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Journal of Veterinary Internal Medicine 2002Lysosomal storage diseases are rare, inherited disorders caused by the deficiency of 1 or more enzymes within the lysosomes of cells or by the deficiency of an... (Review)
Review
Lysosomal storage diseases are rare, inherited disorders caused by the deficiency of 1 or more enzymes within the lysosomes of cells or by the deficiency of an activating protein or cofactor necessary for enzyme activity. The enzyme deficiency leads to a catabolic blockade and subsequent accumulation of storage material, and this in turn leads, albeit indirectly, to a wide array of clinical signs. Many features of storage diseases make them difficult to recognize and diagnose. In this review, we summarize the clinical features of these diseases and outline the steps required to confirm a diagnosis.
Topics: Animals; Biopsy; Cat Diseases; Cats; Dog Diseases; Dogs; Lysosomal Storage Diseases; Lysosomes
PubMed: 11899921
DOI: 10.1892/0891-6640(2002)016<0133:radols>2.3.co;2 -
Annals of Hematology Apr 2024Transfusion reactions induced by platelet transfusions may be reduced and alleviated by leukocyte reduction of platelets. Although leukoreduction of apheresis platelets... (Review)
Review
Transfusion reactions induced by platelet transfusions may be reduced and alleviated by leukocyte reduction of platelets. Although leukoreduction of apheresis platelets can be performed either pre-storage or post-storage, seldom studies directly compare the incidence of transfusion reaction in these two different blood products. We conducted a retrospective study to compare the transfusion reactions between pre-storage and post-storage leukoreduced apheresis platelets. We reviewed the general characteristics and the transfusion reactions, symptoms, and categories for inpatients who received pre-storage or post-storage leukoreduced apheresis platelets. Propensity-score matching was performed to adjust for baseline differences between groups. A total of 40,837 leukoreduction apheresis platelet orders were reviewed. 116 (0.53%) transfusion reactions were reported in 21,884 transfusions with pre-storage leukoreduction, and 174 (0.91%) reactions were reported in 18,953 transfusions with post-storage leukoreduction. Before propensity-score matching, the odds ratio for transfusion reactions in the pre-storage group relative to the post-storage group was 0.57 (95% confidence interval [CI] 0.45-0.72, P < 0.01); the odds ratio after matching was 0.63 (95% CI 0.49-0.80, P < 0.01). A two-proportion z-test revealed pre-storage leukoreduction significantly decreases the symptoms of chills, fever, itching, urticaria, dyspnea, and hypertension as compared with those in post-storage leukoreduction. Pre-storage leukoreduced apheresis platelet significantly decreased febrile non-hemolytic transfusion reaction as compared with post-storage groups. This study suggests pre-storage leukoreduction apheresis platelet significantly decreases the transfusion reaction as compared with those in post-storage leukoreduction.
Topics: Humans; Retrospective Studies; Propensity Score; Blood Platelets; Blood Component Removal; Platelet Transfusion; Transfusion Reaction
PubMed: 38393657
DOI: 10.1007/s00277-024-05652-9 -
IScience Mar 2023DNA Encoding, as a key step in DNA storage, plays an important role in reading and writing accuracy and the storage error rate. However, currently, the encoding...
DNA Encoding, as a key step in DNA storage, plays an important role in reading and writing accuracy and the storage error rate. However, currently, the encoding efficiency is not high enough and the encoding speed is not fast enough, which limits the performance of DNA storage systems. In this work, a DNA storage encoding system with a graph convolutional network and self-attention (GCNSA) is proposed. The experimental results show that DNA storage code constructed by GCNSA increases by 14.4% on average under the basic constraints, and by 5%-40% under other constraints. The increase of DNA storage codes effectively improves the storage density of 0.7-2.2% in the DNA storage system. The GCNSA predicted more DNA storage codes in less time while ensuring the quality of codes, which lays a foundation for higher read and write efficiency in DNA storage.
