Search results for "STORAGE"
showing 10 items of 1239 documents
Skeletal alterations, developmental delay and new mutations in juvenile-onset Pompe disease.
2018
Abstract Pompe disease is an autosomal recessive disorder caused by a deficiency of acid α-glucosidase. In addition to the severe infantile form with cardiac involvement, late-onset variants can affect older children, adolescents (aged >1 year old) or adults. Patients with juvenile (a subgroup of late-onset type) Pompe disease typically do not have cardiac alterations e.g. hypertrophic cardiomyopathy, and the diagnosis is often difficult because it can clinically resemble myriad other neuromuscular disorders. A high level of clinical suspicion is necessary for a timely and accurate diagnosis. We describe 3 interesting cases of patients with juvenile-onset Pompe disease who presented some un…
Lysosomal acid lipase deficiency: Expanding differential diagnosis.
2016
The differential diagnoses for metabolic liver diseases may be challenging in clinical settings, which represents a critical issue for disorders such as lysosomal acid lipase deficiency (LAL-D). LAL-D is caused by deficient activity of the LAL enzyme, resulting in the accumulation of cholesteryl esters and triglycerides throughout the body, predominately in the liver, spleen, gastrointestinal tract, and blood vessel walls. LAL-D is a progressive, multi-organ disease with early mortality and significant morbidity characterized by a combination of hepatic dysfunction and dyslipidemia. Evidence suggests LAL-D may be substantially underdiagnosed or misdiagnosed, which is critical given that dis…
Aromatic and proteomic analyses corroborate the distinction between Mediterranean landraces and modern varieties of durum wheat
2016
AbstractIn this paper volatile organic compounds (VOCs) from durum wheat cultivars and landraces were analyzed using PTR-TOF-MS. The aim was to characterize the VOC’s profile of the wholemeal flour and of the kernel to find out if any VOCs were specific to varieties and sample matrices. The VOC data is accompanied by SDS-PAGE analyses of the storage proteins (gliadins and glutenins). Statistical analyses was carried out both on the signals obtained by MS and on the protein profiles. The difference between the VOC profile of two cultivars or two preparations of the same sample - matrices, in this case kernel vs wholemeal flour - can be very subtle; the high resolution of PTR-TOF-MS - down to…
Native Vineyard Non-Saccharomyces Yeasts Used for Biological Control of Botrytis cinerea in Stored Table Grape
2021
Postharvest spoilage fungi, such as Botrytis cinerea, are considered the main cause of losses of fresh fruit quality and vegetables during storage, distribution, and consumption. The current control strategy is the use of SO2 generator pads whose application is now largely under observation. A high quantity of SO2 can be deleterious for fresh fruits and vegetables and it is not allowed in organic agriculture. For this reason, great attention has been recently focused on identifying Biological Control Agents (BCA) to implement biological approaches devoid of chemicals. In this direction, we carried out our study in isolating five different non-Saccharomyces yeast strains from local vineyards…
Shelf life evaluation of fresh-cut red chicory subjected to different minimal processes
2018
Abstract Microbiological, chemical and physical parameters of minimally processed red chicory (Cichorium intybus L.) subjected to two different transformation processes were investigated. A classic ready-to-eat (RTE) process (P1) and a production without cutting (P2) were monitored during refrigerated (4 °C) storage (15 d). Total mesophilic microorganisms, total psychrotrophic microorganisms and pseudomonads were detected at the highest cell densities in all samples. Presumptive Pseudomonas population dominated the cultivable microbial community of RTE red chicory and were characterized genetically. Twenty-two randomly amplified polymorphic DNA (RAPD) types were investigated by 16S rRNA gen…
Harmonising and linking biomedical and clinical data across disparate data archives to enable integrative cross-biobank research
2015
A wealth of biospecimen samples are stored in modern globally distributed biobanks. Biomedical researchers worldwide need to be able to combine the available resources to improve the power of large-scale studies. A prerequisite for this effort is to be able to search and access phenotypic, clinical and other information about samples that are currently stored at biobanks in an integrated manner. However, privacy issues together with heterogeneous information systems and the lack of agreed-upon vocabularies have made specimen searching across multiple biobanks extremely challenging. We describe three case studies where we have linked samples and sample descriptions in order to facilitate glo…
Multi-criteria framework as an innovative tradeoff approach to determine the shelf-life of high pressure-treated poultry
2016
International audience; A multi-criteria framework combining safety, hygiene and sensorial quality was developed to investigate the possibility of extending the shelf-life and/or removing lactate by applying High Hydrostatic Pressure (HHP) in a ready-to-cook (RTC) poultry product. For this purpose, Salmonella and Listeria monocytogenes were considered as safety indicators and Escherichia coli as hygienic indicator. Predictive modeling was used to determine the influence of HHP and lactate concentration on microbial growth and survival of these indicators. To that end, probabilistic assessment exposure models developed in a previous study (Lerasle, M., Guillou, S., Simonin, H., Anthoine, V.,…
Prickly Pear By-Product in the Feeding of Livestock Ruminants: Preliminary Investigation
2020
Simple Summary The question of sustainability of animal nutrition has become a popular topic. The gains made by recycling safe, yet otherwise valueless, by-products and wastes from human food and fiber production (green feeds) may lessen the competition between humans and animals for crops and decrease the environmental impact of food production. In this context, prickly pear by-product, which results from Opuntia ficus-indica (L.) Mill. fruits processed for juice extraction, could be an optimum by-product to ruminant feeding. This study evaluates the nutritional characteristics and its stability during storage using potassium metabisulfite as a preservative agent. This preliminary investig…
Toward shrimp consumption without chemicals: Combined effects of freezing and modified atmosphere packaging (MAP) on some quality characteristics of …
2015
The combined effects of freezing and modified atmosphere packaging (MAP) (100% N2 and 50% N2 + 50% CO2) on some quality characteristics of Giant Red Shrimp (GRS) (Aristaeomorpha foliacea) was studied during 12-month storage. In particular, the quality characteristics determined proximal and gas compositions, melanosis scores, pH, total volatile basic-nitrogen (TVB-N), thiobarbituric acid (TBA) as well as free amino acid (FAA). In addition, the emergent data were compared to those subject to vacuum packaging as well as conventional preservative method of sulphite treatment (SUL). Most determined qualities exhibited quantitative differences with storage. By comparisons, while pH and TVB-N sta…
Automatic sleep scoring: A deep learning architecture for multi-modality time series
2020
Background: Sleep scoring is an essential but time-consuming process, and therefore automatic sleep scoring is crucial and urgent to help address the growing unmet needs for sleep research. This paper aims to develop a versatile deep-learning architecture to automate sleep scoring using raw polysomnography recordings. Method: The model adopts a linear function to address different numbers of inputs, thereby extending model applications. Two-dimensional convolution neural networks are used to learn features from multi-modality polysomnographic signals, a “squeeze and excitation” block to recalibrate channel-wise features, together with a long short-term memory module to exploit long-range co…