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Objectives: HbS/β+ patients’ presence in Italy increased due to immigration; these patients are clinically heterogeneous, and specific guidelines are lacking. Our aim is to describe a cohort of HbS/β+ patients, with genotype-phenotype correlation, in order to offer guidance for clinical management of such patients. Methods: Retrospective cohort study of HbS/β+ patients among 15 AIEOP Centres. Results: A total of 41 molecularly confirmed S/β+ patients were enrolled (1-55years, median 10.9) and classified on β+ mutation: IVS-I-110, IVS-I-6, promoter, and “others.” Prediagnostic events included VOC 16/41 (39%), ACS 6/41 (14.6%), sepsis 3/41 (3.7%), and avascular necrosis 3/41 (7,3%). Postdiagnostic events were VOC 22/41 (53.6% %), sepsis 4/41 (9.7%), ACS 4/41 (9.7%), avascular necrosis 3/41 (7.3%), aplastic crisis 2/41 (4.8%), stroke 1/41 (2.4%), ACS 1/41 (2.4%), and skin ulcerations 1/41 (2.4%). The IVS-I-110 group presented the lowest median age at first SCD-related event (P=.02 vs promoter group) and the higher median number of severe events/year (0.26 events/patient/year) (P=.01 vs IVS-I-6 and promoter groups). Promoter group presented a specific skeletal phenotype. Treatment regimen applied was variable among the centers. Conclusions: HbS/β+ is not always a mild disease. Patients with IVS-I-110 mutation could benefit from a standard of care like SS and S/β° patients. Standardization of treatment is needed.

Summary: Giant lipomas affecting the retroperitoneum and pelvis are quite rare. The surgical management of these lesions may be technically demanding and controversies exist with respect to diagnosis, competences being involved, type of surgical approach, radicality, and timing. A unique case presentation of a giant lipoma occupying the whole pelvis and the gluteal region is presented. Due to its size, many anatomical areas are involved, requiring the expertise of multiple specialists to treat. After multidisciplinary counseling, the lesion is radically resected in one stage by using a new videolaparoscopically assisted transperineal access to the pelvis. This type of surgical approach may be of interest for resecting pelvic tumors in women and men.

Primary myelofibrosis (PMF) is a Philadelphia-negative (Ph-) myeloproliferative disorder, showing abnormal CD34+ progenitor cell trafficking, splenomegaly, marrow fibrosis leading to extensive extramedullary haematopoiesis, and abnormal neoangiogenesis in either the bone marrow or the spleen. Monocytes expressing the angiopoietin-2 receptor (Tie2) have been shown to support abnormal angiogenic processes in solid tumors through a paracrine action that takes place in proximity to the vessels. In this study we investigated the frequency of Tie2 expressing monocytes in the spleen tissue samples of patients with PMF, and healthy subjects (CTRLs), and evaluated their possible role in favouring spleen angiogenesis. We show by confocal microscopy that in the spleen tissue of patients with PMF, but not of CTRLs, the most of the CD14+ cells are Tie2+ and are close to vessels; by flow cytometry, we found that Tie2 expressing monocytes were Tie2+CD14lowCD16brightCDL62-CCR2- (TEMs) and their frequency was higher (p = 0.008) in spleen tissue-derived mononuclear cells (MNCs) of patients with PMF than in spleen tissue-derived MNCs from CTRLs undergoing splenectomy for abdominal trauma. By in vitro angiogenesis assay we evidenced that conditioned medium of immunomagnetically selected spleen tissue derived CD14+ cells of patients with PMF induced a denser tube like net than that of CTRLs; in addition, CD14+Tie2+ cells sorted from spleen tissue derived single cell suspension of patients with PMF show a higher expression of genes involved in angiogenesis than that found in CTRLs. Our results document the enrichment of Tie2+ monocytes expressing angiogenic genes in the spleen of patients with PMF, suggesting a role for these cells in starting/maintaining the pathological angiogenesis in this organ.

The exchange bias effect has been investigated in a granular system composed of Fe particles (mean size 6 nm) embedded in an Fe oxide matrix. At T=5 K, the exchange field, the remanence and the coercivity have been found to strongly depend on the cooling field and on the temperature at which it is applied. The observed behavior is determined by the exchange coupling between the ferromagnetic nanoparticles and the magnetically disordered, cluster-glass-like matrix, whose spin configuration is affected by the field-cooling process.

We introduce a thermomechanical model describing dissipative phase transitions with thermal memory in terms of the entropy balance and the principle of virtual power written for microscopic movements. The thermodynamical consistence of this model is verified and existence of solutions is proved for a related initial and boundary value problem.

Nowadays, there is a global pressure for agencies with responsibilities in public investment to evaluate their policies and impacts in terms of the economic, social and environmental dimensions, avoiding supporting projects that pose risks to these aspects, thereby making it possible to improve the quality of life of the populations and long-term economic stability. The objective of this study was to verify the guidelines that guide the socio-environmental responsibility policies (SERP) of Brazilian financial institutions. The research was based on the analysis of sustainability reports of development agencies and development banks. The socio-environmental policies of the institutions were also analyzed under the light of the resolution of the National Monetary Council that regulates these issues. It was possible to verify that most financial institutions have socio-environmental policies as a result of the regulation that determines the mandatory nature of such policies. Indeed, more than 3/4 of the studied institutions published their SERP after such regulation. Thus, the institutionalization of these practices might be understood from a process of normative isomorphism.