PubMed: 36876131
DOI: 10.1016/j.isci.2023.106231 -
Blood Research Dec 2021Maintaining the quality of platelet products and increasing their storage time are priorities for treatment applications. The formation of platelet storage lesions that... (Review)
Review
Maintaining the quality of platelet products and increasing their storage time are priorities for treatment applications. The formation of platelet storage lesions that limit the storage period and preservation temperature, which can prepare a decent environment for bacterial growth, are the most important challenges that researchers are dealing with in platelet preservation. Nanotechnology is an emerging field of science that has introduced novel solutions to resolve these problems. Here, we reviewed the reported effects of polymeric nanoparticles-including chitosan, dendrimers, polyethylene glycol (PEG), and liposome-on platelets in articles from 2010 to 2020. As a result, we concluded that the presence of dendrimer nanoparticles with a smaller size, negative charge, low molecular weight, and low concentration along with PEGylation can increase the stability and survival of platelets during storage. In addition, PEGylation of platelets can also be a promising approach to improve the quality of platelet bags during storage.
PubMed: 34880140
DOI: 10.5045/br.2021.2021094 -
Neurotherapeutics : the Journal of the... Oct 2008Adult patients with metabolic myopathies typically present with exercise-induced pain, cramps, fatigue, and myoglobinuria. The current therapeutic options of glycogen... (Review)
Review
Adult patients with metabolic myopathies typically present with exercise-induced pain, cramps, fatigue, and myoglobinuria. The current therapeutic options of glycogen and lipid storage myopathies include dietary treatments, excersise training, and pharmacological supplementations. Herein is a review of evidence from randomized controlled trials in McArdle disease (glycogen storage disease type V, muscle phosphorylase deficiency) and carnitine palmitoyltransferase (CPT) 2 deficiency. A brief overview on current treatment options in rhabdomyolysis is also included because patients with McArdle disease and CPT 2 often experience such potentially life-threatening complications.
Topics: Animals; Carnitine O-Palmitoyltransferase; Creatine; Exercise Therapy; Glycogen Storage Disease; Glycogen Storage Disease Type V; Humans; Lipid Metabolism; Metabolic Diseases; Muscular Diseases; Rhabdomyolysis
PubMed: 19019309
DOI: 10.1016/j.nurt.2008.08.006 -
Heliyon Jul 2020The hydrogen economy is the key solution to secure a long-term energy future. Hydrogen production, storage, transportation, and its usage completes the unit of an... (Review)
Review
The hydrogen economy is the key solution to secure a long-term energy future. Hydrogen production, storage, transportation, and its usage completes the unit of an economic system. These areas have been the topics of discussion for the past few decades. However, its storage methods have conflicted for on-board hydrogen applications. In this review, the promising systems based on solid-state hydrogen storage are discussed. It works generally on the principles of chemisorption and physisorption. The usage of hydrogen packing material in the system enhances volumetric and gravimetric densities of the system and helps in improving ambient conditions and system kinetics. Numerous aspects like pore size, surface area ligand functionalization and pore volume of the materials are intensively discussed. This review also examines the newly developed research based on MOF (Metal-Organic Frameworks). These hybrid clusters are employed for nano-confinement of hydrogen at elevated temperatures. A combination of the various methodologies may give another course to a wide scope in the area of energy storage materials later in the future.
