94 research outputs found

    ORIGINAL RESEARCH Light Chain Amyloidosis: Patient Experience Survey from the Amyloidosis Research Consortium

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    The Author(s) 2015. This article is published with open access at Springerlink.com Introduction: Information detailing the experience of patients with light chain (AL) amyloidosis is lacking. The primary aim of this study was to gather data on the patient experience to understand the challenges i

    Referendum abrogativo e reviviscenza

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    Come già venne fatto in occasione del referendum Guzzetta - Segni nel 2007, il Master in Istituzioni parlamentari europee e il Dottorato di ricerca in Teoria dello Stato e istituzioni politiche comparate indicono un seminario sul tema “La reviviscenza in ambito referendario” giovedì 22 settembre alle ore 15,00 Sala delle Lauree Facoltà di Scienze politiche Università di Roma "La Sapienza Hanno partecipato tra gli altri: Adele Anzon, Francesco Saverio Bertolini , Roberto Borrello, Stefano Ceccanti, AngeloAntonio Cervati, Claudio Chiola, Francesco Clementi, Francesco D’Onofrio, GianniFerrara, Andrea Georgis , Alessandro Gigliotti,PierFrancesco Grossi, Vincenzo Iacovissi, Luca Imarisio, Michela Manetti, AndreaManzella, Valeria Marcenò, Stefano Merlini, Alessandro Pace, Alessandro Palanza , Stefano Passigli, Mario Patrono, Francesca Petrini , Giorgio Rebuffa, Paolo Ridola, Andrea Ridolfi , Teresa Serra, Luciano Violant

    Phillipsite at high pressure: a single-crystal X-ray synchrotron diffraction study

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    Phillipsite is a low Si/Al natural zeolite, often found as autogenic mineral in both “close” and “open” hydrologic system or in vugs of basalt, as an alteration product of volcanic glass. Along with laumontite, it is one of the most common zeolites found in oceanic basalts. In order to investigate the high-pressure behavior of phillipsite and its structural evolution at the atomic scale, we performed an in situ single-crystal synchrotron X-ray diffraction experiment up to 10 GPa with a diamond anvil cell, using a nominally penetrating pressure-transmitting fluid (methanol:ethanol:H2O = 16:3:1 mix) (Gatta, 2010). The unit-cell parameters and the structure refinements within the P-range investigated show that: 1) phillipsite does not adsorb further H2O molecules from the penetrating-transmitting fluid within the P-range investigated; 2) the configuration of the extra-framework population changes with pressure (between 2 and 3 GPa), affecting the elastic behavior of the mineral. More in details, two distinct compressional regimes have been observed, in which the bulk moduli differs drastically (i.e., KV = 89(8) GPa between 0 and 2 GPa, KV = 18.8(7) GPa between 3 and 9 GPa); 3) phillipsite is crystalline at least up to 10 GPa, and this is surprising if we consider its microporous nature; 4) all the P-induced effects are completely reversible in decompression. The structural refinements allowed us to describe the mechanisms, at the atomic scale, that govern its elastic behavior, which are mainly governed by inter-tetrahedral tilting. The relatively low compressibility of phillipsite at room-P and its relatively wide P-stability shown in this experiment suggests that this zeolite is a potential H2O carrier during the first phase of the oceanic crust subduction or, toward the industrial front, its potential use in systems for the mechanical energy storage/dissipation (Eroshenko et al., 2001; Soulard et al., 2004). Acknowledgements: The author acknowledges the Italian Ministry of Education, MIUR-Project: “Futuro in Ricerca 2012 - ImPACT- RBFR12CLQD”

    C-Finite Sequences and Riordan Arrays

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    Many prominent combinatorial sequences, such as the Fibonacci, Lucas, Pell, Jacobsthal and Tribonacci sequences, are defined by homogeneous linear recurrence relations with constant coefficients. These sequences are often referred to as C-finite sequences, and a variety of representations have been employed throughout the literature, largely influenced by the author’s background and the specific application under consideration. Beyond the representation through recurrence relations, other approaches include those based on generating functions, explicit formulas, matrix exponentiation, the method of undetermined coefficients and several others. Among these, the generating function approach is particularly prevalent in enumerative combinatorics due to its versatility and widespread use. The primary objective of this work is to introduce an alternative representation grounded in the theory of Riordan arrays. This representation provides a general formula expressed in terms of the vectors of constants and initial conditions associated with any recurrence relation of a given order, offering a new perspective on the structure of such sequences

    Cyclosporine A in Ullrich congenital muscular dystrophy: long-term results.

