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Journal of Environmental Management May 2024This study has carried out a systematic review of 36 scientific papers (reporting 63 case studies) published in the last 15 years about the treatment of industrial,... (Meta-Analysis)
Meta-Analysis
This study has carried out a systematic review of 36 scientific papers (reporting 63 case studies) published in the last 15 years about the treatment of industrial, agri-food and municipal wastewater in lagoons. A concentration of studies from a few countries (Italy, Algeria and Iran) and about municipal wastewater (70% of papers) was revealed by the bibliographic analysis. Aeration was supplied in more than 50% of case studies; the storage capacity of lagoons (adopted as a measure of size) was extremely variable (over seven orders of magnitude), while their depth was generally lower than a few metres. The efficiency of lagoon treatments at removing COD was in a wide range (25-98%). Very few studies analysed the energy intensity of treatments in lagoons. The meta-analysis applied to a further selection of 10 papers with 29 case studies revealed significant differences in pH and dissolved oxygen concentration, due to aeration or type of treated wastewater. Treatment efficiency was higher in aerated lagoons compared to non-aerated systems, and did not depend on the type of treated wastewater. Based on the analysis of the reviewed papers, an urgent research need on this topic arises, mainly due to the oldness of most analysed studies. Practical suggestions are given to optimise the depuration performances of lagoons: (i) application of intermittent and night aeration; (ii) reduced air flow rates; (iii) adaptation of microbial biomass to high contents of inhibiting compounds in wastewater; (iv) construction of baffles to keep the planned hydraulic retention time avoiding short-circuit; (v) integration of lagoons with other treatments (e.g., constructed wetlands); (vi) ferti-irrigation of crops with lagoon effluents rather than disposal into water bodies.
Topics: Waste Disposal, Fluid; Wastewater; Water Purification
PubMed: 38701584
DOI: 10.1016/j.jenvman.2024.120974 -
JHEP Reports : Innovation in Hepatology Jun 2024Hepatocellular adenomas (HCAs) are rare benign liver tumours. Predisposing factors and complication rates appear to differ among children and adults. In the present... (Review)
Review
Hepatocellular adenomas (HCAs) are rare benign liver tumours. Predisposing factors and complication rates appear to differ among children and adults. In the present study, we aimed to systematically characterise paediatric HCAs and determine their course, complications, and management. Medical history, clinical symptoms, imaging, histopathology, and genetics of children with HCAs were collected through a systematic and comprehensive review of the published literature. A total of 316 children with HCAs were included in the present study. HCAs were diagnosed primarily in girls (59.3%) and at a mean age of 11.5 (range 0-17.7) years. The majority (83.6%) of HCAs occurred in children with predisposing diseases, of which glycogen storage disease was the most common, followed by portosystemic shunts and MODY3 (maturity-onset diabetes of the young type 3). Each of these diseases leads to a well-defined HCA molecular pattern. A significant number of HCAs either bled (24.7%) or transformed (14.8%) over time. HCA transformation was significantly more frequent in children with portosystemic shunts and in β-catenin-mutated HCAs, while haemorrhages were more frequent in children exposed to hormones and those with larger lesions. Management was primarily guided by any predisposing conditions and the number of lesions. Therefore, vascular shunts were closed when possible, while complicated lesions were resected. Liver transplantation has made it possible to treat adenomatosis, as well as any underlying diseases. Progress in understanding genetic and/or malformative contributions, which appear to be significant in paediatric HCAs, have provided insights into tumour pathogenesis and will further guide patient surveillance and management.
PubMed: 38699071
DOI: 10.1016/j.jhepr.2024.101078 -
Molecular Genetics and Metabolism... Jun 2024Pompe disease is a rare genetic disorder characterized by a deficiency of acid α-glucosidase (GAA), leading to the accumulation of glycogen in various tissues,...
