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The logopenic variant of primary progressive aphasia (lvPPA) is the most recent variant of primary progressive aphasia (PPA) to be identified; thus far, it has been poorly investigated. Despite being typically associated with Alzheimer disease (AD), lvPPA has recently been linked to frontotemporal lobe degeneration (FTLD), with distinctive cognitive and neural features that are worthy of further investigation. Here, we describe the neuropsychological and linguistic profile, as well as cerebral abnormalities, of an individual exhibiting PPA and carrying a pathogenetic variant in the GRNgene, from a 3-year longitudinal perspective. The individual’s initial profile resembled lvPPA because it was characterized by word-finding difficulties and phonological errors in spontaneous speech in addition to sentence repetition and phonological short-term memory impairments. The individual’s structural and metabolic imaging data demonstrated left temporal and bilateral frontal atrophy and hypometabolism, respectively. On follow-up, as the pathology progressed, dysprosody, stereotypical speech patterns, agrammatism, and orofacial apraxia appeared, suggesting an overlap with the nonfluent variant of PPA (nfvPPA). Severe sentence comprehension impairment also became evident. Our longitudinal and multidisciplinary diagnostic approach allowed us to better characterize the progression of a GRN-positive lvPPA profile, providing neuropsychological and imaging indicators that might be helpful to improve classification between different PPA variants and to address a nosological issue. Finally, we discuss the importance of early diagnosis of PPA given the possible overlap between different PPA variants during the progression of the pathology.

The article focuses on business landscape we find ourselves is incredibly dynamic, with significant levels of change and variability that can make it hard to understand where or how to focus. Topics include the technology has added even higher levels of uncertainty into this mix, and the low barriers to entry for most small businesses to experiment and discover opportunities that scale unexpectedly quickly.

Cardiac amyloidosis is a progressive infiltrative disease for which new treatments are now available. As therapy should be started as early as possible to avoid complications such as restrictive cardiomyopathy, arrhythmias and heart failure, a prompt and reliable diagnosis by means of non-invasive tests would be highly warranted. Electrocardiography, echocardiography and cardiac magnetic resonance imaging are all used in the evaluation of cardiac amyloidosis with varying diagnostic and prognostic accuracy, but none of these modalities can effectively differentiate the cardiac amyloid subtypes. We aim to highlight the most relevant findings in the literature of molecular imaging in the assessment of patients with cardiac amyloidosis and to underline future clinical perspective. We performed multiple searches using Pub-Med databases in order to find important original articles on the role of molecular imaging in the assessment of patients affected by CA. Several search terms were used, such as “cardiac amyloidosis”; “Light-chain amyloidosis”; “Transthyretin amyloid cardiomyopathy”; “bone scintigraphy”; “single photon emission tomography” or “SPECT”; “Positron emission tomography or PET”, and “cardiac imaging”. All radiopharmaceuticals tracing cardiac amyloidosis were also included. Several studies about the role of SPECT with bone-seeking tracer (47 articles) and innervation tracer (9 articles) in the work-up of CA, as well as new PET amyloid-binding (14 articles) and bone radiotracer (4 articles) have been reviewed and discussed. Molecular imaging represents a sensitive tool for early assessment of both amyloid burden and cardiac innervation, to differentiate between subtypes and to monitor disease burden, disease progression, and potential response to therapy.

Life-threatening ventricular arrhythmias (VA) are a major cause of death in patients with congestive heart failure (HF). Among various factors, the sympathetic nervous system may give rise to VA in several pathophysiological pathways due to an impaired function of presynaptic sympathetic nerve terminals. Positron emission tomography (PET) with labeled catecholamine analogues represents a reliable tool to assess the sympathetic innervation activity. This review aims at summarising the most relevant and recent literature findings on the current role of PET in the evaluation of cardiac sympathetic activity in patients with heart failure. A comprehensive literature search strategy using PubMed databases was carried out looking for articles on the role of Positron emission tomography/Computed Tomography (PET/CT) in the assessment of myocardial sympathetic innervation in patients with heart failure. The literature search limited to the last 5 years retrieved 40 papers. Most of the papers dealt with PET studies with 11C-HED. 19 pre-clinical, first-in-human and clinical studies highlighting the current role of PET and future perspectives resulted eligible for inclusion in the present review. The assessment of myocardial sympathetic activity in patients with heart failure with PET will play a pivotal role in clinical practice. Its capability to predict the occurrence of life-threatening VA and the effectiveness of resynchronization therapy makes this technique ideal in the era of personalized medicine.

