1,855 research outputs found

    RIC-HSCT for MF/SS

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    Advanced-stage mycosis fungoides and Sezary syndrome (MF/SS) have a poor prognosis. Allogeneic hematopoietic stem cell transplantation (HSCT), particularly using a reduced-intensity conditioning (RIC) regimen, is a promising treatment for advanced-stage MF/SS. We performed RIC-HSCT in nine patients with advanced MF/SS. With a median follow-up period of 954days after HSCT, the estimated 3-year overall survival was 85.7% (95% confidence interval, 33.4-97.9%) with no non-relapse mortality. Five patients relapsed after RIC-HSCT; however, in four patients whose relapse was detected only from the skin, persistent complete response was achieved in one patient, and the disease was manageable in other three patients by the tapering of immunosuppressants and donor lymphocyte infusion, suggesting that graft-versus-lymphoma effect and "down-staging" effect from advanced stage to early stage by HSCT improve the prognosis of advanced-stage MF/SS. These results suggest that RIC-HSCT is an effective treatment for advanced MF/SS

    Preparation of mono-sized epoxy/MF microcapsulesin the appearance of polyvinyl alcohol as co-emulsifier

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    For epoxy microcapsules embedded in concrete as mechanic-triggered self-healing adhesive, globular shape with uniform size is the basic requirement to ensure the solid shell broken and the liquid core released at a designed stress. In this paper, monodispersed melamine\u96formaldehyde (MF) resin-walled epoxy E-51 microcapsules were successfully fabricated in an in situ polycondensation process, in which a certain amount of polyvinyl alcohol (PVA) solution was added as coemulsifier to control the microcapsules\u92 shape and size. Detail investigation shows, with the cooperation of PVA, the microcapsule morphologies and size distribution were ease to be adjusted by the parameters such as emulsifying agents, agitation rate, pH value and acidification time

    ACT Family Violence Intervention Program review

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    This paper reports on a review of the Australian Capital Territory’s Family Violence Intervention Program, which provides an interagency response to family violence matters. The scope of the review was to analyse the program’s activities and outcomes using 2007–08 data provided by participating agencies, supported by in-depth interviews with key stakeholders including victims whose matters had been finalised in court. After the completion of this report, additional data from 2008–09 and 2009–10 was made available by some Family Violence Intervention Program (FVIP) participating agencies. Although not within the scope of this evaluation, these data pointed to some preliminary improvements in the FVIP

    NF membrane fouling by aluminum and iron coagulant residuals after coagulation-MF pretreatment

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    The effects of coagulant residuals on fouling of a nanofiltration (NF) membrane were investigated. Experiments were carried out with a laboratory-scale microfiltration (MF)-NF setup and a pilot MF-NF plant. In the laboratory-scale experiments, NF feed water was pretreated with poly-aluminum chloride (PACl) or alum followed by MF. NF membrane permeability declined when the feed water contained residual aluminum at 18 μg/L or more, but not when it was lower than 9 μg/L. When pretreated with ferric chloride, no substantial decline of NF membrane permeability was observed: residual iron did not affect the permeability. When SiO2 was added to the water before the pretreatment with PACl, the NF membrane permeability declined at about double the speed. Thermodynamic calculations and elemental analysis of foulants recovered from the membranes indicated that the majority of inorganic foulants were compounds composed of aluminum, silicate, and possibly potassium. In the pilot plant, NF feed was pretreated by PACl. Transmembrane pressure for NF doubled over 4.5 months of operation. Although the aluminum concentration in the NF feed was not high (30 μg/L), analysis of membrane foulants revealed excessive accumulation of aluminum and silicate, also suggesting that aluminum residuals caused the membrane fouling by alumino-silicates or aluminum hydroxide

    Solving a 50 year mystery of a missing OPA1 mutation: more insights from the first family diagnosed with autosomal dominant optic atrophy

