Long-term follow-up of Philadelphia chromosome-positive (Ph+) chronic myeloid leukaemia (CML) in children and adolescents managed at a single institution over a 20-year period

Chronic myeloid leukaemia (CML) is rare in childhood. In our Institution we managed 30 consecutive Ph+CML patients aged <18 years, according to our adults’ guidelines. Patients with HLA-identical related donor (RD) underwent stem cell transplant (SCT). Since 1989, patients without RD were systematically treated with -interferon (IFN) (median dosage: 6 MU/day). Of 18/19 evaluable patients, 17 (94.5%) achieved haematologic response (HR), 11/17 (65%) cytogenetic response (CyR), complete (CCyR) in 4 (23.5%). Three patients remain in CCyR, 2 achieved BCR-ABL transcript disappearance. Of 13 patients without CCyR, 5 underwent SCT, 4 switched to STI571, 4 progressed. All patients receiving STI571 in chronic phase (CP) obtained sustained CCyR and 3 a persistent molecular response. 8-year survival among IFN-treated patients, censored or not for subsequent therapies, is 62% and 63%. Overall, 13/30 patients underwent SCT: 5 HLA-identical-RD, 5 matched unrelated donor, 2 mismatched-RD, 1 unrelated mismatched umbilical cord blood. Eight allotransplanted patients (6/6 in 1st CP) are in cytogenetic and molecular remission with 8-year survival of 61% from SCT and 69% from diagnosis. In our 20-year experience, the use of IFN in children without matched RD led to prolonged cytogenetic and molecular responses and long-term survival, without impairing the outcome of subsequent SCT

Management of craniofacial development in the Parry-Romberg syndrome: Report of two patients

Objective: The aim of this article is to describe the orthodontic therapy for Parry-Romberg syndrome. The therapeutic goal is to minimize the wasting effects of progressive atrophy on facial development of a part of the face. Design: To correct problems affecting craniofacial development of these patients, occurring during puberty, an orthodontic appliance was employed, which helps maintain parallelism of the facial planes, in particular the mandibular plane. Setting: Orthodontic care was carried out in the Dental Clinic of the Catholic University of the Sacred Heart of Rome. Intervention: Two patients underwent orthodontic therapy for 6 years. Appliances were checked every month and modified periodically so as to adapt to facial bone growth. Results: At the end of craniofacial growth, the mandible was almost symmetric and the problem relating to atrophy remained confined to the initial area. Cephalometric analyses demonstrated that the occlusal plane and the mandibular plane maintained a straight orientation in relation to the bizygomatic plane. The ratio between the left and right side of the ramus and condyle, in the mandible, improved. Conclusions: The use of orthodontic therapy allows patients affected by hemifacial progressive atrophy to present a more harmonic face at the end of puberty when final reconstruction can be planned. These results provide for a limitation of surgical intervention to the sclerodermic area alone

Fold and thrust belts : structural style, evolution and exploration – an introduction

Peer reviewedPublisher PD

Linear Time-History Analysis for EC8 design of CBF structures

Increased availability of strong motions, improved computational capabilities and availability of open-source software for Eurocode 8-compliant record selection make Linear Time History Analysis (LTHA) a potential more rigorous analysis approach for the design of structures. This methodology is not explicitly codified in the Eurocode and it requires some "code-friendly" recommendations for its implementation in practice. Herein, LTHA is employed for the first time as design method for a five-storey archetype high ductility class concentric brace frame (CBF). A new-optimized design method for braces is presented for the smooth implementation of both Response Spectrum Analysis (RSA) and LTHA. In fact, the tension-only design approach for CBF in Eurocode 8 has some criticalities arising even when a routine RSA is employed. The design process is rather smooth only in the case of linear static approach classically implemented for regular, mid-rise structures. Results of this study provide insights for validation of LTHA as design method for CBFs, and for its code implementation as routine design method in EC8, valuable in near source conditions or for irregular structural configurations

Pinsight: A Novel Way of Creating and Sharing Digital Content through 'Things' in the Wild

Existing platforms for sharing locative digital content rely on the use of mobile phones for accessing the content. This can be a major deterrent to wider public access and also hinders immediacy and 'in the moment' discoverability. Building on previous work in situated public installations, we developed Pinsight, a novel platform for enabling end-users, such as local communities, to create and share digital content in-situ with public audiences through physical interactive devices. Pinsight is based on a set of design principles that focus on supporting both the expressiveness of content creators and the appeal to public audiences. This paper describes the design of the platform and how it supports sharing knowledge in ways different to conventional media. Through preliminary evaluations and two in-the-wild studies, we explore how such a situated technology can be used by different user groups (content designers, history communities, local residents) for sharing content with public audiences (visitors, pedestrians, residents) in different contexts

High sensitive TROponin levels In Patients with Chest pain and kidney disease:a multicenter registry: The TROPIC study