Pompe disease is a rare genetic disorder characterized by a deficiency of acid α-glucosidase (GAA), leading to the accumulation of glycogen in various tissues, especially in skeletal muscles. The disease manifests as a large spectrum of phenotypes from infantile-onset Pompe disease (IOPD) to late-onset Pompe disease (LOPD), depending on the age of symptoms onset. Quantifying GAA activity and glycogen content in skeletal muscle provides important information about the disease severity. However, the distribution of GAA and glycogen levels in skeletal muscles from healthy individuals and those impacted by Pompe disease remains poorly understood, and there is currently no universally accepted standard assay for GAA activity measurement. This systematic literature review aims to provide an overview of the available information on GAA activity and glycogen content levels in skeletal muscle biopsies from patients with Pompe disease. A structured review of PubMed and Google Scholar literature (with the latter used to check that no additional publications were identified) was conducted to identify peer-reviewed publications on glycogen storage disease type II [MeSH term] + GAA, protein human (supplementary concept), Pompe, muscle; and muscle, acid alpha-glucosidase. A limit of English language was applied. Results were grouped by methodologies used to quantify GAA activity and glycogen content in skeletal muscle. The search and selection strategy were devised and carried out in line with Preferred Reporting of Items in Systematic Reviews and Meta-Analysis guidelines and documented using a flowchart. Bibliographies of papers included in the analysis were reviewed and applicable publications not already identified in the search were included. Of the 158 articles retrieved, 24 (comprising >100 muscle biopsies from >100 patients) were included in the analysis, with four different assays. Analysis revealed that patients with IOPD exhibited markedly lower GAA activity in skeletal muscles than those with LOPD, regardless of the measurement method employed. Additionally, patients with IOPD had notably higher glycogen content levels in skeletal muscles than those with LOPD. In general, however, it was difficult to fully characterize GAA activity because of the different methods used. The findings underscore the challenges in the interpretation and comparison of the results across studies because of the substantial methodological variations. There is a need to establish standardized reference ranges of GAA activity and glycogen content in healthy individuals and in Pompe disease patients based on globally standardized methods to improve comparability and reliability in assessing this rare disease.
PubMed: 38698877
DOI: 10.1016/j.ymgmr.2024.101085 -
Orphanet Journal of Rare Diseases Apr 2024Fabry disease (FD) is a rare lysosomal storage disease associated with glycolipid accumulation that impacts multiple physiological systems. We conducted a systematic... (Review)
Review
BACKGROUND
Fabry disease (FD) is a rare lysosomal storage disease associated with glycolipid accumulation that impacts multiple physiological systems. We conducted a systematic literature review (SLR) to characterize the humanistic (quality of life [QoL]) and economic burden of FD.
METHODS
Searches were conducted in the Embase, MEDLINE, and MEDLINE In-Process databases from inception to January 19, 2022. Conference abstracts of specified congresses were manually searched. Additional searches were performed in the Cochrane and ProQuest databases for the humanistic SLR and the National Health Service Economic Evaluations Database for the economic SLR. Studies of patients with FD of any sex, race, and age, and published in the English language were included. There was no restriction on intervention or comparator. For the humanistic SLR, studies that reported utility data, database/registry-based studies, questionnaires/surveys, and cohort studies were included. For the economic SLR, studies reporting economic evaluations or assessing the cost of illness and resource use were included.
RESULTS
Of the 1363 records identified in the humanistic search, 36 studies were included. The most commonly used QoL assessments were the 36-item Short-Form Health Survey (n = 16), EQ-5D questionnaire descriptive system or visual analog scale (n = 9), and the Brief Pain Inventory (n = 8). Reduced QoL was reported in patients with FD compared with healthy populations across multiple domains, including pain, physical functioning, and depressive symptoms. Multiple variables-including sex, age, disease severity, and treatment status-impacted QoL. Of the 711 records identified in the economic burden search, 18 studies were included. FD was associated with high cost and healthcare resource use. Contributors to the cost burden included enzyme replacement therapy, healthcare, and social care. In the seven studies that reported health utility values, lower utility scores were generally associated with more complications (including cardiac, renal, and cerebrovascular morbidities) and with classical disease in males.
CONCLUSION
FD remains associated with a high cost and healthcare resource use burden, and reduced QoL compared with healthy populations. Integrating information from QoL and economic assessments may help to identify interventions that are likely to be of most value to patients with FD.
Topics: Fabry Disease; Humans; Quality of Life; Cost of Illness; Male
PubMed: 38689282
DOI: 10.1186/s13023-024-03131-y -
Frontiers in Genetics 2024Bibliometrics can trace general research trends in a particular field. Mucopolysaccharidoses (MPS), as a group of rare genetic diseases, seriously affect the quality of...
