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Journal of Pediatric Endocrinology &... Sep 2023Glycogen storage disease (GSD) type 1a is an inherited autosomal recessive metabolic disease caused by a deficiency in glucose-6-phosphatase activity. The objectives of... (Review)
Review
Glycogen storage disease (GSD) type 1a is an inherited autosomal recessive metabolic disease caused by a deficiency in glucose-6-phosphatase activity. The objectives of this research were to systematically review the published literature on the epidemiology of GSD 1a and to assess the performance of reported epidemiology measures in a simulation model. In this systematic literature review 2,539 record titles and abstracts were screened. Of these, only 11 studies contained relevant data on GSD 1a disease epidemiology. Reported disease frequency ranged from 0.085/100,000 to 10.3/100,000 newborns when considering all the GSD literature. When this was narrowed to GSD 1 and GSD 1a, the range was tightened to 0.25-3.02/100,000 and 0.085-4.9/100,000 newborns, respectively. Most of the identified studies counted the number of diagnoses in a defined period and related to the number of births in the same (Dx method) or different time period (DoB method). The simulation model results indicate that in most of the situations, the Dx method provides a closer estimate to the true disease incidence than the DoB method. Despite the scarcity of epidemiology data, the results of this systematic review strongly support that GSD 1a and its parent disease groups (GSD and GSD 1) are rare diseases.
Topics: Infant, Newborn; Humans; Pregnancy; Female; Glycogen Storage Disease Type I; Glucose-6-Phosphatase; Parents; Parturition
PubMed: 37615591
DOI: 10.1515/jpem-2023-0127 -
Cureus Jul 2023India is native to many tribal communities: Bharia (Madhya Pradesh), Bihl (Rajasthan), Santhal (Bihar, Jharkhand), Bodo (Assam, West Bengal), and many more. They reside... (Review)
Review
India is native to many tribal communities: Bharia (Madhya Pradesh), Bihl (Rajasthan), Santhal (Bihar, Jharkhand), Bodo (Assam, West Bengal), and many more. They reside in isolated geographical regions, which poses challenges in reaching out to them. In addition, they still have firm beliefs and taboos regarding menstruation. Knowledge about menstrual health and hygiene is one of the most important aspects of tribal health. Therefore, it is important to synthesize the results of menstrual hygiene data from the Indian tribal population. We have calculated the pooled prevalence of sanitary pad use, dustbin disposal, and hygienic reuse of menstrual materials. Online databases, namely PubMed, Cochrane Central, CINAHL, Pan African Journals, EBSCO, and Google Scholar, were searched. After the removal of duplicates, a quality check, and screening of cross-references, 19 articles were selected for final review. Statistical analysis was done by Revman 5.4 and STATA 17.0. A p-value of <0.05 was considered statistically significant. PRISMA guidelines were followed. The protocol registration number was CRD42022331376. This is a non-funded article. The pooled prevalence of sanitary pad use in Indian tribal females was 2% (95% CI 1 to 3). The pooled prevalence of dustbin disposal of menstrual material was 1% (95% CI: 0.00 to 0.02). The pooled prevalence of hygienic reuse of menstrual materials was 1%. Sanitary menstrual hygiene practices are very less prevalent in Indian tribal females. Awareness programs and tribal health policies need to be accelerated for the promotion of menstrual hygiene. Also, literature on the use, disposal, and storage of menstrual adsorbents is scarce in Indian tribes. Health research in this area needs to be emphasized.
PubMed: 37605713
DOI: 10.7759/cureus.42216 -
Journal of Medical Internet Research Aug 2023Video recordings of patients may offer advantages to supplement patient assessment and clinical decision-making. However, little is known about the practice of video... (Review)
Review
BACKGROUND
Video recordings of patients may offer advantages to supplement patient assessment and clinical decision-making. However, little is known about the practice of video recording patients for direct care purposes.
OBJECTIVE
We aimed to synthesize empirical studies published internationally to explore the extent to which video recording patients is acceptable and effective in supporting direct care and, for the United Kingdom, to summarize the relevant guidance of professional and regulatory bodies.
