-
Progress in Biophysics and Molecular... May 2024The emergence, evolution, and spread of life on Earth have all occurred in the geomagnetic field, and its extensive biological effects on living organisms have been... (Review)
Review
The emergence, evolution, and spread of life on Earth have all occurred in the geomagnetic field, and its extensive biological effects on living organisms have been documented. The charged characteristics of metal ions in biological fluids determine that they are affected by electromagnetic field forces, thus affecting life activities. Iron metabolism, as one of the important metal metabolic pathways, keeps iron absorption and excretion in a relatively balanced state, and this process is precisely and completely controlled. It is worth paying attention to how the iron metabolism process of living organisms is changed when exposed to electromagnetic fields. In this paper, the processes of iron absorption, storage and excretion in animals (mammals, fish, arthropods), plants and microorganisms exposed to electromagnetic field were summarized in detail as far as possible, in order to discover the regulation of iron metabolism by electromagnetic field. Studies and data on the effects of electromagnetic field exposure on iron metabolism in organisms show that exposure profiles vary widely across species and cell lines. This process involves a variety of factors, and the complexity of the results is not only related to the magnetic flux density/operating frequency/exposure time and the heterogeneity of the observed object. A systematic review of the biological regulation of iron metabolism by electromagnetic field exposure will not only contributes to a more comprehensive understanding of its biological effects and mechanism, but also is necessary to improve human awareness of the health related risks of electromagnetic field exposure.
Topics: Humans; Animals; Electromagnetic Fields; Mammals; Time; Iron
PubMed: 38447710
DOI: 10.1016/j.pbiomolbio.2024.03.001 -
Frontiers in Pediatrics 2024Pompe disease (PD) is a glycogen disorder caused by the deficient activity of acid alpha-glucosidase (GAA). We sought to review the latest available evidence on the... (Review)
Review
Efficacy and safety of enzyme replacement therapy with alglucosidase alfa for the treatment of patients with infantile-onset Pompe disease: a systematic review and metanalysis.
INTRODUCTION
Pompe disease (PD) is a glycogen disorder caused by the deficient activity of acid alpha-glucosidase (GAA). We sought to review the latest available evidence on the safety and efficacy of recombinant human GAA enzyme replacement therapy (ERT) for infantile-onset PD (IOPD).
METHODS
We systematically searched the MEDLINE (via PubMed) and Embase databases for prospective clinical studies evaluating ERT for IOPD on pre-specified outcomes. Meta-analysis was also performed.
RESULTS
Of 1,722 articles identified, 16 were included, evaluating 316 patients. Studies were heterogeneous and with very low certainty of evidence for most outcomes. A moderate/high risk of bias was present for most included articles. The following outcomes showed improvements associated with alglucosidase alfa, over natural history of PD/placebo, for a mean follow-up of 48.3 months: left ventricular (LV) mass {mean change 131.3 g/m [95% confidence interval (CI) 81.02, 181.59]}, time to start ventilation (TSV) [HR 0.21 (95% CI: 0.12, 0.36)], and survival [HR 0.10 (95% CI: 0.05, 0.19)]. There were no differences between the pre- and post-ERT period for myocardial function and psychomotor development. Adverse events (AEs) after ERT were mild in most cases.
CONCLUSION
Our data suggest that alglucosidase alfa potentially improves LV mass, TSV, and survival in IOPD patients, with no important safety issues.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO identifier (CRD42019123700).
PubMed: 38425665
DOI: 10.3389/fped.2024.1310317 -
EJHaem Feb 2024Gaucher disease (GD) is a heterogeneous condition requiring tailored treatment approaches. The aim of this systematic review was to synthesise and evaluate current... (Review)
Review
Gaucher disease (GD) is a heterogeneous condition requiring tailored treatment approaches. The aim of this systematic review was to synthesise and evaluate current evidence pertaining to the use of Ambroxol for the treatment of GD. Published and unpublished literature databases, conference proceedings and the reference lists of included studies were searched until 23 November 2023. A narrative synthesis was performed. Database search and risk of bias assessment were performed independently by two reviewers. Twenty-one studies (182 patients) were included. The evidence was low in quality. Variable responses to Ambroxol were observed. Response rates were 36% and 55% in two studies reporting on type 1 GD. One study found a 22% response rate in type 2 GD, whereas another study found 29% of patients with type 3 GD reported neurological improvements. No severe adverse events were reported in the literature, with mild and reversible side effects reported. Varying response rates are to be expected (29%-100%) when treating neurological manifestations. Varying degrees of symptomatic improvement for the treatment of GD were noted in the literature. Multidisciplinary team input and clinical judgement are advised to provide personalized treatment of this complex and multi-faceted condition.