PubMed: 32743097
DOI: 10.1016/j.heliyon.2020.e04487 -
Materials (Basel, Switzerland) Nov 2021Dielectric capacitors have been widely studied because their electrostatic storage capacity is enormous, and they can deliver the stored energy in a very short time.... (Review)
Review
Dielectric capacitors have been widely studied because their electrostatic storage capacity is enormous, and they can deliver the stored energy in a very short time. Relaxor ferroelectrics-based dielectric capacitors have gained tremendous importance for the efficient storage of electrical energy. Relaxor ferroelectrics possess low dielectric loss, low remanent polarization, high saturation polarization, and high breakdown strength, which are the main parameters for energy storage. This article focuses on a timely review of the energy storage performance of BiFeO-based relaxor ferroelectrics in bulk ceramics, multilayers, and thin film forms. The article begins with a general introduction to various energy storage systems and the need for dielectric capacitors as energy storage devices. This is followed by a brief discussion on the mechanism of energy storage in capacitors, ferroelectrics, anti-ferroelectrics, and relaxor ferroelectrics as potential candidates for energy storage. The remainder of this article is devoted to reviewing the energy storage performance of bulk ceramics, multilayers, and thin films of BiFeO-based relaxor ferroelectrics, along with a discussion of strategies to address some of the issues associated with their application as energy storage systems.
PubMed: 34885340
DOI: 10.3390/ma14237188 -
Optics Express Nov 2021We theoretically and numerically study the efficiency of Brillouin-based opto-acoustic data storage in a photonic waveguide in the presence of thermal noise and laser...
We theoretically and numerically study the efficiency of Brillouin-based opto-acoustic data storage in a photonic waveguide in the presence of thermal noise and laser phase noise. We compare the physics of the noise processes and how they affect different storage techniques, examining both amplitude and phase storage schemes. We investigate the effects of storage time and pulse properties on the quality of the retrieved signal and find that phase storage is less sensitive to thermal noise than amplitude storage.
PubMed: 34809312
DOI: 10.1364/OE.439926 -
Materials (Basel, Switzerland) Dec 2021Thermal energy storage (TES) is an important means for the conservation and efficient utilization of excessive and renewable energy. With a much higher thermal storage... (Review)
Review
Thermal energy storage (TES) is an important means for the conservation and efficient utilization of excessive and renewable energy. With a much higher thermal storage capacity, latent heat storage (LHS) may be more efficient than sensible heat storage. Phase change materials (PCMs) are the essential storage media for LHS. PCM emulsions have been developed for LHS in flow systems, which act as both heat transfer and thermal storage media with enhanced heat transfer, low pumping power, and high thermal storage capacity. However, two major barriers to the application of PCM emulsions are their instability and high degree of supercooling. To overcome these, various strategies have been attempted, such as the reduction of emulsion droplet size, addition of nucleating agents, and optimization of the formulation. To the best of our knowledge, however, there is still a lack of review articles on fabrication methods for PCM emulsions or their latest applications. This review was to provide an up-to-date and comprehensive summary on the effective strategies and the underlying mechanisms for the preparation of stable PCM emulsions and reduction of supercooling, especially with the organic PCMs of paraffin. It was also to share our insightful perspectives on further development and potential applications of PCM emulsions for efficient energy storage.
PubMed: 35009265
DOI: 10.3390/ma15010121 -
Environmental Research Sep 2022To prevent the COVID-19 transmission, personal protective equipment (PPE) and packaging materials have been extensively used but often managed inappropriately,... (Review)
Review
To prevent the COVID-19 transmission, personal protective equipment (PPE) and packaging materials have been extensively used but often managed inappropriately, generating huge amount of plastic waste. In this review, we comprehensively discussed the plastic products utilized and the types and amounts of plastic waste generated since the outbreak of COVID-19, and reviewed the potential treatments for these plastic wastes. Upcycling of plastic waste into biochar was addressed from the perspectives of both environmental protection and practical applications, which can be verified as promising materials for environmental protections and energy storages. Moreover, novel upcycling of plastic waste into biochar is beneficial to mitigate the ubiquitous plastic pollution, avoiding harmful impacts on human and ecosystem through direct and indirect micro-/nano-plastic transmission routes, and achieving the sustainable plastic waste management for value-added products, simultaneously. This suggests that the plastic waste could be treated as a valuable resource in an advanced and green manner.
Topics: COVID-19; Charcoal; Ecosystem; Humans; Pandemics; Plastics
PubMed: 35660402
DOI: 10.1016/j.envres.2022.113495