METHODS
Five electronic databases (MEDLINE, Embase, APA PsycINFO, CENTRAL, and HMIC) were searched from 2012 to 2022. Eligible studies evaluated an intervention involving video recording of adult patients (≥18 years) to support diagnosis, care, or treatment. All study designs and countries of publication were included. Websites of UK professional and regulatory bodies were searched to identify relevant guidance. The acceptability of video recording patients was evaluated using study recruitment and retention rates and a framework synthesis of patients' and clinical staff's perspectives based on the Theoretical Framework of Acceptability by Sekhon. Clinically relevant measures of impact were extracted and tabulated according to the study design. The framework approach was used to synthesize the reported ethico-legal considerations, and recommendations of professional and regulatory bodies were extracted and tabulated.
RESULTS
Of the 14,221 abstracts screened, 27 studies met the inclusion criteria. Overall, 13 guidance documents were retrieved, of which 7 were retained for review. The views of patients and clinical staff (16 studies) were predominantly positive, although concerns were expressed about privacy, technical considerations, and integrating video recording into clinical workflows; some patients were anxious about their physical appearance. The mean recruitment rate was 68.2% (SD 22.5%; range 34.2%-100%; 12 studies), and the mean retention rate was 73.3% (SD 28.6%; range 16.7%-100%; 17 studies). Regarding effectiveness (10 studies), patients and clinical staff considered video recordings to be valuable in supporting assessment, care, and treatment; in promoting patient engagement; and in enhancing communication and recall of information. Observational studies (n=5) favored video recording, but randomized controlled trials (n=5) did not demonstrate that video recording was superior to the controls. UK guidelines are consistent in their recommendations around consent, privacy, and storage of recordings but lack detailed guidance on how to operationalize these recommendations in clinical practice.
CONCLUSIONS
Video recording patients for direct care purposes appears to be acceptable, despite concerns about privacy, technical considerations, and how to incorporate recording into clinical workflows. Methodological quality prevents firm conclusions from being drawn; therefore, pragmatic trials (particularly in older adult care and the movement disorders field) should evaluate the impact of video recording on diagnosis, treatment monitoring, patient-clinician communication, and patient safety. Professional and regulatory documents should signpost to practical guidance on the implementation of video recording in routine practice.
TRIAL REGISTRATION
PROSPERO CRD42022331825: https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=331825.
Topics: Humans; Aged; Patient Participation; Empirical Research; Communication; Narration; Clinical Decision-Making
PubMed: 37585249
DOI: 10.2196/46478 -
International Journal of Surgery... Nov 2023The increasing use of extended criteria donors (ECD) sets higher requirements for graft preservation. Machine perfusion (MP) improves orthotopic liver transplantation... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The increasing use of extended criteria donors (ECD) sets higher requirements for graft preservation. Machine perfusion (MP) improves orthotopic liver transplantation (OLT) outcomes, but its effects on different donor types remains unclear. The authors' aim was to assess the effects of hypothermic machine perfusion (HMP), normothermic machine perfusion (NMP), or normothermic regional perfusion (NRP) versus static cold storage (SCS) on different donor types.
MATERIALS AND METHODS
A literature search comparing the efficacy of MP versus SCS in PubMed, Cochrane, and EMBASE database was conducted. A meta-analysis was performed to obtain pooled effects of MP on ECD, donation after circulatory death (DCD), and donor after brainstem death.
RESULTS
Thirty nine studies were included (nine randomized controlled trials and 30 cohort studies). Compared with SCS, HMP significantly reduced the risk of non-anastomotic biliary stricture (NAS) [odds ratio (OR) 0.43, 95% confidence interval (CI) 0.26-0.72], major complications (OR 0.55, 95% CI 0.39-0.78), and early allograft dysfunction (EAD) (OR 0.46, 95% CI 0.32-0.65) and improved 1-year graft survival (OR 2.36, 95% CI 1.55-3.62) in ECD-OLT. HMP also reduced primary non-function (PNF) (OR 0.40, 95% CI 0.18-0.92) and acute rejection (OR 0.62, 95% CI 0.40-0.97). NMP only reduced major complications in ECD-OLT (OR 0.56, 95% CI 0.34-0.94), without favorable effects on other complications and survival. NRP lowered the overall risk of NAS (OR 0.27, 95% CI 0.11-0.68), PNF (OR 0.43, 95% CI 0.22-0.85), and EAD (OR 0.58, 95% CI 0.42-0.80) and meanwhile improved 1-year graft survival (OR 2.40, 95% CI 1.65-3.49) in control DCD-OLT.
