The diagnostic challenge of very early-onset enterocolitis in an infant with XIAP deficiency

Background: Aggressive course and resistance to treatments usually characterize very early onset inflammatory bowel disease (VEO-IBD). Some VEO-IBD cases are due to monogenic immune defects and can benefit from hematopoietic stem cell transplantation (HSCT). Case presentation: We describe a Caucasian male baby who presented in the first months of life macrophage activation syndrome, followed by intractable colitis, recurrent episodes of fever and mild splenomegaly. After several immunological, genetic and clinical investigations, subsequently a therapeutic attempt with colectomy, analysis of VEO-IBD-associated genes, revealed a causative mutation in XIAP. The genetic diagnosis of a primary immune deficiency allowed curing the boy with hematopoietic stem cell transplantation. Conclusion: Our report, together with novel findings from recent literature, should contribute to increase awareness of monogenic immune defects as a cause of VEO-IBD. Comprehensive genetic analysis can allow a prompt diagnosis, resulting in the choice of effective treatments and sparing useless and damaging procedures

Akzeptanz von Futterpflanzen mit Vorkommen von kondensierten Tanninen bei Schafen und ihre Gehalte an nutzbarem Rohprotein

Plants with moderate contents of condensed tannins are assumed to have anthelmintic Plants with moderate contents of condensed tannins are assumed to have anthelmintic properties and to improve protein supply of ruminants by reducing its ruminal degradability. In the present study, birdsfoot trefoil (Lotus corniculatus), chicory (Cychorium intybus) and sainfoin (Onobrychis viciifolia), suitable to be grown in Central Europe, were tested for their suitability as feed for ruminants. Sainfoin proved to be the most promising forage plant since it was very well accepted by the sheep and presented the highest content of utilizable crude protein as determined in vitro. Only few condensed tannins were detected in chicory. Further in vivo studies are required to confirm a better metabolic protein supply of ruminants when fed sainfoin

Four-Year Environmental Surveillance Program of Legionella spp. in One of Palermo’s Largest Hospitals

Legionella is a ubiquitous bacterium that lives in freshwater environments and colonizes human-made water systems. Legionella pneumophila is the most virulent species, and risk factors for Legionnaires’ disease include increasing age, smoking, chronic diseases, and immunodeficiency. For this reason, it is very important to assess and monitor hospital water systems in order to prevent legionellosis. We have monitored a large hospital in Palermo for four years. To determine the presence of microorganisms, according to national guidelines, we used the culture method, which is considered the gold standard for Legionella detection. Sampling was divided into five macro-areas, and a total of 251 samples were collected during the period of investigation, 49% of which were Legionella spp.- positive and 51% were Legionella spp.-negative. Positive samples with L. pneumophila. sgr 2-15 were most frequent in the Underground (55.6%, p = 0.0184), Medicine (42.9%, p = 0.0184) and Other (63.2%, p = 0.002) areas; while positive samples for L. pneumophila sgr 1 were less frequent in the Underground (0.0%, p = 0.0184) and Surgery areas (4.5%, p = 0.033), and for Legionella anisa, were less frequent in the Medicine (4.1%, p = 0.021), Oncohematology (0.0%, p = 0.0282), and Other (0.0%, p = 0.016) areas. Finally, no significant differences were observed among the areas for each isolate considered. The surveillance carried out in these years demonstrates the importance of monitoring, which allows us to analyze the conditions of hospital facilities and, therefore, prevent Legionella spp. infections

A Case Report of a Solitary Fibrous Tumor of the Maxillary Sinus

A solitary fibrous tumor (SFT) is a benign neoplasm, firstly described as a mesenchymal tumor of the pleura. Its incidence range in the head and neck region is about 5\u201327%, but only rarely does it affect paranasal sinuses. The differential diagnosis is challenging, owing to its erosive growth pattern and immuno-histochemical features. SFTs have an aggressive behavior and an important recurrence potential. Therefore, a radical surgical excision is the gold standard therapeutic procedure. A rare SFT originating from the right maxillary sinus is reported here. The 37-year-old patient presented to the outpatient clinic with a painful expansive lesion in the whole right maxillary region. The overlying skin was inflamed and the patient had no epistaxis episodes. The 1.5 dentary element tested negative for vitality; however, a puncture of the lesion led to a hematic spill and no purulent discharge. An endoscopic-guided biopsy was suggestive either of SFT or hemangioperictoma, excluding a malignant neoplasm. A multi-equipe surgical team was activated. The lesion was embolized in order to achieve a good hemostatic control and, after 48 h, the neoplasm was radically excised with a combined open and endoscopic approach. The patient was disease-free at 12-month radiological and clinical follow-up. Given the rarity of this lesion and the delicacy required in addressing head and neck neoplasms, we believe that the present case report might be of help in further understanding how to approach cranio-facial SFTs

Biomolecular electronic devices based on self-organized deoxyguanosine nanocrystals.