The article discusses the differences between agile architecture and enterprise architecture (EA). Topics include the common pitfalls and bad practices when using agile and enterprise architectures, the advantages and disadvantages of both systems, and the factors that make EA inferior to Agile. Also explored are the challenges in introducing agile practices.

Introduction: Streptococcal infection can be responsible for multiple complications, such as toxic, septic or allergic disorders. Toxin-mediated complications (TMC) can appear during the acute phase of disease and can involve any organ, causing carditis, arthritis, nephritis, hepatitis etc.

Sepsis represents a severe pathology that requires both rapid and precise positive and differential diagnosis to identify patients who need immediate antimicrobial therapy. Monitoring septic patients′ outcome leads to prolonged hospitalisation and antibacterial therapy, often accompanied by substantial side effects, complications and a high mortality risk. Septic patients present with complex pathophysiological and immunological disorders and with a predominance of pro-inflammatory or anti-inflammatory mediators which are heterogeneous with respect to the infectious focus, the aetiology of sepsis or patients′ immune status or comorbidities. Previous studies performed have analysed inflammatory biomarkers, but a test or combinations of tests that can quickly and precisely establish a diagnosis or prognosis of septic patients has yet to be discovered. Recent research has focused on re-analysing older accessible parameters found in the complete blood count to determine the sensitivity, specificity, positive and negative predictive values for the diagnosis and prognosis of sepsis. The neutrophil/lymphocyte count ratio (NLCR), mean platelet volume (MPV) and red blood cells distribution width (RDW) are haemogram indicators which have been evaluated and which are of proven use in septic patients′ management.

We examine, from a theoretical viewpoint, how the physical parameters of H II regions are controlled in both normal galaxies and in starburst environments. These parameters are the H II region luminosity function, the time-dependent size, the covering fraction of molecular clouds, the pressure in the ionized gas, and the ionization parameter. The factors that control them are the initial mass function (IMF) of the exciting stars, the cluster mass function, the metallicity, and the mean pressure in the surrounding interstellar medium. We investigate the sensitivity of the Ha luminosity to the IMF, and find that this can translate to more than a factor 2 variation in derived star formation rates. The molecular cloud dissipation timescale is estimated from a case study of M17 to be ~1 Myr for this object. Based on H II luminosity function fitting for nearby galaxies, we suggest that the H II region cluster mass function is fitted by a lognormal form peaking at ~100 M. The cluster mass function continues the stellar IMF to a higher mass regime. The pressure in the H II regions is controlled by the mechanical luminosity flux from the central cluster. Since this is closely related to the ionizing photon flux, we show that the ionization parameter is not a free variable, and that the diffuse ionized medium may be composed of many large, faint, and old H II regions. Finally, we derive theoretical probability distributions for the ionization parameter as a function of metallicity and compare these to those derived for SDSS galaxies.

This paper presents both stellar mass and H II region diagnostics based on dusty, radiation-pressure-dominated photoionization models for compact and ultracompact H II regions, and compares these with observational constraints. These models successfully reproduce the observed relationship between the density and the thickness of the ionized layer. The absorption of ionizing photons in the dusty ionized plasma makes denser ionized regions thinner than simple photoionization models would predict, improving the fit with the observations. The models provide a good fit to observed diagnostic plots involving ratios of infrared emission lines, all accessible with the IRS instrument of the Spitzer Space Telescope. These give the effective temperature to an accuracy of about 2500 K and the mass of the ionizing star to a precision of about +-30%. The S IV/S III ratio is sensitive to foreground extinction as well as to stellar effective temperature or mass. From this ratio, we determine that the mean extinction to observed compact H II regions is typically AV ~ 30 mag. The electron temperature depends on the chemical abundances, the pressure, and the effective temperature of the exciting star. We use these models to rederive the slope of the Galactic abundance gradient, with the result that d log(O/H)/dRG = 0.06 +- 0.01 dex kpc-1, bringing the Galactic abundance gradient derived from compact H II regions into closer agreement with those based on other techniques. The shape of the far-IR SED of compact H II regions can be used to constrain the mean pressure or density in the H II region. The Spitzer MIPS instrument should be very helpful in this regard.

