Quantification of paravalvular leaks associated with TAVI implants using 4D MRI in an aortic root phantom made possible by the use of 3D printing

IntroductionTranscatheter aortic valve implantation (TAVI) has become an alternative to surgical replacement of the aortic valve elderly patients. However, TAVI patients may suffer from paravalvular leaks (PVL). Detecting and grading is usually done by echocardiography, but is limited by resolution, 2D visualization and operator dependency. 4D flow magnetic resonance imaging (MRI) is a promising alternative, which did not reach clinical application in TAVI patients. The aim of this study was applying 3D printing technologies in order to evaluate flow patterns and hemodynamics of PVLs following TAVI, exploiting 4D flow MRI and standard ultrasound. Materials and methodsAn MR-compatible, anatomically left ventricle, aortic root, and ascending aorta model was fabricated by combining 3D-printed parts and various soft silicone materials to match physiological characteristics. An Abbott Portico (TM) valve was used in continuous antegrade flow (12-22 l/min), retrograde flow with varying transvalvular pressures (60-110 mmHg), and physiological pulsatile hemodynamics (aortic pressure: 120/80 mmHg, cardiac output: 5 l/min) Time-resolved MR measurements were performed above and below the TAVI stent and compared with color Doppler ultrasound measurements in exactly the same setup. ResultsThe continuous antegrade flow measurements from MRI largely agreed with the flowmeter measurements, and a maximum error of only 7% was observed. In the retrograde configuration, visualization of the paravalvular leaks was possible from the MR measurements, but flow was overestimated by up to 33%. The 4D MRI measurement in the pulsatile setup revealed a single main PVL, which was also confirmed by the color Doppler measurements, and velocities were similar (2.0 m/s vs. 1.7 m/s). Discussion4D MRI techniques were used to qualitatively assess flow in a patient-specific, MR-compatible and flexible model, which only became possible through the use of 3D printing techniques. Flow patterns in the ascending aorta, identification and quantification of PVLs was possible and the location and extent of PVLs were confirmed by ultrasound measurements. The 4D MRI flow technique allowed evaluation of flow patterns in the ascending aorta and the left ventricle below the TAVI stent with good results in identifying PVLs, demonstrating its capabilities over ultrasound by providing the ability to visualize the paravalvular jets in three dimensions at however, additional expenditure of time and money

Early onset of Chanarin-Dorfman syndrome with severe liver involvement in a patient with a complex rearrangement of ABHD5 promoter

BACKGROUND: \u3b1/\u3b2-hydrolase domain-containing protein 5 (ABHD5) plays an important role in the triacylglycerols (TAG) hydrolysis. Indeed, ABHD5 is the co-activator of adipose triglyceride lipase (ATGL), that catalyses the initial step of TAG hydrolysis. Mutations in ABHD5 gene are associated with the onset of Chanarin-Dorfman syndrome (CDS), a rare autosomal recessive lipid storage disorder, characterized by non-bullous congenital ichthyosiform erythroderma (NCIE), hepatomegaly and liver steatosis. CASE PRESENTATION: We describe here a 5-years-old Brazilian child who presented with NCIE at birth and diffuse micro and macro-vesicular steatosis on liver biopsy since she was 2 years old. Molecular analysis of coding sequence and putative 5' regulatory region of ABHD5 gene was performed. A homozygous novel deletion, affecting the promoter region and the exon 1, was identified, confirming the suspected diagnosis of CDS for this patient. RT-PCR analysis showed that the genomic rearrangement completely abolished the ABHD5 gene expression in the patient, while only a partial loss of expression was detected in her parents. This is the first report describing the identification of a large deletion encompassing the promoter region of ABHD5 gene. The total loss of ABHD5 expression may explain the early onset of CDS and the severe liver involvement. After molecular diagnosis, the patient started a special diet, poor in fatty acids with medium chain triglycerides (MCT), and showed hepatic and dermatologic improvement in spite of severe molecular defect. CONCLUSIONS: This case report extends the spectrum of disease-causing ABHD5 mutations in CDS providing evidence for a novel pathogenic mechanism for this rare disorder. Moreover, our preliminary data show that early diagnosis and prompt treatment of neutral lipid accumulation might be useful for CD patients