We report on a new class of hybrid electronic devices based on a DNA nucleoside (deoxyguanosine lipophilic derivative) whose assembled polymeric ribbons interconnect a submicron metallic gate. The device exhibits large conductivity at room temperature, rectifying behavior and strong current-voltage hysteresis. The transport mechanism through the molecules is investigated by comparing films with different self-assembling morphology. We found that the main transport mechanism is connected to pi-pi interactions between guanosine molecules and to the formation of a strong dipole along ribbons, consistently with the results of our first-principles calculations

Hsp27 (HspB1) and αB-crystallin (HspB5) as therapeutic targets

AbstractHsp27 and αB-crystallin are molecular chaperones that are constitutively expressed in several mammalian cells, particularly in pathological conditions. These proteins share functions as diverse as protection against toxicity mediated by aberrantly folded proteins or oxidative-inflammation conditions. In addition, these proteins share anti-apoptotic properties and are tumorigenic when expressed in cancer cells. This review summarizes the current knowledge about Hsp27 and αB-crystallin and the implications, either positive or deleterious, of these proteins in pathologies such as neurodegenerative diseases, myopathies, asthma, cataracts and cancers. Approaches towards therapeutic strategies aimed at modulating the expression and/or the activities of Hsp27 and αB-crystallin are presented

Photoluminescence Emission Induced by Localized States in Halide Passivated Colloidal Two-Dimensional WS2 Nanoflakes

Engineering physicochemical properties of two-dimensional transition metal dichalcogenide (2D-TMD) materials by surface manipulation is essential for their practical and large-scale application especially for colloidal 2D-TMDs that are plagued by the unintentional formation of structural defects during the synthetic procedure. However, the available methods to manage surface states of 2D-TMDs in solution-phase are still limited hampering the production of high quality colloidal 2D-TMD inks to be straightforwardly assembled into actual devices. Here, we demonstrate an efficient solution-phase strategy to passivate surface defect states of colloidally synthetized WS2 nanoflakes with halide ligands, resulting in the activation of the photoluminescence emission. Photophysical investigation and density functional theory calculations suggest that halide atoms enable the suppression of non-radiative recombination through the elimination deep gap trap states, and introduce localized states in the energy band structure from which excitons efficiently recombine. Halide passivated WS2 nanoflakes importantly preserve colloidal stability and photoluminescence emission after several weeks of storing in ambient atmosphere, corroborating the potential of our developed 2D-TMD inks

Zooplankton and micronekton respond to climate fluctuations in the Amundsen Sea polynya, Antarctica

The vertical migration of zooplankton and micronekton (hereafter 'zooplankton') has ramifications throughout the food web. Here, we present the first evidence that climate fluctuations affect the vertical migration of zooplankton in the Southern Ocean, based on multi-year acoustic backscatter data from one of the deep troughs in the Amundsen Sea, Antarctica. High net primary productivity (NPP) and the annual variation in seasonal ice cover make the Amundsen Sea coastal polynya an ideal site in which to examine how zooplankton behavior responds to climate fluctuations. Our observations show that the timing of the seasonal vertical migration and abundance of zooplankton in the seasonally varying sea ice is correlated with the Southern Annular Mode (SAM) and El Nino Southern Oscillation (ENSO). Zooplankton in this region migrate seasonally and overwinter at depth, returning to the surface in spring. During +SAM/La Nina periods, the at-depth overwintering period is shorter compared to -SAM/El Nino periods, and return to the surface layers starts earlier in the year. These differences may result from the higher sea ice cover and decreased NPP during +SAM/La Nina periods. This observation points to a new link between global climate fluctuations and the polar marine food web

The MASSIMO system for the safeguarding of historic buildings in a seismic area: operationally-oriented platforms

In this paper, the non-invasive system MASSIMO is presented for the monitoring and the seismic vulnerability mitigation of the cultural heritage. It integrates ground-based, airborne and space-borne remote sensing tools with geophysical and in situ surveys to provide the multi-spatial (regional, urban and building scales) and multi-temporal (long-term, short-term, near-real-time and real-time scales) monitoring of test areas and buildings. The measurements are integrated through web-based GIS and 3D visual platforms to support decision-making stakeholders involved in urban planning and structural requalification. An application of this system is presented over the Calabria region for the town of Cosenza and a test historical complex