226 research outputs found

    Oxidative stress in Duchenne muscular dystrophy: focus on the NRF2 redox pathway

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    Oxidative stress is involved in the pathogenesis of Duchenne muscular dystrophy (DMD), an X-linked genetic disorder caused by mutations in the dystrophin gene and characterized by progressive, lethal muscle degeneration and chronic inflammation. In this study, we explored the expression and signaling pathway of a master player of the anti-oxidant and anti-inflammatory response, namely NRF2, in muscle biopsies of DMD patients. We classified DMD patients in two age groups (Class I, 0-2 years and Class II, 2-9 years), in order to evaluate the antioxidant pathway expression during the disease progression. We observed that altered enzymatic antioxidant responses, increased levels of oxidized glutathione and oxidative damage are differently modulated in the two age classes of patients and well correlate with the severity of pathology. Interestingly, we also observed a modulation of relevant markers of the inflammatory response, such as heme oxygenase 1 and IL-6, suggesting a link between oxidative stress and chronic inflammatory response. Of note, using a transgenic mouse model, we demonstrated that IL-6 overexpression parallels the antioxidant expression profile and the severity of dystrophic muscle observed in DMD patients. This study advances our understanding of the pathogenic mechanisms underlying DMD and defines the critical role of oxidative stress on muscle wasting with clear implications for disease pathogenesis and therapy in human

    Oxidative stress in Duchenne muscular dystrophy: focus on the NRF2 redox pathway

    Get PDF
    Oxidative stress is involved in the pathogenesis of Duchenne muscular dystrophy (DMD), an X-linked genetic disorder caused by mutations in the dystrophin gene and characterized by progressive, lethal muscle degeneration and chronic inflammation. In this study, we explored the expression and signaling pathway of a master player of the anti-oxidant and anti-inflammatory response, namely NRF2, in muscle biopsies of DMD patients. We classified DMD patients in two age groups (Class I, 0-2 years and Class II, 2-9 years), in order to evaluate the antioxidant pathway expression during the disease progression. We observed that altered enzymatic antioxidant responses, increased levels of oxidized glutathione and oxidative damage are differently modulated in the two age classes of patients and well correlate with the severity of pathology. Interestingly, we also observed a modulation of relevant markers of the inflammatory response, such as heme oxygenase 1 and IL-6, suggesting a link between oxidative stress and chronic inflammatory response. Of note, using a transgenic mouse model, we demonstrated that IL-6 overexpression parallels the antioxidant expression profile and the severity of dystrophic muscle observed in DMD patients. This study advances our understanding of the pathogenic mechanisms underlying DMD and defines the critical role of oxidative stress on muscle wasting with clear implications for disease pathogenesis and therapy in human

    Implant insertion torque value in immediate loading : a retrospective study

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    The aim of this study is to verify if the Insertion Torque Value (ITV) of 32 Ncm for immediate loading protocol (ILP), as indicated by literature, is still, with the advance in implant research, a real significant cut-off for long-term implant survival. In this retrospective study, data from 224 patients that during three years of clinical practice, were submitted to the insertion of 322 implants with immediate loading protocol, have been recorded, pooled and analyzed. Data were organized based on Insertion Torque Value (ITV): > 32 Ncm (CG) and 32 Ncm are still characterized by a lower crestal bone resorption, there are no statistically significant differences among the two groups for what concerning the failure rate during the 2 years of follow-up and OR. These results permit us to suppose that the cut-off of ITV >32 Ncm for immediate loading implants, could be reduced to inferior values. However further studies are necessary to indicate precise clinical guidelines

    The Impact of COVID-19 on Horticulture: Critical Issues and Opportunities Derived from an Unexpected Occurrence

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    The COVID-19 pandemic is causing many victims worldwide and has generated a serious economic crisis. Substantial changes have occurred in the food and ornamental production chains. The aim of the present review has been to summarize some of the main effects that the pandemic is having on horticulture and on the new habits of people. Infections and quarantine measures have prevented the regular flow of certain goods and of connected services. Cases of shortages and/or surpluses, a lack of the availability of labor, and a reduction in demand for some food products and flowers have occurred. New food production approaches have emerged and a reconnection between farmers and consumers has been spreading, thereby facilitating product distribution. Moreover, during the forced isolation, people have had to face periods of stress. The benefits that can be derived from leisure activities related to flowers and ornamental plants, and from access to nature and urban green spaces are increasingly being recognized as relevant. The seriousness of the pandemic will inevitably lead to lasting changes. Therefore, the vulnerability of the pre-COVID-19 distribution chains should be considered and a new food production chain should be drawn up, to increase the resilience of such systems

    Prethymic cytoplasmic CD3 negative acute lymphoblastic leukemia or acute undifferentiated leukemia: a case report