CONCLUSIONS
HMP might currently be considered for marginal livers as it comprehensively improves ECD-OLT outcomes. NMP assists some outcomes in ECD-OLT, but more evidence regarding NMP-ECD is warranted. NRP significantly improves DCD-OLT outcomes and is recommended where longer non-touch periods exist.
Topics: Humans; Liver Transplantation; Tissue Donors; Liver; Graft Survival; Perfusion; Organ Preservation
PubMed: 37578436
DOI: 10.1097/JS9.0000000000000661 -
Journal of the Mechanical Behavior of... Oct 2023This study aims to systematically review the literature and evaluate the effect of post-printing factors such as aging, heat, appliance fabrication and storage on the... (Review)
Review
OBJECTIVES
This study aims to systematically review the literature and evaluate the effect of post-printing factors such as aging, heat, appliance fabrication and storage on the dimensional accuracy of full-arch dental models manufactured by additive manufacturing (AM) technology for the intended use of working model purposes.
MATERIALS AND METHODS
Three online databases, Medline (Ovid), Scopus and Web of Science were screened and last searched in March 2023. In-vitro studies and publications involving any distortions and shrinkage to the additively manufactured (AMed) model after printing and post-processing were included. However, literature reviews, abstracts, publications in a language different from English, or publications not testing a dental model with an arch or dentition were excluded. The references cited in the studies included were also checked via Google Scholar to identify relevant published studies potentially missed.
RESULTS
The systematic search identified and screened 769 different studies after the removal of duplicates. After applying inclusion and exclusion criteria, a total of 30 relevant titles and abstracts were found, yielding six final selections after full-text screening. Four out of the six studies evaluated the effect of both storage and aging on the dimensional accuracy of AMed dental models. The other two studies assessed the dimensional accuracy after the fabrication of thermoformed and vacuum-formed appliances on the AMed dental model.
CONCLUSIONS
AMed models can be utilised as working models on the condition that specific printing parameters are followed and additional model design features are employed. No definitive conclusions can be drawn on standardised methods to assess the dimensional accuracy of AMed dental models after storage, aging and appliance fabrication. In addition, there is no consensus on specific storage periods for an AMed model. Majority of study designs removed the palatal region to create a horseshoe shaped model, making the results less applicable to a working model scenario requiring the palate for retention purposes. The parameters investigated on AMed models include storage, aging, and appliance fabrication through thermoforming and vacuum-forming. Printing densities of solid models and wall thickness of hollow models were shown to influence the accuracy of AMed models. Dimensional accuracy of AMed models have been shown to be affected during appliance fabrication through thermoforming and vacuum-forming in certain conditions.
SIGNIFICANCE
There is a clear need of standardisation when manufacturing AMed dental models for working model purposes. The current methods investigated in this study lack established protocols to accurately manufacture the AMed models, and effectively store and utilise an AMed dental model for fabrication of orthodontic and prosthodontic appliances.
PubMed: 37542769
DOI: 10.1016/j.jmbbm.2023.106057 -
Australian Critical Care : Official... Mar 2024The objective of this review was to establish the learning needs and clinical requirements of postgraduate critical care nursing students preparing for clinical practice... (Review)
Review
OBJECTIVES
The objective of this review was to establish the learning needs and clinical requirements of postgraduate critical care nursing students preparing for clinical practice in rural and regional contexts.
REVIEW METHOD USED
Scoping review.
DATA SOURCES
Published and unpublished empirical studies.
REVIEW METHODS
A scoping review based on database searches (CINAHL and Medline) using Aromataris and Munn's four-step search strategy, plus subsequent forward reference search strategy was undertaken, applying predetermined selection criteria. The review aligned to the Preferred Reporting Items for Systematic Reviews and Meta-analysis Extension for Scoping Review. Studies were uploaded into Endnote 20© for storage and into Covidence 2.0© for data extraction. Screening was undertaken by a primary reviewer, with a secondary reviewer evaluating the studies identified as relevant by the first reviewer. Qualitative codes were derived, and reflexive thematic analysis synthesised the results of the review, using Braun and Clarke's six-phase process.