  Background: Accuracy of high sensitive troponin (hs-cTn) to detect coronary artery disease (CAD) in patients with renal insufficiency is not established. The aim of this study was to evaluate the prognostic role of hs-cTn T and I in patients with chronic kidney disease (CKD). Methods: All consecutive patients with chest pain, renal insufficiency (eGFR &lt; 60 mL/min/1.73 m2) and high sensitive troponin level were included. The predictive value of baseline and interval troponin (hs-cTnT and hs-cTnI) for the presence of CAD was assessed. Results: One hundred and thirteen patients with troponin I and 534 with troponin T were included, with 95 (84%) and 463 (87%) diagnosis of CAD respectively. There were no differences in clinical, procedural and outcomes between the two assays. For both, baseline hs-cTn values did not differ be­tween patients with/without CAD showing low area under the curve (AUC). For interval levels, hs-cTnI was significantly higher for patients with CAD (0.2 ± 0.8 vs. 8.9 ± 4.6 ng/mL; p = 0.04) and AUC was more accurate for troponin I than hs-cTnT (AUC 0.85 vs. 0.69). Peak level was greater for hs-cTnI in patients with CAD or thrombus (0.4 ± 0.6 vs. 15 ± 20 ng/mL; p = 0.02; AUC 0.87: 0.79–0.93); no differences were found for troponin T assays (0.8 ± 1.5 vs. 2.2 ± 3.6 ng/mL; p = 1.7), with lower AUC (0.73: 0.69–0.77). Peak troponin levels (both T and I) independently predicted all cause death at 30 days. Conclusions: Patients with CKD presenting with altered troponin are at high risk of coronary disease. Peak level of both troponin assays predicts events at 30 days, with troponin I being more accurate than troponin T. (Cardiol J 2017; 24, 2: 139–150

Similarities and differences in the dolomitization history of two coeval Middle Triassic carbonate platforms, Balaton Highland, Hungary

Dolomitization of platform carbonates is commonly the result of multiphase processes. Documentation of the complex dolomitization history is difficult if completely dolomitized sections are studied. Two Middle Anisian sections representing two coeval carbonate platforms were investigated and compared in the present study. Both sections are made up of meter-scale peritidal–lagoonal cycles with significant pedogenic overprint. One of the sections contains non-dolomitized, partially dolomitized, and completely dolomitized intervals, whereas the other is completely dolomitized. Based on investigations of the partially dolomitized section, penecontemporaneous dolomite formation and/or very early post-depositional dolomitization were identified in various lithofacies types. In shallow subtidal facies, porphyrotopic dolomite was found preferentially in microbial micritic fabrics. Microbially induced dolomite precipitation and/or progressive replacement of carbonate sediments could be interpreted for stromatolites. Cryptocrystalline to very finely crystalline dolomite, probably of pedogenic origin, was encountered in paleosoil horizons. Fabric-destructive dolomite commonly found below these horizons was likely formed via reflux of evaporated seawater. As a result of the different paleogeographic settings of the two platforms, their shallow-burial conditions were significantly different. One of the studied sections was located at the basinward platform margin where pervasive fabric-retentive dolomitization took place in a shallow-burial setting, probably via thermal convection. In contrast, in the area of the other, smaller platform shallow-water carbonates were covered by basinal deposits, preventing fluid circulation and accordingly pervasive shallow-burial dolomitization. In the intermediate to deep burial zone, recrystallization of partially dolomitized limestone and occlusion of newly opened fractures and pores by coarsely crystalline dolomite took place

Design of steel CBF structures through Linear Time-History Analysis

This thesis points out the problems related to the design of Steel Concentrically Braced Frame structures in seismic areas. The European seismic code (EC8) defines the limits in design of CBFs, which are very difficult to comply with by using steel profiles available on market. Moreover, the linear dynamic analyses of CBF structures have several difficulties in implementation. In this work, a five storeys steel structure, located in a seismic area, has been selected for design and analysed through RSA and LTHA. The Response Spectrum Analysis has the problem of the loss of the sign for axial loads in concentric braces. For this reason, Linear Time History Analysis (LTHA) as alternative linear methodology for seismic design according to Eurocode8 becomes an appealing alternative for CBFs

La comunicazione politica di Matteo Renzi = The political communication of Matteo Renzi

The political communication of Matteo Renzi. On 8the December 2012, Matteo Renzi became the secretary of the Democratic Party with 68.1 percent of the vote. A few months later he is the youngest prime minister in the history of the Italian Republic. Renzi becomes the icon of the national policy, the central character in the redefinition of the new communication strategies. From the beginning, the already former mayor of Florence is characterized by a language distinguished by an informal register, which compels political opponents to change the approach to the electorate. A skilled user of the new instruments offered by the so-called Web 2.0 and Social Networks, Matteo Renzi feels the effects of the acceleration that the television contents and the new rhythms of the 80s have marked our language. The analysis aims to investigate, from the rhetorical of view, the different aspects of consensus-building and electoral tactics adopted by the Secretary of the Democratic Party. In the background, the changes recorded in the broader context of political communication in the light of the extraordinary success of television, with its logic of simplification, the collapse of the parties, the loss of consent and the increasing personalization of politic

L'approccio SCM ai problemi di operabilità in gasolio B7. Il caso Shell Italia.

According to recent European Directives 2009/28/EC and FQD 2009/30/EC, the promoting of biofuels use and their sustainability appears to be an interesting and relevant topic. Biodiesel has an important role because of his availability and standardization but some technical limits, significantly affecting the operability, have been observed in the mixture of it with diesel fuel. Microbial growth and poor cold flow properties impacted on the industry and created several issues on the correct supply operations in retail site. A particular feature of the work is the Supply Chain Management approach in which the Shell Italia PQ Team took up the issue. The management approach to the problem made it possible, through careful analytical study of existing data, to trace the causes, identity the impacts, solve it and generate operational procedures to safeguard the quality of oil products. The analysis and subsequent actions have covered all the key points of Shell Italia’s supply chain and required a deep study on biodiesel production processes, on the physical and chemical properties of the B7 mixture and on the associated European regulations