The presence of plastids in diverse eukaryotic lineages that have lost the capacity for photosynthesis is well documented. The metabolic functions of such organelles, however, are poorly understood except in the case of the apicoplast in the Apicomplexa, a group of intracellular parasites including Plasmodium falciparum, and the plastid of the green alga Helicosporidium sp., a parasite for which the only host-free stage identified in nature so far is represented by cysts. As a first step in the reconstruction of plastid functions in a nonphotosynthetic, predominantly free-living organism, we searched for expressed sequence tags (ESTs) that correspond to nucleus-encoded plastid-targeted polypeptides in the green alga Prototheca wickerhamii. From 3,856 ESTs, we found that 71 unique sequences (235 ESTs) correspond to different nucleus-encoded putatively plastid-targeted polypeptides. The identified proteins predict that carbohydrate, amino acid, lipid, tetrapyrrole, and isoprenoid metabolism as well as de novo purine biosynthesis and oxidoreductive processes take place in the plastid of P. wickerhamii. Mg-protoporphyrin accumulation and, therefore, plastid-to-nucleus signaling might also occur in this nonphotosynthetic organism, as we identified a transcript which encodes subunit I of Mg-chelatase, the enzyme which catalyzes the first committed step in chlorophyll synthesis. Our data indicate a far more complex metabolism in P. wickerhamii's plastid compared with the metabolic pathways predicted to be located in the apicoplast of P. falciparum and the plastid of Helicosporidium sp.

In this paper, we combine the stellar spectral synthesis code STARBURST99, the nebular modeling code MAPPINGS IIIq, a one-dimensional dynamical evolution model of H II regions around massive clusters of young stars, and a simplified model of synchrotron emissivity to produce purely theoretical self-consistent synthetic spectral energy distributions (SEDs) for (solar metallicity) starbursts lasting ~108 yr. These SEDs extend from the Lyman limit to beyond 21 cm. We find that two ISM parameters control the form of the SED: the pressure in the diffuse phase of the ISM (or, equivalently, its density), and the molecular cloud dissipation timescale. In particular, the shape of the far-infrared (dust re-emission) bump is strongly dependent on the mean pressure in the star-forming or starburst galaxy. This can explain the range of far-infrared (FIR) colors seen in starburst galaxies. In the case of objects of composite excitation, such diagrams potentially provide a means of estimating the fraction of the FIR emission that is contributed by an active nucleus. We present detailed SED fits to Arp 220 and NGC 6240, and we give the predicted colors for starburst galaxies derived from our models for the IRAS and the Spitzer Space Telescope MIPS and IRAC instruments. Our models reproduce the spread in observed colors of starburst galaxies. From both the SED fits and the color : color diagrams, we infer the presence of a population of compact and ultracompact H II regions around single OB stars or small OB clusters. Finally, we present absolute calibrations to convert observed fluxes into star formation rates in the UV (GALEX), at optical wavelengths (Ha), and in the IR (IRAS or Spitzer). We show that 25 mm fluxes are particularly valuable as star formation indicators, since they largely eliminate one of the parameters controlling the IR SED.

