Il Pooling-score (P-score): Variabilit\ue0 inter- e intra-individuale nella valutazione endoscopica della gravit\ue0 della disfagia

This study evaluated the intra- and inter-rater reliability of the Pooling score (P-score) in clinical endoscopic evaluation of severity of swal- lowing disorder, considering excess residue in the pharynx and larynx. The score (minimum 4 - maximum 11) is obtained by the sum of the scores given to the site of the bolus, the amount and ability to control residue/bolus pooling, the latter assessed on the basis of cough, raclage, number of dry voluntary or re ex swallowing acts ( 5). Four judges evaluated 30 short lms of pharyngeal transit of 10 solid (1/4 of a cracker), 11 creamy (1 tablespoon of jam) and 9 liquid (1 tablespoon of 5 cc of water coloured with methlyene blue, 1 ml in 100 ml) boluses in 23 subjects (10 M/13 F, age from 31 to 76 yrs, mean age 58.56\ub111.76 years) with different pathologies. The lms were randomly distributed on two CDs, which differed in terms of the sequence of the lms, and were given to judges (after an explanatory ses- sion) at time 0, 24 hours later (time 1) and after 7 days (time 2). The inter- and intra-rater reliability of the P-score was calculated using the intra-class correlation coef cient (ICC; 3,k). The possibility that consistency of boluses could affect the scoring of the lms was considered. The ICC for site, amount, management and the P-score total was found to be, respectively, 0.999, 0.997, 1.00 and 0.999. Clinical evaluation of a criterion of severity of a swallowing disorder remains a crucial point in the management of patients with pathologies that predispose to complications. The P-score, derived from static and dynamic parameters, yielded a very high correlation among the scores attributed by the four judges during observations carried out at different times. Bolus consistencies did not affect the outcome of the test: the analysis of variance, performed to verify if the scores attributed by the four judges to the parameters selected, might be in uenced by the different consistencies of the boluses, was not signi cant. These initial data validate the clinical use of the P-score in the management of patients with deglutition disorders by a multidisciplinary team

Improved production of succinic acid from Basfia succiniciproducens growing on A-donax and process evaluation through material flow analysis

BackgroundDue to its wide range of applications in the food, pharmaceutical and chemical fields, microbial synthesis of succinic acid is receiving growing attention, generating already relevant industrial results, as well as fueling constant research for improvements. In order to develop a sustainable process, a special focus is now set on the exploitation and conversion of lignocellulosic biomasses into platform chemicals.ResultsIn the present work we used Basfia succiniciproducens BPP7 in separated hydrolysis and fermentation experiments with Arundo donax as starting material. Fed-batch strategies showed a maximal production of about 37g/L of succinic acid after 43h of growth and a productivity of 0.9g/Lh on the pilot scale. Global mass balance calculations demonstrated a hydrolysis and fermentation efficiency of about 75%. Moreover, the application of a material flow analysis showed the obtainment of 88.5 and 52 % of succinic acid, per kg of virgin biomass and on the total generated output, respectively.ConclusionsThe use of fed-batch strategies for the growth of B. succiniciproducens on A. donax improved the titer and productivity of succinic acid on pre-pilot scale. Process evaluation through material flow analysis showed successful results and predicted a yield of succinic acid of about 30% in a fed-batch process that uses A. donax as only carbon source also in the feed. Preliminary considerations on the possibility to achieve an energetic valorization of the residual solid coming from the fermentation process were also carried out

Proteomic analysis of mucopolysaccharidosis IIIB mouse brain

Mucopolysaccharidosis IIIB (MPS IIIB) is an inherited metabolic disease due to deficiency of α-N-Acetylglucosaminidase (NAGLU) enzyme with subsequent storage of undegraded heparan sulfate (HS). The main clinical manifestations of the disease are profound intellectual disability and neurodegeneration. A label-free quantitative proteomic approach was applied to compare the proteome profile of brains from MPS IIIB and control mice to identify altered neuropathological pathways of MPS IIIB. Proteins were identified through a bottom up analysis and 130 were significantly under-represented and 74 over-represented in MPS IIIB mouse brains compared to wild type (WT). Multiple bioinformatic analyses allowed to identify three major clusters of the differentially abundant proteins: proteins involved in cytoskeletal regulation, synaptic vesicle trafficking, and energy metabolism. The proteome profile of NAGLU−/− mouse brain could pave the way for further studies aimed at identifying novel therapeutic targets for the MPS IIIB. Data are available via ProteomeXchange with the identifier PXD017363