Bibliometrics can trace general research trends in a particular field. Mucopolysaccharidoses (MPS), as a group of rare genetic diseases, seriously affect the quality of life of patients and their families. Scholars have devoted themselves to studying MPS's pathogenesis and treatment modalities and have published many papers. Therefore, we conducted a bibliometric and visual study of the top 100 most highly cited articles to provide researchers with an indication of the current state of research and potential directions in the field. The Web of Science Core Collection was searched for articles on MPS from 1 January 1900, to 8 November 2023, and the top 100 most cited articles were screened. The title, year of publication, institution, country, and first author of the articles were extracted and statistically analyzed using Microsoft Excel 2007. Keyword co-occurrence and collaborative networks were analyzed using VOSviewer 1.6.16. A total of 9,273 articles were retrieved, and the top 100 most cited articles were filtered out. The articles were cited 18,790 times, with an annual average of 188 citations (122-507). Forty-two journals published these articles, with Molecular Genetics and Metabolism and Proceedings of the National Academy of Sciences of the United States being the most published journal (N = 8), followed by Pediatrics (N = 7), Blood (N = 6). The United States (N = 68), the UK (N = 25), and Germany (N = 20) were the top contributing countries. The Royal Manchester Children's Hospital (N = 20) and the University of North Carolina (N = 18) were the most contributing institutions. Muenzer J was the most prolific author (N = 14). We conducted a bibliometric and visual analysis of the top 100 cited articles in MPS. This study identifies the most influential articles currently available in the field of MPS, which provides a good basis for a better understanding of the disease and informs future research directions.
PubMed: 38680422
DOI: 10.3389/fgene.2024.1377743 -
Heliyon Apr 2024Seafood, being highly perishable, faces rapid deterioration in freshness, posing spoilage risks and potential health concerns without proper preservation. To combat... (Review)
Review
Seafood, being highly perishable, faces rapid deterioration in freshness, posing spoilage risks and potential health concerns without proper preservation. To combat this, various innovative preservation and packaging technologies have emerged. This review delves into these cutting-edge interventions designed to minimize spoilage and effectively prolong the shelf life of fresh seafood products. Techniques like High-Pressure Processing (HPP), Modified Atmosphere Packaging (MAP), bio-preservation, and active and vacuum packaging have demonstrated the capability to extend the shelf life of seafood products by up to 50%. However, the efficacy of these technologies relies on factors such as the specific type of seafood product and the storage temperature. Hence, careful consideration of these factors is essential in choosing an appropriate preservation and packaging technology.
PubMed: 38655319
DOI: 10.1016/j.heliyon.2024.e29066 -
Advanced Science (Weinheim,... Jun 2024Hydrogen-bonded organic frameworks (HOFs) are emerging porous materials that show high structural flexibility, mild synthetic conditions, good solution processability,... (Review)
Review
Hydrogen-bonded organic frameworks (HOFs) are emerging porous materials that show high structural flexibility, mild synthetic conditions, good solution processability, easy healing and regeneration, and good recyclability. Although these properties give them many potential multifunctional applications, their frameworks are unstable due to the presence of only weak and reversible hydrogen bonds. In this work, the development history and synthesis methods of HOFs are reviewed, and categorize their structural design concepts and strategies to improve their stability. More importantly, due to the significant potential of the latest HOF-related research for addressing energy and environmental issues, this work discusses the latest advances in the methods of energy storage and conversion, energy substance generation and isolation, environmental detection and isolation, degradation and transformation, and biological applications. Furthermore, a discussion of the coupling orientation of HOF in the cross-cutting fields of energy and environment is presented for the first time. Finally, current challenges, opportunities, and strategies for the development of HOFs to advance their energy and environmental applications are discussed.
PubMed: 38647267
DOI: 10.1002/advs.202400101 -
Heliyon Apr 2024Numerous published studies have highlighted discrepancies in the duration and storage temperature used for preserving vitamin C content on various citrus genotypes...
Numerous published studies have highlighted discrepancies in the duration and storage temperature used for preserving vitamin C content on various citrus genotypes worldwide. The present study aimed to analyze the variation in vitamin C content as influenced by citrus genotype, duration, and storage temperature using meta-analysis approaches. Data searching, selection, and tabulation resulted in a comprehensive database constructed from 1412 data points gathered from 54 individual studies, following PRISMA-P guidelines. The vitamin C content varied widely, ranging from 0 to 76.2 mg/100 mL in whole data of citrus fruit. Meta-analysis findings revealed that the duration of storage significantly impacted the vitamin C content in citrus fruits. Specifically, for grapefruit, mandarin, and orange, the length of storage significantly influenced their vitamin C levels ( < 0.01), with a consistent decrease observed over time. The correlation coefficients (R) were 0.63 for grapefruit, 0.9 for mandarin, and 0.69 for orange. In contrast, no significant difference was found in terms of vitamin C levels between hybrid and lime citrus concerning the impact of storage time. However, other results indicated a significant influence of storage temperature on the variation in vitamin C levels for both citrus and hybrid varieties ( < 0.001). Depending on the genotype, tangerine had significantly lower vitamin C content compared to other varieties, at 16.9 mg/100 mL, with vitamin C ranging from 13.2 to 20.9 mg/100 mL ( < 0.001). The highest vitamin C content was found in lemon and hybrid varieties, around 65.5 (range 59.3-76.2) and 48.3 (range 29.6-75.5), respectively, all in mg/100 mL ( < 0.001). Furthermore, there was a tendency for decreasing vitamin C concentration due to temperature ( = 0.078), while citrus variety experienced a decrease, although not significant. The ideal temperature (15 °C) and duration of storage (56 days) to minimize vitamin C loss in citrus fruits are at their optimum point. In conclusion, the deterioration of vitamin C in citrus fruits is influenced by both temperature and storage duration, and its content is also impacted by the variety of citrus.