Cultural Mapping

Cultural mapping is a mode of inquiry and a methodological tool that aims to make visible the ways local stories, practices, relationships, memories, and rituals constitute places as meaningful locations. Although there is still a fuzziness to the boundaries of this field, cultural mapping has generally evolved along two main branches: The first begins with cultural assets, seeking to identify and document tangible and intangible assets of a place to ultimately develop a cultural resource or asset mapping. The second branch begins with a culturally sensitive humanistic approach, seeking to articulate a “sense of place,” people-place meanings, and distinctive elements. While the former approach tends to emphasize the documentation of “information” and the latter tends to focus more on “participation” and “meaning,” they are increasingly mutually informing approaches. Cultural mapping/cartography is allied with deep mapping, community mapping, participatory asset mapping, counter-mapping, qualitative GIS, and emotional mapping. These are connected through their focus on bottom-up processes for making visible the knowledge of citizens/residents, and shared topics of narratives, identity, histories, and local practices that bring meanings to places. Cultural mapping has shifted from focusing on tangible cultural assets to intangible aspects of place, aiming to discover what makes a place distinctive. Cultural products such as literature, film, and music draw from and contribute to the cultural meanings of a place; and the mapping of these onto a territory also forms a branch of cultural mapping. This work is also found within the fields of geography (see the separate Oxford Bibliographies in Geography articles “Geography and Literature,” “Geography and Film,” and “Geographies of Music, Sound, and Auditory Culture”), tourism, and digital humanities. Artist map traditions also influence the field of cultural mapping (see the section on “Map Art” within the separate Oxford Bibliographies in Geography article “Community Mapping”), with artists taking on a leadership role in many community-engaged cultural mapping initiatives. Cultural mapping has been co-developed through a loosely formulated international community of practice consisting of scholars, in-community practitioners, and policy/governance agencies (e.g., UNESCO, national cultural ministries, local authorities). While this annotated bibliography focuses mainly on the leading scholarly work in this field, it also provides an international selection of cultural mapping handbooks and toolkits as well as examples of cultural mapping projects. Following General Overviews, Special Journal Issues, Methodologies, and Theoretical Underpinnings of the field, this entry is organized according to seven domains of contemporary cultural mapping research and practice: Community Engagement, Participation, and Empowerment; Indigenous Cultural Mapping; Cultural and Creative Industries Mapping; Local Cultural Planning and Governance; Artistic Approaches; Literary and Film Mapping; and Technological Approaches

[In Press] Arts in the city : debates in the Journal of Urban Affairs

There has been growing interest in the urban affairs literature in investigating the role of the arts in the city because of its multifaceted potential to impact a place. To document central elements of the emerging debates on the arts and the city over the last 20 years, we examined key articles on the subject published in the Journal of Urban Affairs and high-lighted their links with several special issues of other journals. The debates canvased over this time can be organized as (1) defining artists as human capital, (2) studying cultural amenities, and (3) examining the role of art around the city. This analysis uncovers some of the themes, methods, and places articulating these academic debates and identifies pivotal overarching economic, symbolic, and social issues. The paper suggests that key directions for future research include widening the journals to be analyzed; developing bibliometric analyses; broadening the geographic selection of case studies, including research in languages other than English; and encouraging more special journal issues focused on art and the city to facilitate multidisciplinary conversations

Different approaches for minimising proton beam losses on the 11 T dipole in the IR7 dispersion suppressor