PubMed: 38406552
DOI: 10.1002/jha2.852 -
Human Reproduction Open 2024Does sperm cryopreservation serve as a feasible and effective method for preserving fertility in adult male patients with cancer? (Review)
Review
STUDY QUESTION
Does sperm cryopreservation serve as a feasible and effective method for preserving fertility in adult male patients with cancer?
SUMMARY ANSWER
Sperm cryopreservation is an effective fertility preservation method and may benefit patients with cancer.
WHAT IS KNOWN ALREADY
Sperm cryopreservation is the only way to efficiently preserve male fertility. It is an important procedure in ART. Recently, due to remarkable advances in cancer treatment, an increasing number of studies have reported the outcomes of sperm cryopreservation in patients with cancer.
STUDY DESIGN SIZE DURATION
We conducted an extensive literature search for relevant studies published through to 31 December 2021, in the following databases: CENTRAL, CNKI, Cochrane Systematic Reviews, EMBASE, MEDLINE, PUBMED, and Web of Science. The search terms used were '(cryopreservation OR freeze OR freezing OR banking OR cryostorage OR storage) AND (sperm OR semen OR spermatozoon) AND (cancer OR tumor OR malignancy OR neoplasm)'.
PARTICIPANTS/MATERIALS SETTING METHODS
We included all studies that reported offering or attempting to cryopreserve sperm before or during cancer treatment in male patients considered at risk of treatment-related fertility impairment. We evaluated the eligibility of all data in each study. The major exclusion criteria were as follows: non-cancer patients; pediatric and adolescent cancer patients; not reporting the use of cryopreserved sperm; use of fresh semen for ART; not reporting the number of patients with cancer offered sperm cryopreservation or attempting to do so before or during treatment; using an experimental fertility preservation technique such as preservation of testicular tissue or spermatogonial stem cells; duplicate data; abstracts, case report, comments, reviews, or editorials; insufficient data reported. The quality of the included studies was assessed using the Newcastle-Ottawa scale and the Methodological Index for Non-Randomized Studies.
MAIN RESULTS AND THE ROLE OF CHANCE
This meta-analysis included 69 non-randomized studies, with 32 234 patients referred for sperm analysis and 23 178 patients cryopreserving at least one sperm sample. The pooled failed-to-cryopreserve rate was 10% (95% CI, 8-12%), and the sperm disposal and sperm use rates were 23% (95% CI, 16-30%) and 9% (95% CI, 8-10%), respectively. The pregnancy, miscarriage, and delivery rates were 28% (95% CI, 22-33%), 13% (95% CI, 10-17%), and 20% (95% CI, 15-25%), respectively. Subgroup analysis showed higher pregnancy and delivery rates, as well as a lower failed-to-cryopreserve rate, in recent studies compared to those released a decade ago. The studies from Asia reported higher sperm disposal and pregnancy rates than in other continents. Our analysis showed clinical pregnancy rates per cycle of 34% (27-41%), 24% (14-35%), and 9% (5-15%) and delivery rates per cycle of 23% (17-30%), 18% (11-26%), and 5% (1-9%) for ICSI, IVF, and IUI, respectively.
LIMITATIONS REASONS FOR CAUTION
As with all meta-analyses, some limitations should be considered. The first limitation of our study is that the data span 36 years. During this time, the World Health Organization has revised its sperm analysis standards, and other important changes have been made. There is also a limitation in that the outcome does not analyze the correlation between the type of cancer and sperm quality. Many of the earlier studies were limited by small sample sizes and a lack of control groups. Furthermore, almost all studies did not consider the severity of the disease, which could potentially have a substantial impact on the results. Consequently, further research should evaluate the effect of the type of cancer and, in particular, the severity of the condition on sperm quality in order to draw more precise conclusions. Similarly, it is inappropriate that most studies failed to differentiate between patients with different types of tumors and instead drew generalized conclusions that are presumed to apply to all patients with cancer. In the present analysis, we did not have in-depth information on patients' disease, and although extensive efforts were made to conduct a thorough systematic review and meta-analysis of the outcomes for patients with various types of tumors, the results must be acknowledged as being subject to bias. However, the use of average results obtained in each study, without the patient-level data, might also represent a source of bias.