RESULTS
Nine foundational learning needs for critical care nursing students were extracted from the literature. The nine established foundational learning needs were: behavioural attributes/personal base; critical thinking and analysis; ethical practice; identification of risk; leadership, collaboration, and management; professional practice; provision and coordination of clinical care; research knowledge, standards of care, and policy development; and the health consumer experience. Discerning learning needs specific to rural and regional critical care nursing students was difficult. Only one study that met the inclusion criteria was identified. This study identified some instances of interest in relation to rural and regional learning needs. These instances were related to preparation of rural students for low-volume, high-stake situations; transfer of critically ill patients; stabilisation and preparation of critically ill patients; and care of specific patient groups such as, critically ill, bariatric, paediatric, obstetric, trauma, and patients with behavioural issues.
CONCLUSIONS
Limited literature exists within the rural and regional critical care nursing educational context, making it difficult to determine the unique learning needs of students within this group. This scoping review lays the groundwork for further research into the needs of critical care nursing students situated within the rural and regional context.
Topics: Female; Pregnancy; Humans; Child; Critical Illness; Critical Care Nursing; Students; Clinical Competence; Qualitative Research
PubMed: 37541909
DOI: 10.1016/j.aucc.2023.06.001 -
Open Heart Jul 2023Fabry disease (FD) is an X-linked lysosomal storage disorder caused by enzyme deficiency, leading to glycosphingolipid accumulation. Cardiac accumulation triggers local...
INTRODUCTION
Fabry disease (FD) is an X-linked lysosomal storage disorder caused by enzyme deficiency, leading to glycosphingolipid accumulation. Cardiac accumulation triggers local tissue injury, electrical instability and arrhythmia. Bradyarrhythmia and atrial fibrillation (AF) incidence are reported in up to 16% and 13%, respectively.
OBJECTIVE
We conducted a systematic review evaluating AF burden and bradycardia requiring permanent pacemaker (PPM) implantation and report any predictive risk factors identified.
METHODS
We conducted a literature search on studies in adults with FD published from inception to July 2019. Study outcomes included AF or bradycardia requiring therapy. Databases included Embase, Medline, PubMed, Web of Science, CINAHL and Cochrane. The Risk of Bias Agreement tool for Non-Randomised Studies (RoBANS) was utilised to assess bias across key areas.
RESULTS
11 studies were included, eight providing data on AF incidence or PPM implantation. Weighted estimate of event rates for AF were 12.2% and 10% for PPM. Age was associated with AF (OR 1.05-1.20 per 1-year increase in age) and a risk factor for PPM implantation (composite OR 1.03). Left ventricular hypertrophy (LVH) was associated with AF and PPM implantation.
CONCLUSION
Evidence supporting AF and bradycardia requiring pacemaker implantation is limited to single-centre studies. Incidence is variable and choice of diagnostic modality plays a role in detection rate. Predictors for AF (age, LVH and atrial dilatation) and PPM (age, LVH and PR/QRS interval) were identified but strength of association was low. Incidence of AF and PPM implantation in FD are variably reported with arrhythmia burden likely much higher than previously thought.
PROSPERO DATABASE
CRD42019132045.
Topics: Adult; Humans; Bradycardia; Atrial Fibrillation; Fabry Disease; Incidence; Pacemaker, Artificial
PubMed: 37460269
DOI: 10.1136/openhrt-2023-002316 -
Injury Epidemiology Jul 2023Preventing firearm-involved injuries is a critical public health priority. Firearm locking devices can prevent firearm injuries, such as suicide and unintentional...
BACKGROUND
Preventing firearm-involved injuries is a critical public health priority. Firearm locking devices can prevent firearm injuries, such as suicide and unintentional shootings, as well as theft. Various firearm locking devices exist; however, little is known about firearm owners' preferred locking devices for secure firearm storage. In this systematic review, we examined existing literature on preferred locking devices for secure storage of personal firearms among United States (US) firearm owners with the purpose of understanding practical implications and needs for future research.
METHODS
We searched 8 major databases, as well as the grey literature, for English-language sources published on or before January 24, 2023, that empirically examined firearm locking device preferences. Following PRISMA guidelines, coders independently screened and reviewed 797 sources using pre-determined criteria. Overall, 38 records met inclusion criteria and were included in this review.
RESULTS
The majority of studies measure and report on participant use of various types of locking devices, but few go on to measure preference between device options and the attributes and features that may contribute to an individual's preference. Included studies suggest that a preference for larger devices, such as lockboxes and gun safes, may exist among US firearm owners.
