3,248 research outputs found
Luspatercept diminishes the need for red blood cell replacement in transfusion-dependent β-thalassemia patients
A clinical decision report appraising:
Cappellini MD, Viprakasit V, Taher AT, et al. A phase 3 trial of luspatercept in patients with transfusion-dependent β-thalassemia. N Engl J Med. 2020;382(13):1219-1231. https://doi.org/10.1056/NEJMoa1910182
for a patient with transfusion-dependent beta-thalassemia
A Survey on the Classical Limit of Quantum Dynamical Entropies
We analyze the behavior of quantum dynamical entropies production from
sequences of quantum approximants approaching their (chaotic) classical limit.
The model of the quantized hyperbolic automorphisms of the 2-torus is examined
in detail and a semi-classical analysis is performed on it using coherent
states, fulfilling an appropriate dynamical localization property.
Correspondence between quantum dynamical entropies and the Kolmogorov-Sinai
invariant is found only over time scales that are logarithmic in the
quantization parameter.Comment: LaTeX, 21 pages, Presented at the 3rd Workshop on Quantum Chaos and
Localization Phenomena, Warsaw, Poland, May 25-27, 200
Accurate first principles detailed balance determination of Auger recombination and impact ionization rates in semiconductors
The technologically important problem of predicting Auger recombination
lifetimes in semiconductors is addressed by means of a fully first--principles
formalism. The calculations employ highly precise energy bands and wave
functions provided by the full--potential linearized augmented plane wave
(FLAPW) code based on the screened exchange local density approximation. The
minority carrier Auger lifetime is determined by two closely related
approaches: \emph{i}) a direct evaluation of the Auger rates within Fermi's
Golden Rule, and \emph{ii}) an indirect evaluation, based on a detailed balance
formulation combining Auger recombination and its inverse process, impact
ionization, in a unified framework. Calculated carrier lifetimes determined
with the direct and indirect methods show excellent consistency \emph{i})
between them for -doped GaAs and \emph{ii}%) with measured values for GaAs
and InGaAs. This demonstrates the validity and accuracy of the computational
formalism for the Auger lifetime and indicates a new sensitive tool for
possible use in materials performance optimization.Comment: Phys. Rev. Lett. accepte
How I manage medical complications of beta-thalassemia in adults
The complex pathophysiology in beta-thalassemia can translate to multiple morbidities that affect every organ system. Improved survival due to advances in management meant that patients are exposed to the harmful effects of ineffective erythropoiesis, anemia and iron overload for a longer duration, and we started seeing new or more frequent complications in adult compared with younger patients. In this article, we highlight particular aspects of managing adult patients with beta-thalassemia utilizing our own experience in treating such patients. We cover both transfusion-dependent and non-transfusion-dependent forms of the disease and tackle specific morbidities of highest interest
Coagulopathy in Beta-Thalassemia: Current Understanding and Future Perspectives
As the life expectancy of β-thalassemia patients has markedly improved over the last decade, several new complications are being recognized. The presence of a high incidence of thromboembolic events, mainly in thalassemia intermedia patients, has led to the identification of a hypercoagulable state in thalassemia. In this review, the molecular and cellular mechanisms leading to hypercoagulability in thalassemia are highlighted, and the current clinical experience is summarized. Recommendations for thrombosis prophylaxis are also discussed
Electronic and optical properties of families of polycyclic aromatic hydrocarbons: a systematic (time-dependent) density functional theory study
Homologous classes of Polycyclic Aromatic Hydrocarbons (PAHs) in their
crystalline state are among the most promising materials for organic
opto-electronics. Following previous works on oligoacenes we present a
systematic comparative study of the electronic, optical, and transport
properties of oligoacenes, phenacenes, circumacenes, and oligorylenes. Using
density functional theory (DFT) and time-dependent DFT we computed: (i)
electron affinities and first ionization energies; (ii) quasiparticle
correction to the highest occupied molecular orbital (HOMO)-lowest unoccupied
molecular orbital (LUMO) gap; (iii) molecular reorganization energies; (iv)
electronic absorption spectra of neutral and charged systems. The
excitonic effects are estimated by comparing the optical gap and the
quasiparticle corrected HOMO-LUMO energy gap. For each molecular property
computed, general trends as a function of molecular size and charge state are
discussed. Overall, we find that circumacenes have the best transport
properties, displaying a steeper decrease of the molecular reorganization
energy at increasing sizes, while oligorylenes are much more efficient in
absorbing low-energy photons in comparison to the other classes.Comment: 26 pages, 9 figures, 4 tables, accepted for pubblication in Chemical
Physics (14/04/2011
Update on the use of deferasirox in the management of iron overload
Regular blood transfusions as supportive care for patients with chronic anemia inevitably lead to iron overload as humans cannot actively remove excess iron. The cumulative effects of iron overload cause significant morbidity and mortality if not effectively treated with chelation therapy. Based on a comprehensive clinical development program, the once-daily, oral iron chelator deferasirox (Exjade®) is approved for the treatment of transfusional iron overload in adult and pediatric patients with various transfusion-dependent anemias, including β-thalassemia and the myelodysplastic syndromes. Deferasirox dose should be titrated for each individual patient based on transfusional iron intake, current iron burden and whether the goal is to decrease or maintain body iron levels. Doses of >30 mg/kg/day have been shown to be effective with a safety profile consistent with that observed at doses <30 mg/kg/day. Recent data have highlighted the ability of deferasirox to decrease cardiac iron levels and to prevent the accumulation of iron in the heart. The long-term efficacy and safety of deferasirox for up to 5 years of treatment have now been established. The availability of this effective and generally well tolerated oral therapy represents a significant advance in the management of transfusional iron overload
Thalassaemia Intermedia: an Update
Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with thalassaemia intermedia (TI) has substantially increased over the past decade. TI encompasses a wide clinical spectrum of beta-thalassaemia phenotypes. Some TI patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with TI are rarely seen in thalassaemia major, including extramedullary hematopoiesis, leg ulcers, gallstones, thrombosis and pulmonary hypertension. There are a number of options currently available for managing patients with TI, including transfusion therapy, iron chelation therapy, modulation of foetal haemoglobin production and haematopoietic stem cell transplantation. However, at present, there are no clear guidelines for an orchestrated optimal treatment plan
- …