WIDER IMPLICATIONS OF THE FINDINGS
Sperm cryopreservation is an effective fertility preservation method and may benefit patients with cancer. The observed utilization rate of frozen sperm at 9% may underestimate the actual usage, as the short follow-up period is inadequate for obtaining comprehensive data on the use of frozen sperm in young cancer survivors. ART plays an important role in fertility preservation and the achievement of pregnancy, with this meta-analysis showing that ICSI delivers better clinical outcomes than IVF or IUI in patients with cancer undergoing fertility preservation.
STUDY FUNDING/COMPETING INTERESTS
This work was supported by the National Natural Science Foundation of China (grant no. 82001634, 81960550), and the China Postdoctoral Science Foundation (2019M661521). There are no competing interests to declare.
REGISTRATION NUMBER
CRID 42022314460.
PubMed: 38389980
DOI: 10.1093/hropen/hoae006 -
Orphanet Journal of Rare Diseases Feb 2024Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease caused by deficiency in arylsulfatase A (ASA) activity arising primarily from ASA...
BACKGROUND
Metachromatic leukodystrophy (MLD) is an autosomal recessive lysosomal storage disease caused by deficiency in arylsulfatase A (ASA) activity arising primarily from ASA gene (ARSA) variants. Late-infantile, juvenile and adult clinical subtypes are defined by symptom onset at ≤ 2.5, > 2.5 to < 16 and ≥ 16 years, respectively. Epidemiological data were sought to address knowledge gaps and to inform decisions regarding the clinical development of an investigational drug.
METHODS
To synthesize all available estimates of MLD incidence and birth prevalence worldwide and in selected countries, Ovid MEDLINE and Embase were searched systematically (March 11, 2022) using a population, intervention, comparator, outcome, time and setting framework, complemented by pragmatic searching to reduce publication bias. Where possible, results were stratified by clinical subtype. Data were extracted from non-interventional studies (clinical trials, non-clinical studies and case reports were excluded; reviews were used for snowballing only).
RESULTS
Of the 31 studies included, 14 reported birth prevalence (13 countries in Asia-Pacific, Europe, the Middle East, North America and South America), one reported prevalence and none reported incidence. Birth prevalence per 100,000 live births ranged from 0.16 (Japan) to 1.85 (Portugal). In the three European studies with estimates stratified by clinical subtypes, birth prevalence was highest for late-infantile cases (0.31-1.12 per 100,000 live births). The distribution of clinical subtypes reported in cases diagnosed over various time periods in 17 studies varied substantially, but late-infantile and juvenile MLD accounted for at least two-thirds of cases in most studies.
CONCLUSIONS
This review provides a foundation for further analysis of the regional epidemiology of MLD. Data gaps indicate the need for better global coverage, increased use of epidemiological measures (e.g. prevalence estimates) and more stratification of outcomes by clinical and genetic disease subtype.
Topics: Adult; Humans; Cerebroside-Sulfatase; Europe; Leukodystrophy, Metachromatic; Lysosomal Storage Diseases; Prevalence
PubMed: 38383398
DOI: 10.1186/s13023-024-03044-w -
Orphanet Journal of Rare Diseases Feb 2024Late-onset multiple acyl-CoA dehydrogenase deficiency (MADD) is the most common lipid storage myopathy. There are sex differences in fat metabolism and it is not known... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Late-onset multiple acyl-CoA dehydrogenase deficiency (MADD) is the most common lipid storage myopathy. There are sex differences in fat metabolism and it is not known whether late-onset MADD affects men and women equally.
METHODS
In this systematic review and meta-analysis, the PubMed, Embase, Web of Science, CNKI, CBM, and Wanfang databases were searched until 01/08/2023. Studies reporting sex distribution in patients with late-onset MADD were included. Two authors independently screened studies for eligibility, extracted data, and assessed risk of bias. Pre-specified outcomes of interest were the male-to-female ratio (MFR) of patients with late-onset MADD, the differences of clinical characteristics between the sexes, and factors influencing the MFR.
RESULTS
Of 3379 identified studies, 34 met inclusion criteria, yielding a total of 609 late-onset MADD patients. The overall pooled percentage of males was 58% (95% CI, 54-63%) with low heterogeneity across studies (I = 2.99%; P = 0.42). The mean onset ages, diagnostic delay, serum creatine kinase (CK), and allelic frequencies of 3 hotspot variants in ETFDH gene were similar between male and female patients (P > 0.05). Meta-regressions revealed that ethnic group was associated with the MFR in late-onset MADD, and subgroup meta-analyses demonstrated that East-Asian patients had a higher percentage of male, lower CK, and higher proportion of hotspot variants in ETFDH gene than non-East-Asian patients (P < 0.05).