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    Acute undiffentiated leukemia (AUL) is an acute leukemia with no more than one membrane marker of any given lineage. Blasts often express HLA-DR, CD34, and/or CD38 and may be positive for terminal deoxynucleotidyl transferase (TdT). The expression of CD34, HLA-DR, and CD38 has been shown in pro-T-ALL, although in this case, blasts should also express CD7 and cyCD3. However, some cases of T-ALL without CD3 in the cytoplasm and all TCR chain genes in germ line configuration are reported, features that fit well with a very early hematopoietic cell. We report a case of acute leukemia CD34+/-HLADR+CD7+CD38+cyCD3- in which a diagnosis of AUL was considered. However the blasts were also positive for CD99 and TCR delta gene rearrangement which was found on molecular studies. Therefore a differential diagnosis between AUL and an early cyCD3 negative T-ALL was debated

    Osteoblasts and Fibroblasts Interaction with a Porcine Acellular Dermal Matrix Membrane

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    The use of collagen membranes has remained the gold standard in GTR/GBR. In this study, the features and the biological activities of an acellular porcine dermis collagen matrix membrane applicable during dental surgery were investigated, and also by applying hydration with NaCl. Thus, two tested membranes were distinguished, the H-Membrane and Membrane, compared to the control cell culture plastic. The characterization was performed by SEM and histological analyses. In contrast, the biocompatibility was investigated on HGF and HOB cells at 3, 7, and 14 days by MTT for proliferation study; by SEM and histology for cell interaction study; and by RT-PCR for function-related genes study. In HOBs seeded on membranes, mineralization functions by ALP assay and Alizarin Red staining were also investigated. Results indicated that the tested membranes, especially when hydrated, can promote the proliferation and attachment of cells at each time. Furthermore, membranes significantly increased ALP and mineralization activities in HOBs as well as the osteoblastic-related genes ALP and OCN. Similarly, membranes significantly increased ECM-related and MMP8 gene expression in HGFs. In conclusion, the tested acellular porcine dermis collagen matrix membrane, mainly when it is hydrated, behaved as a suitable microenvironment for oral cells

    Simultaneous presentation of Waldenström macroglobulinemia and multiple myeloma: multidisciplinary diagnosis, treatment and 30-month follow-up.

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    Waldenström macroglobulinemia and multiple myeloma are mature B-cell neoplasms deriving from post-germinal cells at different stages of differentiation. The simultaneous presentation of Waldenström macroglobulinemia and multiple myeloma in the same patient is a very rare phenomenon and, so far, only two cases have been described. We report the case of a 75-year Caucasian female patient, with a silent clinical history, who presented with anemia and two different monoclonal proteins (IgMκ and IgGκ). The trephine biopsy showed the presence of a dual population, represented by small lymphoplasmacytoid cells and by plasma cells, which infiltrated the bone marrow with a clearly different pattern. Both immunohistochemistry and flow cytometry demonstrated the biclonal origin such neoplastic cells, since lymphoplasmacytoid cells resulted IgMκ while plasma cells were IgGκ. This biclonal pattern was further confirmed by the demonstration of a different IgH gene rearrangement of the two neoplasms. The patient was treated with bortezomib, dexamethasone and rituximab, achieving partial remission of both Waldenström macroglobulinemia and multiple myeloma. After a 30-month follow-up, she is in stable disease. Multiple myeloma has been described in association with other indolent B-cell neoplasms, mostly chronic lymphocytic leukemia, while Waldenström macroglobulinemia can be followed by diffuse large B-cell lymphoma in some instances, after chemotherapy. The association of Waldenström macroglobulinemia and multiple myeloma seems to be very rare. Our study shows that an integrated diagnostic work-up is very useful in such cases, with an interesting role for flow cytometry. [J Clin Exp Hematop 53(1): 29-36, 2013]

    O árbitro de futebol e a importância dos aspectos psicológicos: olhares da psicologia do esporte

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    Designed to impose and lead athletes to comply with the rules of the game during a match, referees are pressured from all sides, because unlike players, a single mistake can be costly. Therefore, the present study aims to organize contents about the psychological skills necessary for the good performance of the Football referee, proposing strategies to improve the psychological skills of the football referees. Thus, this study is characterized as a descriptive-purpose bibliographic study. In the content organization, it was observed that the psychological preparation should be part of the preparation of the same, as well as the physical preparation, that is, the referee should be well physically and psychologically, presenting psychological qualities that help him to face all the negative psychological demands that a football match can cause. In addition, the physical preparation will contribute to the good performance of the referee, since he/she can position himself /herself adequately to observe the bid, reducing doubts about what has occurred and, consequently, reducing the psychological burden in decision making. Thus, it is concluded that psychological skills, if worked properly, only favor the work of the football referee, leading to success
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