Human wild-type and D76N β_{2}-microglobulin variants are significant proteotoxic and metabolic stressors for transgenic C. elegans

β2-microglobulin (β2-m) is a plasma protein derived from physiological shedding of the class I major histocompatibility complex (MHCI), causing human systemic amyloidosis either due to persistently high concentrations of the wild-type (WT) protein in hemodialyzed patients, or in presence of mutations, such as D76N β2-m, which favor protein deposition in the adulthood, despite normal plasma levels. Here we describe a new transgenic Caenorhabditis elegans (C. elegans) strain expressing human WT β2-m at high concentrations, mimicking the condition that underlies dialysis-related amyloidosis (DRA) and we compare it to a previously established strain expressing the highly amyloidogenic D76N β2-m at lower concentrations. Both strains exhibit behavioral defects, the severity of which correlates with β2-m levels rather than with the presence of mutations, being more pronounced in WT β2-m worms. β2-m expression also has a deep impact on the nematodes' proteomic and metabolic profiles. Most significantly affected processes include protein degradation and stress response, amino acids metabolism, and bioenergetics. Molecular alterations are more pronounced in worms expressing WT β2-m at high concentration compared to D76N β2-m worms. Altogether, these data show that β2-m is a proteotoxic protein in vivo also in its wild-type form, and that concentration plays a key role in modulating pathogenicity. Our transgenic nematodes recapitulate the distinctive features subtending DRA compared to hereditary β2-m amyloidosis (high levels of non-mutated β2-m vs. normal levels of variant β2-m) and provide important clues on the molecular bases of these human diseases

A multinational consensus on dysphagia in Parkinson's disease: screening, diagnosis and prognostic value

Background Parkinson's disease (PD) is a neurodegenerative disorder characterized by a combination of motor and non-motor dysfunction. Dysphagia is a common symptom in PD, though it is still too frequently underdiagnosed. Consensus is lacking on screening, diagnosis, and prognosis of dysphagia in PD. Objective To systematically review the literature and to define consensus statements on the screening and the diagnosis of dysphagia in PD, as well as on the impact of dysphagia on the prognosis and quality of life (QoL) of PD patients. Methods A multinational group of experts in the field of neurogenic dysphagia and/or PD conducted a systematic revision of the literature published since January 1990 to February 2021 and reported the results according to PRISMA guidelines. The output of the research was then analyzed and discussed in a consensus conference convened in Pavia, Italy, where the consensus statements were drafted. The final version of statements was subsequently achieved by e-mail consensus. Results Eighty-five papers were used to inform the Panel's statements even though most of them were of Class IV quality. The statements tackled four main areas: (1) screening of dysphagia: timing and tools; (2) diagnosis of dysphagia: clinical and instrumental detection, severity assessment; (3) dysphagia and QoL: impact and assessment; (4) prognostic value of dysphagia; impact on the outcome and role of associated conditions. Conclusions The statements elaborated by the Consensus Panel provide a framework to guide the neurologist in the timely detection and accurate diagnosis of dysphagia in PD

Happy Feet in a Hostile World? The Future of Penguins Depends on Proactive Management of Current and Expected Threats

This is the final version. Available on open access from Frontiers Media via the DOI in this recordPenguins face a wide range of threats. Most observed population changes have been negative and have happened over the last 60 years. Today, populations of 11 penguin species are decreasing. Here we present a review that synthesizes details of threats faced by the world's 18 species of penguins. We discuss alterations to their environment at both breeding sites on land and at sea where they forage. The major drivers of change appear to be climate, and food web alterations by marine fisheries. In addition, we also consider other critical and/or emerging threats, namely human disturbance near nesting sites, pollution due to oil, plastics and chemicals such as mercury and persistent organic compounds. Finally, we assess the importance of emerging pathogens and diseases on the health of penguins. We suggest that in the context of climate change, habitat degradation, introduced exotic species and resource competition with fisheries, successful conservation outcomes will require new and unprecedented levels of science and advocacy. Successful conservation stories of penguin species across their geographical range have occurred where there has been concerted effort across local, national and international boundaries to implement effective conservation planning.WWF-UKPEW FoundationNational Science Foundation (NSF