CONCLUSIONS
Male patients with late-onset MADD were more common than female patients. Ethnicity was proved to be a factor influencing the MFR in late-onset MADD. These findings suggest that male sex may be a risk factor for the disease.
Topics: Humans; Male; Female; Multiple Acyl Coenzyme A Dehydrogenase Deficiency; Mutation; Delayed Diagnosis; Electron-Transferring Flavoproteins; Iron-Sulfur Proteins; Oxidoreductases Acting on CH-NH Group Donors; Acyl-CoA Dehydrogenase
PubMed: 38365830
DOI: 10.1186/s13023-024-03072-6 -
Journal of Human Lactation : Official... May 2024Raw, never stored or pasteurized mother's own milk (MOM) is not always available to feed preterm infants; however, storage and pasteurization of MOM diminishes some... (Review)
Review
BACKGROUND
Raw, never stored or pasteurized mother's own milk (MOM) is not always available to feed preterm infants; however, storage and pasteurization of MOM diminishes some bioactive components. It can be difficult to feed raw MOM to preterm infants due to transportation and storage of small volumes that might be pumped away from the infant, and a concern that they might harbor bacteria. However, the higher availability of bioactive components in raw MOM may provide benefits to preterm infants compared to frozen or pasteurized MOM.
RESEARCH AIM
To systematically review and summarize the results of studies on feeding raw MOM versus frozen or pasteurized MOM to preterm infants born at less than 37 weeks of gestation.
METHODS
Four databases were searched (Cochrane, Embase, Ovid MEDLINE, and Web of Science) for this systematic review. Of 542 studies identified, nine met inclusion criteria and were critically evaluated using the quality assessment tool for quantitative studies by the Effective Public Health Practice Project. Studies were organized using the Breastfeeding Challenges Facing Preterm Mother-Infant Dyads theoretical framework.
RESULTS
Included studies evaluated the outcomes of preterm infants fed raw versus pasteurized MOM ( = 7, 77.8%) or raw versus frozen MOM ( = 2, 22.2%). Researchers found that raw MOM did not increase infant infections and may have improved health and growth outcomes for study participants.
CONCLUSION
There is laboratory evidence supporting the safety and efficacy of the use of raw MOM for preterm infants. A raw MOM diet is recommended for preterm infants by professional organizations. Despite this, it may not be universally prioritized and could require purposeful implementation by each institution. Further research is needed to pursue the potential benefits of a raw MOM diet for preterm infants.
Topics: Infant; Female; Infant, Newborn; Humans; Infant, Premature; Breast Feeding; Milk, Human; Mothers; Diet; Infant, Very Low Birth Weight; Intensive Care Units, Neonatal
PubMed: 38328919
DOI: 10.1177/08903344241227941 -
BMC Oral Health Feb 2024Human saliva as a bodily fluid-similar to blood-is utilized for diagnostic purposes. Unlike blood sampling, collecting saliva is non-invasive, inexpensive, and readily...
BACKGROUND
Human saliva as a bodily fluid-similar to blood-is utilized for diagnostic purposes. Unlike blood sampling, collecting saliva is non-invasive, inexpensive, and readily accessible. There are no previously published systematic reviews regarding different collection, transportation, preparation, and storage methods for human saliva.
DESIGN
This study has been prepared and organized according to the preferred reporting items for systematic reviews and meta-analyses (PRISMA) 2020 guidelines. This systematic review has been registered at PROSPERO (Registration ID: CRD42023415384). The study question according to the PICO format was as followed: Comparison of the performance (C) of different saliva sampling, handling, transportation, and storage techniques and methods (I) assessed for analyzing stimulated or unstimulated human saliva (P and O). An electronic search was executed in Scopus, Google Scholar, and PubMed.
RESULTS
Twenty-three descriptive human clinical studies published between 1995 and 2022 were included. Eight categories of salivary features and biomarkers were investigated (i.e., salivary flow rate, total saliva quantity, total protein, cortisol, testosterone, DNA quality and quantity, pH and buffering pH). Twenty-two saliva sampling methods/devices were utilized. Passive drooling, Salivette®, and spitting were the most utilized methods. Sampling times with optimum capabilities for cortisol, iodine, and oral cancer metabolites are suggested to be 7:30 AM to 9:00 AM, 10:30 AM to 11:00 AM, and 14:00 PM to 20:00 PM, respectively. There were 6 storage methods. Centrifuging samples and storing them at -70 °C to -80 °C was the most utilized storage method. For DNA quantity and quality, analyzing samples immediately after collection without centrifuging or storage, outperformed centrifuging samples and storing them at -70 °C to -80 °C. Non-coated Salivette® was the most successful method/device for analyzing salivary flow rate.