CONCLUSIONS
Review of included studies suggests that current prevention efforts may not be aligned with firearm owners' preferences. Additionally, findings from this systematic review emphasize the need for additional methodological rigorous research to understand firearm locking device preferences. Expanded knowledge in this area will result in actionable data and foundational best practices for programming that encourages behavior change concerning secure storage of personal firearms to prevent injury and death.
PubMed: 37415242
DOI: 10.1186/s40621-023-00436-7 -
Journal of Clinical Medicine Jun 2023The high demand for organs in kidney transplantation and the expansion of the donor pool have led to the widespread implementation of machine perfusion technologies. In... (Review)
Review
The high demand for organs in kidney transplantation and the expansion of the donor pool have led to the widespread implementation of machine perfusion technologies. In this study, we aim to provide an up-to-date systematic review of the developments in this expanding field over the past 10 years, with the aim of answering the question: "which perfusion technique is the most promising technique in kidney transplantation?" A systematic review of the literature related to machine perfusion in kidney transplantation was performed. The primary outcome measure was delayed graft function (DGF), and secondary outcomes included rates of rejection, graft survival, and patient survival rates after 1 year. Based on the available data, a meta-analysis was performed. The results were compared with data from static cold storage, which is still the standard of care in many centers worldwide. A total of 56 studies conducted in humans were included, and 43 studies reported outcomes of hypothermic machine perfusion (HMP), with a DGF rate of 26.4%. A meta-analysis of 16 studies showed significantly lower DGF rates in the HMP group compared to those of static cold storage (SCS). Five studies reported outcomes of hypothermic machine perfusion + O, with an overall DGF rate of 29.7%. Two studies explored normothermic machine perfusion (NMP). These were pilot studies, designed to assess the feasibility of this perfusion approach in the clinical setting. Six studies reported outcomes of normothermic regional perfusion (NRP). The overall incidence of DGF was 71.5%, as it was primarily used in uncontrolled DCD (Maastricht category I-II). Three studies comparing NRP to in situ cold perfusion showed a significantly lower rate of DGF with NRP. The systematic review and meta-analysis provide evidence that dynamic preservation strategies can improve outcomes following kidney transplantation. More recent approaches such as normothermic machine perfusion and hypothermic machine perfusion + O do show promising results but need further results from the clinical setting. This study shows that the implementation of perfusion strategies could play an important role in safely expanding the donor pool.
PubMed: 37373568
DOI: 10.3390/jcm12123871 -
PharmacoEconomics - Open Sep 2023Evidence-based guidelines on platelet transfusion therapy assist clinicians to optimize patient care, but currently do not take into account costs associated with...
BACKGROUND AND OBJECTIVE
Evidence-based guidelines on platelet transfusion therapy assist clinicians to optimize patient care, but currently do not take into account costs associated with different methods used during the preparation, storage, selection and dosing of platelets for transfusion. This systematic review aimed to summarize the available literature regarding the cost effectiveness (CE) of these methods.
METHODS
Eight databases and registries, as well as 58 grey literature sources, were searched up to 29 October 2021 for full economic evaluations comparing the CE of methods for preparation, storage, selection and dosing of allogeneic platelets intended for transfusion in adults. Incremental CE ratios, expressed as standardized cost (in 2022 EUR) per quality-adjusted life-year (QALY) or per health outcome, were synthesized narratively. Studies were critically appraised using the Philips checklist.
RESULTS
Fifteen full economic evaluations were identified. Eight investigated the costs and health consequences (transfusion-related events, bacterial and viral infections or illnesses) of pathogen reduction. The estimated incremental cost per QALY varied widely from EUR 259,614 to EUR 36,688,323. For other methods, such as pathogen testing/culturing, use of apheresis instead of whole blood-derived platelets, and storage in platelet additive solution, evidence was sparse. Overall, the quality and applicability of the included studies was limited.
CONCLUSIONS
Our findings are of interest to decision makers who consider implementing pathogen reduction. For other preparation, storage, selection and dosing methods in platelet transfusion, CE remains unclear due to insufficient and outdated evaluations. Future high-quality research is needed to expand the evidence base and increase our confidence in the findings.
PubMed: 37365482
DOI: 10.1007/s41669-023-00427-w