine, by the enzyme choline oxidase. This enzymatic reaction was investigated under two different sets of conditions: two different choline molecular probes were used, [1,1,2,2‐D4, 1‐13C]choline chloride and [1,1,2,2‐D4, 2‐13C]choline chloride, in different MR systems (clinical scanner and high‐resolution spectrometer), as well as in different reactors and reaction volumes (4.8 and 0.7 mL). The kinetic analysis according to the model yielded similar results in both set‐ups, supporting the robustness of the model. This was achieved despite the complex and negating influences of reaction kinetics and polarization decay, and in the presence of uncontrolled mixing characteristics, which may introduce uncertainties in both effective timing and effective pulses. The ability to quantify rate constants using hyperpolarized MR in the first seconds of consecutive enzyme activity is important for further development of the utilization of dynamic nuclear polarization‐MR for biological determinations. Copyright © 2014 John Wiley & Sons, Ltd. 相似文献
7.
Danya Rosen Jaime Chu Rahul Patel Jang Moon Kishore Iyer Ronen Arnon 《Pediatric transplantation》2014,18(6):E193-E196
Gastric variceal bleeding is associated with high morbidity and mortality. Balloon‐occluded retrograde transvenous obliteration is a relatively new treatment used to control bleeding gastric varices that involves transvenous sclerosis of gastric varices through a spontaneous gastrorenal shunt. Here, we report on a 14‐yr‐old patient that underwent balloon‐occluded retrograde transvenous obliteration for refractory bleeding fundal varices in the setting of esophageal varices and cirrhosis, which did not respond to medical management or endoscopic injection. This case report serves as a reminder that balloon‐occluded retrograde transvenous obliteration can successfully control fundal variceal bleeding in pediatric patients and may serve as a bridge to liver transplantation. 相似文献
8.
Joseph L. Chin Brian J. Donnelly Shiva M. Nair Arnon Lavi 《Canadian Urological Association journal》2020,14(10):299
Although not commonly available in Canada, cryosurgery (cryoablation) for prostate cancer has been practiced in many countries. The field of cryoablation has evolved significantly over the past 30 years. Two prostate cryoablation programs were started in Canada in the early 1990s, in London, ON and Calgary, AB, focusing, respectively, on salvage therapy following radiation failure and primary local treatment. This article chronicles the development of the two programs and outlines the scientific and clinical contributions by investigators at the two centers. 相似文献
9.
Kassem Sharif Abdulla Watad Louis Coplan Benjamin Lichtbroun Alec Krosser Michael Lichtbroun Nicola Luigi Bragazzi Howard Amital Arnon Afek Yehuda Shoenfeld 《Autoimmunity reviews》2018,17(10):967-983
Stress is defined as the pscyophysiological reaction in which the steady state is disturbed or threatened. Stress is not always perceived as a negative response. Stress results when environmental demands exceed an individuals' adaptive capacities. Autoimmune diseases are heterogeneous group of chronic diseases which occur secondary to loss of self antigen tolerance. The etiopathogenesis of autoimmune disease is uncertain. Genetic factors as well as environmental factors appear to interplay, leading to a cascade of events resulting in disease onset. Stress has been postulated to play a role in disease onset in the genetically susceptible patients. During the stress response, catecholamines and glucocorticoids are released from locus coeruleus and adrenal gland. These biomolecules exert control over various immune cells in the innate and adaptive arms of the immune system, thereby altering the cytokine profile released. The increase of IL-4 promotes T-helper 2 (Th2) cell differentiation, while the decrease in IL-12 and the increased IL-10 production reduce the number of T-helper 1 (Th1) cells. The relationship between stress and autoimmune diseases is intricate. Stress has been shown to be associated with disease onset, and disease exacerbations in rheumatoid arthritis, systemic lupus erythematosus, inflammatory bowel disease, multiple sclerosis, Graves' disease as well as other autoimmune conditions. In certain conditions such as psoriasis, stress has been implicated in delaying lesion clearance upon the application of standard treatment regimes. Finally, psychological therapy and cognitive behavioral therapy aimed to reduce stress levels was shown to be effective in influencing better outcomes in many autoimmune diseases. The purpose of this paper is to closer inspect the clinical evidence regarding the role of stress on influencing the various aspects of disease entities. 相似文献
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