Gaucher Disease and Myelofibrosis: A Combined Disease or a Misdiagnosis?

Background: Gaucher disease (GD) and primary myelofibrosis (PMF) share similar clinical and laboratory features, such as cytopenia, hepatosplenomegaly, and marrow fibrosis, often resulting in a misdiagnosis. Case Report: We report here the case of a young woman with hepatosplenomegaly, leukopenia, and thrombocytopenia. Based on bone marrow (BM) findings and on liver biopsy showing extramedullary hematopoiesis, an initial diagnosis of PMF was formulated. The patient refused stem cell transplantation from an HLA-identical sibling. Low-dose melphalan was given, without any improvement. Two years later, a BM evaluation showed Gaucher cells. Low glucocerebrosidase and high chitotriosidase levels were indicative for GD. Molecular analysis revealed N370S/complex I mutations. Enzyme replacement therapy with imiglucerase was commenced, resulting in clinical and hematological improvements. Due to an unexpected and persistent organomegaly, PMF combined with GD were suspected. JAK2V617F, JAK2 exon 12, MPL, calreticulin, and exon 9 mutations were negative, and BM examination showed no marrow fibrosis. PMF was excluded. Twenty years after starting treatment, the peripheral cell count and liver size were normal, whereas splenomegaly persisted. Conclusion: In order to avoid a misdiagnosis, a diagnostic algorithm for patients with hepatosplenomegaly combined with cytopenia is suggested

Parametric resonance and spin-charge separation in 1D fermionic systems

We show that the periodic modulation of the Hamiltonian parameters for 1D correlated fermionic systems can be used to parametrically amplify their bosonic collective modes. Treating the problem within the Luttinger liquid picture, we show how charge and spin density waves with different momenta are simultaneously amplified. We discuss the implementation of our predictions for cold atoms in 1D modulated optical lattices, showing that the fermionic momentum distribution directly provides a clear signature of spin-charge separation.Comment: 6 pages, 3 figures, published versio

Macroscopic fluctuations theory of aerogel dynamics

We consider the thermodynamic potential describing the macroscopic fluctuation of the current and local energy of a general class of Hamiltonian models including aerogels. We argue that this potential is neither analytic nor strictly convex, a property that should be expected in general but missing from models studied in the literature. This opens the possibility of describing in terms of a thermodynamic potential non-equilibrium phase transitions in a concrete physical context. This special behaviour of the thermodynamic potential is caused by the fact that the energy current is carried by particles which may have arbitrary low speed with sufficiently large probability.Comment: final versio

The effect of individual, group, and shared organizational identification on job satisfaction and collective actual turnover

Drawing on the Social Identity Approach principles, we explored the relationship between organizational identification (individual, group, and shared), job satisfaction, and collective actual turnover. We hypothesize that (a) shared identification moderates the within-person relationship between individual organizational identification and job satisfaction, namely, the effect is stronger for groups in which the level of shared organizational identification is higher; (b) group job satisfaction mediates the relationship between group organizational identification and collective actual turnover. This study was conducted in a large Italian firm (N = 1090; sale locations = 91). Data were collected using both surveys (e.g., job satisfaction) and archive data (collective actual turnover). By means of Bayesian Multilevel Structural Equation Models, we supported the moderating role played by shared organizational identification in the relationship between individual organizational identification and job satisfaction, while no evidence was found for the mediational hypothesis. We discuss the theoretical and practical implications for management

Analysis of agroclimatic resources for Georgian viticulture

One of the results of the \u201cResearch Project for the Study of Georgian Grapes and Wine Culture\u201d promoted by the National Wine Agency of the Republic of eorgia was the production of a bilingual handbook for modern viticulture. The first sections of the handbook were devoted to the agrometeorological analysis of environmental resources and limitations, comprising a general analysis of Georgian climate and agrometeorological features, followed by detailed regional cards. The agrometeorological analysis of Georgia was based on daily data collected by National and International networks for the period 1974-2013. Several agrometeorological indexes were calculated in order to define resources and limitations for viticulture for each viticultural region of Georgia, providing fundamental information for grape-growing and wine-making

Parametric amplification of magnetoplasmons in semiconductor quantum dots

We show that the magnetoplasmon collective modes in quasi-two-dimensional semiconductor quantum dots can be parametrically amplified by periodically modulating the magnetic field perpendicular to the nanostructure. The two magnetoplasmon modes are excited and amplified simultaneously, leading to an exponential growth of the number of bosonic excitations in the system. We further demonstrate that damping mechanisms as well as anharmonicities in the confinement of the quantum dot lead to a saturation of the parametric amplification. This work constitutes a first step towards parametric amplification of collective modes in many-body fermionic systems beyond one dimension.Comment: 12 pages, 5 figures; published versio

Monitoring and ming bio-physical parameters for hypoxia hazard in a coastal sand pit

Management of coastal areas requires monitoring and modeling of the anthropogenic drivers and the bio-physical processes affecting water quality. To assess the range of hydrographic conditions controlling oxygen distribution in the bottom layers of sand pits, a multi-year oceanographic survey has been conducted in a coastal area with several extraction pits. Hydrographic data including profiles of temperature, salinity and oxygen were collected and related to local wind conditions and circulation. Moreover, 1D and 3D high-resolution non-hydrostatic ocean models were used to describe turbulent mixing regimes and to obtain the range of wind speeds for which the critical anoxic conditions may occur. It is shown that wind speed appears to control the dynamics of oxygen concentrations, with oxygen depleted zones developing in a short time in low wind speed conditions. Moreover, the depth and the shape of the extraction pit contribute to decrease the mixing of the bottom layers and increase the water retention in the hole increasing the output and the persistence of oxygen depleted zones in the excavated area. The results of the numerical simulations show that the risk of hypoxia at the bottom of the sand pits is associated with higher temperatures and wind speed lower than 5 m/s, which is not infrequent during the summer season. However, the number of consecutive days of oxygen depletion can be considered lower than the danger threshold level assumed in the literature