CONCLUSION
It is highly suggested that scientists take aid from the reported categorized outcomes, and design their study questions based on the current voids for each method/device.
Topics: Humans; Hydrocortisone; Saliva; Biomarkers; Specimen Handling; DNA
PubMed: 38308289
DOI: 10.1186/s12903-024-03902-w -
Ecology and Evolution Jan 2024Naturalistic plantings, such as meadow-style plantings, can improve the quality of urban green spaces through aesthetic, biodiversity and low maintenance features.... (Review)
Review
Naturalistic plantings, such as meadow-style plantings, can improve the quality of urban green spaces through aesthetic, biodiversity and low maintenance features. Species selection for, and maintenance of naturalistic plantings are key to their success. While herbaceous and grassy meadows can be mowed, naturalistic plantings with woody plants require more intense maintenance to remove biomass and promote resprouting. We aim to understand woody plant responses to diverse disturbance regimes to potentially inform the selection and management of woody species in urban plantings. We conducted a quantitative systematic literature review of 72 papers and investigated what main external (climate, disturbance regime) and internal (buds, life stage, storage reserves) factors influence the resprouting response of woody plants. We found resprouting literature is geographically widespread for woody plants, but studies are skewed towards Temperate climates in USA and Australia, with a focus on high severity and high frequency fire disturbance. Resprouting response was mostly defined as a continuous response to disturbance dependent on disturbance regime, climate and plant traits. Maintenance and management of naturalistic woody plantings, through hard pruning techniques such as coppicing, may be informed by analogous high severity and high frequency disturbance studies. However, the literature on woody plant resprouting has several knowledge gaps for lower severity and lower frequency disturbance regimes and in more arid climates. Future research should evaluate the response of naturalistic woody plantings to disturbance in specific urban contexts.
PubMed: 38235408
DOI: 10.1002/ece3.10839 -
JMIR MHealth and UHealth Feb 2024In the modern world, mobile apps are essential for human advancement, and pandemic control is no exception. The use of mobile apps and technology for the detection and... (Review)
Review
BACKGROUND
In the modern world, mobile apps are essential for human advancement, and pandemic control is no exception. The use of mobile apps and technology for the detection and diagnosis of COVID-19 has been the subject of numerous investigations, although no thorough analysis of COVID-19 pandemic prevention has been conducted using mobile apps, creating a gap.
OBJECTIVE
With the intention of helping software companies and clinical researchers, this study provides comprehensive information regarding the different fields in which mobile apps were used to diagnose COVID-19 during the pandemic.
METHODS
In this systematic review, 535 studies were found after searching 5 major research databases (ScienceDirect, Scopus, PubMed, Web of Science, and IEEE). Of these, only 42 (7.9%) studies concerned with diagnosing and detecting COVID-19 were chosen after applying inclusion and exclusion criteria using the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) protocol.
RESULTS
Mobile apps were categorized into 6 areas based on the content of these 42 studies: contact tracing, data gathering, data visualization, artificial intelligence (AI)-based diagnosis, rule- and guideline-based diagnosis, and data transformation. Patients with COVID-19 were identified via mobile apps using a variety of clinical, geographic, demographic, radiological, serological, and laboratory data. Most studies concentrated on using AI methods to identify people who might have COVID-19. Additionally, symptoms, cough sounds, and radiological images were used more frequently compared to other data types. Deep learning techniques, such as convolutional neural networks, performed comparatively better in the processing of health care data than other types of AI techniques, which improved the diagnosis of COVID-19.
CONCLUSIONS
Mobile apps could soon play a significant role as a powerful tool for data collection, epidemic health data analysis, and the early identification of suspected cases. These technologies can work with the internet of things, cloud storage, 5th-generation technology, and cloud computing. Processing pipelines can be moved to mobile device processing cores using new deep learning methods, such as lightweight neural networks. In the event of future pandemics, mobile apps will play a critical role in rapid diagnosis using various image data and clinical symptoms. Consequently, the rapid diagnosis of these diseases can improve the management of their effects and obtain excellent results in treating patients.
Topics: Humans; COVID-19; Pandemics; Artificial Intelligence; SARS-CoV-2; Mobile Applications; COVID-19 Testing
PubMed: 38231538
DOI: 10.2196/44406