We have statistically analyzed the spatially integrated far-infrared (FIR) emissions of the complete volume- and luminosity-limited sample of late-type (later than S0) Virgo Cluster galaxies measured using the Infrared Space Observatory by Tuffs and coworkers in bands centered on 60, 100, and 170 mm. Thirty of 38 galaxies detected at all three wavelengths contain a cold dust emission component, present within all morphological types of late-type systems ranging from early giant spiral galaxies to blue compact dwarfs (BCDs) and which could not have been recognized by IRAS. We fitted the data with a superposition of two modified blackbody functions, physically identified with a localized warm dust emission component associated with H II regions (whose temperature was constrained to be 47 K), and a diffuse emission component of cold dust. The cold dust temperatures were found to be broadly distributed, with a median of 18 K, some 8-10 K lower than would have been predicted from IRAS. The derived total dust mass is correspondingly increased by factors of typically 6-13. A good linear correlation is found between the "warm FIR" luminosities and the Ha equivalent widths (EWs), supporting the assumptions of our constrained spectral energy distribution fit procedure. We also found a good nonlinear correlation between the "cold FIR" luminosities and the Ha EWs, consistent with the prediction of Popescu and coworkers that the FIR-submillimeter emission should mainly be due to diffuse nonionizing UV photons. Both the "warm" and the "cold" FIR luminosity components are nonlinearly correlated with the (predominantly nonthermal) radio luminosities. There is a tendency for the temperatures of the cold dust component to become colder and for the cold dust surface densities (normalized to optical area) to increase for later morphological types. A particularly significant result concerns the low dust temperatures (ranging down to less than 10 K) and large dust masses associated with the Im and BCD galaxies in our sample. We propose two scenarios to account for the FIR characteristics of these systems.

IntroductionBacterial dysentery represents an acute intestinal infection caused by an array of species of the Shigella genus, clinically characterised by the dysenteric syndrome – fever, abdominal pain, tenesmuses, bloody, mucoid diarrhoea and, which can sometimes complicate with systemic toxic manifestations.Material and methodWe present the case of a young child aged 1 year and 4 months admitted in the 9th Paediatric Department at The National Institute of Infectious Diseases „Prof. Dr. Matei Bals’ for fever, bloody, mucoid diarrhoea, vomiting, poor feeding, and one paroxysmal neurological manifestation associated with altered general status. Biological specimens were collected and immediate treatment was initiated – intravenous fluid therapy, antibiotic (Ceftriaxone), anticonvulsant and symptomatic treatment.ResultsWorkup revealed leukocytosis with neutrophilia, important inflammatory syndrome, associated hepatic cytolysis and acid-base and hydro-electrolytic imbalances and positive stool cultures for Shigella sonneiwere obtained after cultivation on specific media. The neurologic evaluation and EEG confirmed neurological involvement secondary to systemic shigellosis. Under treatment, the child’s general status improved, with subsequent normalisation of the stools and after seven days, the patient was cured and discharged in a normal general state, without any neurological sequelae and with negative stool cultures.ConclusionThe presented case is an example of a toxic systemic form of shigellosis, with favourable evolution and complete clinical recovery, without sequelae but who requires subsequent neurological monitoring. Even though rarely encountered, the systemic severe form of paediatric dysentery can sometimes cause severe complications, sequelae and can lead to death in the absence of adequate treatment.

Background and aimsAcute rotavirus infection constitutes a serious health issue both nationally and worldwide, accounting for numerous cases in children, especially under the age of 5. Presently, due to the polymorphic clinical presentation of acute rotavirus infection in children, many authors have described the concept of rotaviral disease.ObjectiveIn this study we propose to analyse cases of acute rotavirus infection in children with the aim of identifying extra-intestinal manifestations.Material and methodWe conducted a retrospective study on cases of acute rotavirus infection in children who were hospitalised in the Paediatric Department of the National Institute of Infectious Diseases ’Prof. Dr. Matei Bals’ during 2013–2016. In these cases we have analysed the following parameters: age, sex, and background. We have also analysed the clinical forms of the disease and the extra-intestinal manifestations of the rotavirus infection. The diagnosis was established through rapid antigen testing from stool samples and PCR from urine, nasopharyngeal swabs, and CSF.ResultsIn the aforementioned period, we have registered 1028 cases of acute rotavirus infection in children. No deaths were registered and all cases had a favourable evolution. Most affected were male patients, those who belonged to the 1–4 years age group and children from urban areas. 10.4% of all cases had extra-intestinal manifestations. Respiratory, hepatic, cutaneous and neurological manifestations were the most encountered. The most severe extra-intestinal complications were neurological, accompanied by sequelae (seizures, retard, hypotonia).ConclusionsAcute rotavirus infection is a frequent among preschool children and can take severe disease clinical forms with extra-intestinal manifestations. The neurological complications are the most severe complications of the paediatric rotavirus infection.