Search Results (23,492)

Background: Nutrition management for GSD Type I (GSDI; OMIM #232200, 232220) is complex, with the goal being to maintain euglycemia while minimizing metabolic derangements. Management guidelines were published in 2002 and 2014. However, there is limited information on the nuances of nutrition management and the unique feeding challenges of children. Methods: A REDCap survey focusing on staffing and current practices in the nutrition management of children with GSD I who were <5 years of age was sent to the metabolic dietitian’s listserv and GMDI membership in 8/2023. Results: There were 21 North American respondents. In 17/21 clinics (81%), Prosobee^® was the primary choice for infant formula. Dietitians used different methods to determine hourly glucose needs. Fasting recommendations ranged from 1 to 3 h, and the use of nighttime continuous feeding was common. Cornstarch was started between 6 and 12 months of age. Most clinics did not use Glycosade^® for children <5 years of age. Oral motor dysfunction, gagging, and lack of interest in food were common. Continuous glucose monitoring (CGM) devices were recommended in 20 clinics (95%). Most clinics followed patients on an outpatient basis. All clinics provided a hypoglycemia management plan; however, there was wide variability in practice. Conclusion: This survey highlights the variability in the care of individuals <5 years of age with GSD I. Updated guidelines are needed to help address the unique nutrition challenges in this age group. Full article

Background: Acral melanocytic lesions often pose a diagnostic and therapeutic challenge for many clinicians. Reflectance confocal microscopy (RCM) is an imaging technique widely used for the assessment of skin cancers. The aim of this review is to explore the applicability of RCM for the diagnosis of nevi and melanoma on the acral sites. Methods: Study selection was conducted based on the application of RCM for acral melanocytic lesions. All types of articles (original articles, short reports, and single case reports) were included in the analysis following PRISMA updated guidelines. Results: The search retrieved 18 papers according to the selection criteria; after removing duplicate records and additional articles by one or more of the exclusion criteria, a total of seven studies were carefully evaluated. Conclusions: RCM seems a valuable and useful additional tool for the diagnosis of acral melanocytic lesions, and its use may decrease the need for invasive procedures to some extent. Visualization of deeper layers may be achieved through mechanical removal of the superficial stratum corneum. Full article

Background: Leigh syndrome spectrum (LSS) is a novel nomenclature that encompasses both classical Leigh syndrome and Leigh-like phenotypes. Given the heterogeneity of disease presentation, a new consensus published recently addressed the main issues and proposed general guidelines towards diagnosis. Based on these recommendations, we developed a simple pipeline that can be useful in the diagnosis of LSS. Methods: We combined previously published criteria with our own experience to achieve a diagnostic framework that can provide faster satisfactory results with fewer resources. Results: We suggest adding basic biochemical tests for amino acids, acylcarnitine, and urinary organic acids as parallel investigations, as these results can be obtained in a short time. This approach characterized 80% of our cohort and promoted specific intervention in 10% of confirmed cases. Conclusions: Genetic studies are crucial in the diagnosis of LSS, but they are time-consuming and might delay tailored interventions. Therefore, we suggest adding more affordable and less complex biochemical studies as primary tests when investigating treatable causes of LSS. Full article

Background: To prevent hypoxic–ischemic brain damage in patients with post-cardiac arrest syndrome (PCAS), international guidelines have emphasized performing targeted temperature management (TTM). However, the most optimal targeted core temperature and cooling duration reached no consensus to date. This study aimed to clarify the optimal targeted core temperature and cooling duration, selected according to the time interval from collapse to return of spontaneous circulation (ROSC) in patients with PCAS due to cardiac etiology. Methods: Between 2014 and 2020, the targeted core temperature was 34 °C or 35 °C, and the cooling duration was 24 h. If the time interval from collapse to ROSC was within 20 min, we performed the 35 °C targeted core temperature (Group A), and, if not, we performed the 34 °C targeted core temperature (Group B). Between 2009 and 2013, the targeted core temperature was 34 °C, and the cooling duration was 24 or 48 h. If the interval was within 20 min, we performed the 24 h cooling duration (Group C), and, if not, we performed the 48 h cooling duration (Group D). Results: The favorable neurological outcome rates at 30 days following cardiac arrest were 45.7% and 45.5% in Groups A + B and C + D, respectively (p = 0.977). In patients with ROSC within 20 min, the favorable neurological outcome rates at 30 days following cardiac arrest were 75.6% and 86.4% in Groups A and C, respectively (p = 0.315). In patients with ROSC ≥ 21 min, the favorable neurological outcome rates at 30 days following cardiac arrest were 29.3% and 18.2% in Groups B and D, respectively (p = 0.233). Conclusions: Selecting the optimal target core temperature and the cooling duration for TTM, according to the time interval from collapse to ROSC, may be helpful in patients with PCAS due to cardiac etiology. Full article