PubMed: 38644865
DOI: 10.1016/j.heliyon.2024.e29125 -
Spinal Cord Jun 2024Systematic review and meta-analysis. (Meta-Analysis)
Meta-Analysis
STUDY DESIGN
Systematic review and meta-analysis.
OBJECTIVES
The current study aimed to assess the efficacy and safety of Onabotulinum toxin A (OBTX-A) treatment for neurogenic detrusor overactivity (NDO) in spinal cord injury (SCI) patients.
SETTING
Iran.
METHODS
All relevant articles of clinical trials and cohort studies indexed in PubMed/MEDLINE, Embase, Scopus, and Web of Science databases up to September 6, 2022, that addressed OBTX-A treatment for NDO following SCI were included. The quality of eligible studies was evaluated using Cochrane criteria. Also, the weighted mean difference (WMD) was measured with a random-effect model.
RESULTS
Regarding the overall efficacy after OBTX-A treatment in the short term, volume per void (VV) (WMD = 118.8, 95% CI: 90.9-146.7, p < 0.01), incontinence-quality of life (IQoL) (WMD = 24.3, 95% CI: 15.8-32.8, p < 0.01), and maximum cystometric capacity (MCC) (WMD = 144.5, 95% CI: 132.3 to 156.7, p < 0.01) significantly increased, while maximum detrusor pressure during storage (MDP) (WMD = -30.5, 95% CI: -35.9 to -25.1, p < 0.01) showed a significant decrease. Furthermore, compared to the placebo group at the 200-unit dose, there was a significant increase in MCC (WMD = 113.5, 95% CI: 84.7 to 142.3, p < 0.01) and a significant decrease in MDP (WMD = -27.2, 95% CI: -39.2 to -15.1, p < 0.01). Urinary tract infection (UTI), hematuria, and autonomic dysreflexia were the most common side effects, occurring at rates of 29.6%, 14.8%, and 13.4%, respectively.
CONCLUSION
Our findings highlighted the effectiveness and safety of OBTX-A as a promising treatment of NDO following SCI.
Topics: Humans; Botulinum Toxins, Type A; Neuromuscular Agents; Spinal Cord Injuries; Urinary Bladder, Neurogenic; Urinary Bladder, Overactive
PubMed: 38637637
DOI: 10.1038/s41393-024-00992-9 -
The British Journal of Surgery Apr 2024Machine perfusion is an organ preservation strategy used to improve function over simple storage in a cold environment. This article presents an updated systematic... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Machine perfusion is an organ preservation strategy used to improve function over simple storage in a cold environment. This article presents an updated systematic review and meta-analysis of machine perfusion in deceased donor kidneys.
METHODS
RCTs from November 2018 to July 2023 comparing machine perfusion versus static cold storage in kidney transplantation were evaluated for systematic review. The primary outcome in meta-analysis was delayed graft function.
RESULTS
A total 19 studies were included, and 16 comparing hypothermic machine perfusion with static cold storage were analysed. The risk of delayed graft function was lower with hypothermic machine perfusion (risk ratio (RR) 0.77, 95% c.i. 0.69 to 0.86), even in kidneys after circulatory death (RR 0.78, 0.68 to 0.90) or brain death (RR 0.73, 0.63 to 0.84). Full hypothermic machine perfusion decreased the risk of delayed graft function (RR 0.69, 0.60 to 0.79), whereas partial hypothermic machine perfusion did not (RR 0.92, 0.69 to 1.22). Normothermic machine perfusion or short-term oxygenated hypothermic machine perfusion preservation after static cold storage was equivalent to static cold storage in terms of delayed graft function and 1-year graft survival.
CONCLUSION
Hypothermic machine perfusion reduces delayed graft function risks and normothermic approaches show promise.
Topics: Humans; Delayed Graft Function; Graft Survival; Kidney; Kidney Transplantation; Organ Preservation; Perfusion; Randomized Controlled Trials as Topic
PubMed: 38637312
DOI: 10.1093/bjs/znae102