The High Luminosity Large Hadron Collider (HL–LHC) project aims at increasing the integrated luminosity by a factor of 10 beyond the LHC design value. With the increased intensities of the proton beams expected for HL–LHC, the losses on superconducting magnets in the dispersion suppressor (DS) regions of the accelerator could limit the machine performance by inducing magnet quenches. In order to improve the collimation cleaning, the HL–LHC upgrade baseline foresees to replace one standard 8.33 T main dipole with a pair of shorter and stronger 11 T dipoles and a collimator (Target Collimator Long Dispersion suppressor, TCLD) installed in between for each beam and on each side of the betatron cleaning insertion. Different locations were compared for the optimal installation of the TCLD in terms of global and local cleaning, leading to the choice of cell 9 as baseline location. This note reviews different mitigation strategies and techniques performed to minimize the amount of losses reaching the upstream 11 T dipole that is not protected by the new collimator. Possible operational scenarios are also presented

Impact of COVID-19 lockdown in children with neurological disorders in Italy

Background: The costs and benefits of full lockdown measures are debated. Neurologically impaired children are a vulnerable population with specific needs in terms of protection against infection and access to health services. Objectives: We investigated the effects of lockdown on the health of children with neurological disorders and on their access to care during lockdown. Methods: Data from 514 children (282 males e 232 females) were collected through physician administered interviews to investigate: the occurrence of viral-like physical symptoms, the correlation between the risk of developing such symptoms and several demographic and clinical variables, the occurrence of any worsening of the children's neurological conditions during lockdown, and their access to care services during this period.Results: 49.1% experienced at least one symptom during the study period, but no child developed severe complications. The prevalence of symptoms was significantly lower during lockdown than during the previous two months. The underlying neurological condition worsened in 11.5% of the patients. Children who regularly left the home during lockdown were greater risk of exhibiting symptoms. During lock down, 67.7% had a specialist appointment cancelled, 52.6% contacted their paediatrician, and 30.9% contacted their child neuropsychiatrist. Among patients who usually receive rehabilitation, 49.5% continued remotely.Conclusion: Lockdown protected children from infections. Telemedicine and telerehabilitation constituted a valid alternative for the care and treatment of these children, but they should not become a widespread and definitive model of care. COVID-19 and other emergency response plans must take into account the specific needs of children with disabilities.(c) 2020 Elsevier Inc. All rights reserved

Coronary Perfusion After Valve-in-Valve Transcatheter Aortic Valve Implantation in Small Aortic Root: In Vitro Experimental Assessment

Coronary flow obstruction following transcatheter aortic valve-in-valve implantation (VIV-TAVI) is associated with a high mortality risk. The aim of this work was to quantify the coronary perfusion after VIV-TAVI in a high-risk aortic root anatomy. 3D printed models of small aortic root were used to simulate the implantation of a TAVI prosthesis (Portico 23) into surgical prostheses ( Trifecta 19 and 21). The aortic root models were tested in a pulsatile in vitro bench setup with a coronary perfusion simulator. The tests were performed at baseline and post-VIV-TAVI procedure in aligned and misaligned commissural configurations under simulated hemodynamic rest and exercise conditions. The experimental design provided highly controllable and repeatable flow and pressure conditions. The left and right coronary mean flow did not differ significantly at pre- and post-VIV-TAVI procedure in any tested configurations. The commissural misalignment did not induce any significant alterations to the coronary flow

Power Deposition in Superconducting Dispersion Suppressor Magnets Downstream of the Betatron Cleaning Insertion for HL-LHC

The power deposited in dispersion suppressor magnets downstream of the Large Hadron Collider (LHC) betatron cleaning insertion is governed by off-momentum particles scattered out of the primary collimators. In order to mitigate the risk of magnet quenches during periods of short beam lifetime in future High-Luminosity (HL-LHC) operation, new dispersion suppressor (DS) collimators are considered for installation (one per beam). In this paper, we present FLUKA simulations for both protons and Pb ions at 7 TeV, predicting the power deposition in the DS magnets, including the new higher-field dipoles 11T which are needed to integrate the collimator in the cold region next to the cleaning insertion. The simulated power deposition levels for the adopted HL-LHC collimator configuration and settings are used to assess the quench margin by comparison with the present estimated quench levels