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    Six individuals with Ullrich congenital muscular dystrophy (UCMD) and mutations in the genes-encoding collagen VI, aging 5-9, received 3-5 mg/kg of cyclosporine A (CsA) daily for 1 to 3.2 years. The primary outcome measure was the muscle strength evaluated with a myometer and expressed as megalimbs. The megalimbs score showed significant improvement (P = 0.01) in 5 of the 6 patients. Motor function did not change. Respiratory function deteriorated in all. CsA treatment corrected mitochondrial dysfunction, increased muscle regeneration, and decreased the number of apoptotic nuclei. Results from this study demonstrate that long-term treatment with CsA ameliorates performance in the limbs, but not in the respiratory muscles of UCMD patients, and that it is well tolerated. These results suggest considering a trial of CsA or nonimmunosuppressive cyclosporins, that retains the PTP-desensitizing properties of CsA, as early as possible in UCMD patients when diaphragm is less compromised

    Seismic stratigraphy of the Miocene–Pleistocene sedimentary basins of the Northern Tyrrhenian Sea and western Tuscany (Italy)

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    Seismic and stratigraphic data of the inland Volterra Basin and of the Tuscan Shelf (Northern Tyrrhenian Sea) have been analysed to determine the tectono-sedimentary evolution of this part of the Northern Apennines from the early Miocene (about 20 Ma) to the present. The area is a good example for better understanding the evolution of postcollisional related basins. The study area is characterized by a series of sedimentary basins separated by tectonic ridges. Similar environmental conditions existed both onshore and offshore as indicated by the occurrence of similar seismic units. The units are separated by major unconformities. The cross-sectional geometries of the deposits of these basins, as defined through seismic reflection profiles, change in a quasi-regular manner through time and space. Early stages (late Burdigalian to early Tortonian) of evolution of the basins are marked by either flat-lying deposits, quasi-uniform in thickness, probably remnants of originally wider and shallow settings, or, in places, by relatively small bowl-shaped basins. The latter may have been strongly affected by the pre-existing topography and tectonics, as they developed at or near the leading edges of pre-Neogene substrate thrusts. These early deposits represent sedimentation during a transitional period from the end of compressional tectonics to the start of an extensional phase and represent a pre-narrow rift stage of evolution of the region. The subsequent stage of tectonic evolution (late Tortonian to early Messinian), where preserved, is recorded by fault-bounded triangular-shaped basins interpreted as half-grabens. This is one of the periods of major development of narrow rifts in the area. The following stage (late Messinian to Early Pliocene) is marked by variable types of basins, showing wide and deep bowl-shaped geometries persistent in the offshore, whereas inshore (Volterra Basin) they alternate with half-graben, synrift deposits. This period thus represents a transitional stage where part of the system is still affected by synrift sedimentation and part is developing into incipient post-rift conditions. This stage was followed in early to middle Pliocene times by wide bowl-shaped to blanket-type deposits both in offshore and in inshore areas, indicating regional post-rifting conditions. The pre-, syn- and post-rift stages have followed each other through time and space, starting first in the westernmost offshore area and shifting later toward the east, inshore

    Cyclosporine A in Ullrich congenital muscular dystrophy: long-term results.

    No full text
    Six individuals with Ullrich congenital muscular dystrophy (UCMD) and mutations in the genes-encoding collagen VI, aging 5-9, received 3-5 mg/kg of cyclosporine A (CsA) daily for 1 to 3.2 years. The primary outcome measure was the muscle strength evaluated with a myometer and expressed as megalimbs. The megalimbs score showed significant improvement (P = 0.01) in 5 of the 6 patients. Motor function did not change. Respiratory function deteriorated in all. CsA treatment corrected mitochondrial dysfunction, increased muscle regeneration, and decreased the number of apoptotic nuclei. Results from this study demonstrate that long-term treatment with CsA ameliorates performance in the limbs, but not in the respiratory muscles of UCMD patients, and that it is well tolerated. These results suggest considering a trial of CsA or nonimmunosuppressive cyclosporins, that retains the PTP-desensitizing properties of CsA, as early as possible in UCMD patients when diaphragm is less compromised
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