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    Background: Up to the 1950s, there was an ongoing debate about the diversity of hereditary optic neuropathies, in particular as to whether all inherited optic atrophies can be ascribed to Leber's hereditary optic neuropathy (LHON) or represent different disease entities. In 1954 W. Jaeger published a detailed clinical and genealogical investigation of a large family with explicit autosomal dominant segregation of optic atrophy thus proving the existence of a discrete disease different from LHON, which is nowadays known as autosomal dominant optic atrophy (ADOA). Since the year 2000 ADOA is associated with genomic mutations in the OPA1 gene, which codes for a protein that is imported into mitochondria where it is required for mitochondrial fusion. Interestingly enough, the underlying mutation in this family has not been identified since then. Results: We have reinvestigated this family with the aim to identify the mutation and to further clarify the underlying pathomechanism. Patients showed a classical non-syndromic ADOA. The long term deterioration in vision in the two teenagers examined 50 years later is of particular note 5/20 to 6/120. Multiplex ligation probe amplification revealed a duplication of the OPA1 exons 7-9 which was confirmed by long distance PCR and cDNA analysis, resulting in an in-frame duplication of 102 amino acids. Segregation was verified in 53 available members of the updated pedigree and a penetrance of 88% was calculated. Fibroblast cultures from skin biopsies were established to assess the mitochondrial network integrity and to qualitatively and quantitatively study the consequences of the mutation on transcript and protein level. Fibroblast cultures demonstrated a fragmented mitochondrial network. Processing of the OPA1 protein was altered. There was no correlation of the OPA1 transcript levels and the OPA1 protein levels in the fibroblasts. Intriguingly an overall decrease of mitochondrial proteins was observed in patients' fibroblasts, while the OPA1 transcript levels were elevated. Conclusions: The thorough study of this family provides a detailed clinical picture accompanied by a molecular investigation of patients' fibroblasts. Our data show a classic OPA1-associated non-syndromic ADOA segregating in this family. Cell biological findings suggest that OPA1 is regulated by post-translational mechanisms and we would like to hypothesize that loss of OPA1 function might lead to impaired mitochondrial quality control. With the clinical, genetic and cell biological characterisation of a family described already more than 50 years ago, we span more than half a century of research in optic neuropathies

    Correction Factor on Dynamic Force in a Marsh Funnel Test for Tunneling

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    This paper presents an improvement on a previous model for predicting the Marsh funnel (MF) test that is used in slurry shield tunneling for evaluating the rheological properties of bentonite slurries. The improvement focuses on the prediction of the dynamic part for fluids with small MF times. The velocity profile of the Herschel-Bulkley fluid in a laminar pipe flow condition is first investigated and a correction factor is introduced in the improved model. Comparisons of results from experiments and calculations with the previous model confirm the improved performance over the existing model. The rheological parameters obtained from the improved model show good resemblance to those obtained from a laboratory viscometer. The work also provides a reference to similar applications such as fluid transportation through pipelines where dynamic pressure dominates and therefore should be correctly predicted considering its velocity profile in a laminar condition.Green Open Access added to TU Delft Institutional Repository 'You share, we take care!' - Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Offshore and Dredging Engineerin

    Thermal analysis of a miniature magnetic fluid seal installed in an implantable rotary pump

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    The capacity of a magnetic fluid (MF) seal is decreased by increased MF temperature. A cooling system for MF is limited in a miniature MF seal installed in an implantable rotary pump. MF temperature in an MF seal installed in an implantable rotary pump was studied. The temperature of MF in a rotary pump was measured in vitro. Also, steady-state thermal analyses were conducted for an implantable rotary pump model. The results showed that (1) the decrease in magnetization of an MF due to increased temperature is negligible when the heat transfer coefficient of the seal housing is greater than 500 W/(m2·K) and (2) the increased temperature is mainly due to heat flux from the motor, and the magnitude of temperature increase due to viscous friction in the MF is low. In conclusion, an MF seal can be used in an implantable rotary pump from the standpoint of heat characteristics
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