First report of anti-TIF1γ dermatomyositis in a patient with myelodysplastic syndrome.

Inflammatory myopathies as para-neoplastic phenomena were first described by Sterz in 1916. Recently, myositis specific autoantibodies were described in cancer-associated myositis. Anti-transcription intermediary factor 1 gamma (anti-TIF1γ) antibodies have been found in both young adults affected by juvenile dermatomyositis and in elderly patients with cancer-associated myositis. In this regard, we report herein the first case of anti-TIF1γ dermatomyositis secondary to a myelodysplastic syndrome

Treatment strategy introducing immunosuppressive drugs with glucocorticoids ab initio or very early in giant cell arteritis: A multicenter retrospective controlled study

Objective: Glucocorticoids (GC) are associated with side effects in giant cell arteritis (GCA). Immunosuppressive therapies (ITs) have given conflicting results in GCA, regarding GC sparing effect. Primary endpoint is to evaluate whether very early introduction of ITs in GCA minimize the rate of GC-induced adverse events, in terms of infections, new onset systemic arterial hypertension, GC-induced diabetes and osteoporotic fractures. Methods: A multicenter retrospective case-control study included 165 patients. One group included 114 patients who were treated with at least one IT given at diagnosis or within 3 months from the start of GC. A second group included 51 GCA who received only GC or an IT more than 3 months later. Results: The most frequently used ITs were: methotrexate (138 patients), cyclophosphamide (48 patients) and tocilizumab (27 patients). No difference was observed as concerns the follow-up time between groups [48.5 (IQR 26\u201372) vs 40 (IQR 24\u201369), p \u200b= \u200b0.3)]. The first group showed a significantly lower incidence of steroid-induced diabetes (8/114, 7% vs 12/51, 23.5%; p \u200b= \u200b0.003) and no differences for the rate of infections (p \u200b= \u200b0.64). The group was also exposed to lower doses of GC at first (p \u200b< \u200b0.0001) and third (p \u200b< \u200b0.0001, rank-sum test) month. Forty-four patients in the first group (38.6%) compared with 34 in the second one (66.7%) experienced at least one relapse (p \u200b= \u200b0.001). Conclusion: Very early introduction of IT in GCA lowered the incidence of steroid-induced diabetes, possibly due to the lower doses of GC in the first three months. Relapse rate was even lower

Forests and sustainable development in the Brazilian Amazon: history, trends, and future prospects.

Ongoing deforestation in the Brazilian Amazon is the outcome of an explicit federal project to occupy, integrate, and "modernize" the region. Although there have been isolated periods of deforestation control, most recently between 2004 and 2012, the overall trajectory of the region since the colonial period has been one of forest loss and degradation. Addressing this challenge is especially urgent in the context of adverse climate-ecology feedbacks and tipping points. Here we describe the trends and outcomes of deforestation and degradation in the Amazon.We then highlight how historical development paradigms and policies have helped to cement the land use activities and structural lock-ins that underpin deforestation and degradation. We emphasize how the grounds for establishing a more sustainable economy in the Amazon were never consolidated, leading to a situation where forest conservation and development remain dependent on external programs?punitive measures against deforestation and fire and public social programs. This situation makes progress toward a forest transition (arresting forest loss and degradation and restoring forest landscapes) highly vulnerable to changes in political leadership, private sector engagement, and global market signals. After summarizing these challenges, we present a suite of measures that collectively could be transformational to helping overcome destructive path dependencies in the region. These include innovations in agricultural management, improved forest governance through landscape approaches, developing a local forest economy, sustainable peri-urbanization, and the empowerment of women and youth. These initiatives must be inclusive and equitable, enabling the participation and empowerment of local communities, particularly indigenous groups who have faced numerous historical injustices and are increasingly under threat by current politics

Toxicity and Clinical Results after Proton Therapy for Pediatric Medulloblastoma: A Multi-Centric Retrospective Study

Medulloblastoma is the most common malignant brain tumor in children. Even if current treatment dramatically improves the prognosis, survivors often develop long-term treatment-related sequelae. The current radiotherapy standard for medulloblastoma is craniospinal irradiation with a boost to the primary tumor site and to any metastatic sites. Proton therapy (PT) has similar efficacy compared to traditional photon-based radiotherapy but might achieve lower toxicity rates. We report on our multi-centric experience with 43 children with medulloblastoma (median age at diagnosis 8.7 years, IQR 6.6, M/F 23/20; 26 high-risk, 14 standard-risk, 3 ex-infant), who received active scanning PT between 2015 and 2021, with a focus on PT-related acute-subacute toxicity, as well as some preliminary data on late toxicity. Most acute toxicities were mild and manageable with supportive therapy. Hematological toxicity was limited, even among HR patients who underwent hematopoietic stem-cell transplantation before PT. Preliminary data on late sequelae were also encouraging, although a longer follow-up is needed

PO-0773: Reirradiation by extracranial stereotactic treatment: preliminary results of a dose escalation study

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Italian and English local funding networks: Is there a winning formula?

This article contributes to the understanding of European Local Governance by exploring the European funding networks developed within local areas under Objective 2 of the Structural Funds. The article describes the EU funding networks (within the theoretical perspective of network theory) and examines the relationship between the presence of local EU funding networks and councils' success in accessing EU funds. This exploratory study focuses on the appearance of policy networks at the local government level in Italy and England and discusses their durability and implications for their future