Chronic cough in children is a common and multifaceted symptom, often requiring a comprehensive approach for accurate diagnosis and effective management. This review explores the use of spirometry, radiology (chest X-rays and computed tomography (CT) scans), and flexible bronchoscopy in the assessment of chronic cough in children through current guidelines and studies. The strengths, clinical indications, and limitations of each modality are examined. Spirometry, radiology, and in some cases flexible bronchoscopy are integral to the assessment of chronic cough in children; however, a tailored approach, leveraging the strengths of each modality and guided by clinical indications, enhances diagnostic accuracy and therapeutic outcomes of pediatric chronic cough. Full article

For millennia, springs have provided water for drinking, domestic use, balneological treatment, liminality rites as well as tourist attractions. Amidst these uses, anthropogenic activities, especially urbanization and agriculture, continue to impair the functionality of springs. With the looming decadal climate change, freshwater springs could be a sustainable source of clean water for the realisation of Sustainable Development Goal 6. This paper presents the results of the sanitary inspection and assessment of limnochemical characteristics and quality of water samples (n = 64) from four freshwater springs (coded SPR1, SPR2, SPR3, and SPR4) in Kansanga, a flash flood-prone area in the African Great Lakes region of Uganda. Each sample was analysed for 17 parameters (temperature, pH, electrical conductivity, turbidity, fluorides, sulphates, chlorides, nitrates, orthophosphates, total dissolved solids, dissolved oxygen, total alkalinity, potassium, sodium, total, magnesium and calcium hardness) following the standard methods. Water quality index (WQI) was calculated to establish the quality of the water samples based on the physicochemical parameters measured. Based on the sanitary risk assessment results, the springs had medium- to high-risk scores, but most water parameters were within the WHO guidelines for potable water, except for nitrates (in SPR1 and SPR2), hardness levels (in SPR2), and dissolved oxygen (in all the samples). Sampling season and location had significant effects on the limnochemistry of the freshwater springs (p < 0.05). The water quality indices calculated indicated that the water from the springs was of good quality (WQI = 50–57), but there was a reduction in water quality during the wet season. The best water quality was recorded in samples from SPR4, followed by those for SPR3, SPR1, and SPR2. These results provide insights into the contribution of floods and poor sanitation facilities to the deterioration of spring water quality in Kansanga, and the need to leverage additional conservation strategies to support vulnerable communities in the area. Further studies are required to establish the risk posed by trace metals and microbes that may contaminate freshwater in the studied springs, especially following flood events. Full article

Background: Inflammatory bowel disease (IBD) is characterized by persistent inflammation and is often associated with metabolic dysfunction-associated steatotic liver disease (MASLD). IBD patients are at risk of developing MASLD due to shared risk factors such as gut dysbiosis and systemic inflammation. The new MASLD nomenclature emphasizes the link between liver steatosis and cardiometabolic comorbidities. However, the prevalence of MASLD in IBD patients remains poorly explored. The main aim of this cross-sectional study is to assess the prevalence of ultrasound (US) and the clinical features of MASLD in patients with IBDs. Materials and Methods: We conducted a retrospective study enrolling 272 Italian IBD patients attending Renato Dulbecco Teaching Hospital in a period between 1 January 2021 and 31 December 2023. MASLD was diagnosed based on the presence of liver steatosis with cardiometabolic risk factors, using established guidelines. Demographic, clinical, and laboratory data were collected and analyzed. Statistical significance was determined at a p-value < 0.05. Results: Of the 272 IBD patients, 6% had non-alcoholic fatty liver disease (NAFLD), while 18% had MASLD. Patients with IBD-MASLD were significantly older, had higher body mass index, waist circumference, and triglyceride levels, and were more likely to have type 2 diabetes mellitus and hypertension compared to those with IBD-NAFLD. IBD-MASLD patients also showed higher disease activity scores and required more frequent surgical interventions. Bivariate logistic regression revealed triglyceride levels as a significant predictor of MASLD in IBD patients. Conclusions: MASLD is more prevalent in IBD patients, highlighting the importance of early detection of liver steatosis in this at-risk population. The association between MASLD and cardiometabolic risk factors underscores the need for a multidisciplinary approach to manage these patients effectively. Further studies in larger cohorts are necessary to confirm these findings and explore the pathophysiological mechanisms involved. Full article

Background: Compound fractures of the distal tibia (with or without the middle third) represent a challenge for orthopedic and plastic surgeons because of the scarcity of available soft tissue reconstruction and the important comminution of the fractures that usually appear. Methods: The design of this study is based on the PRISMA guidelines. Databases were searched for articles published and available until the first half of 2023. Articles that presented the evolution of patients treated by combining circular external fixators with reconstructive methods were selected. Results: After searching the literature using keywords, we obtained 3355 articles, out of which 14 articles met all the inclusion criteria, with a total number of participants of 283. The bone loss varied between 0.7 and 18.2 cm, while the soft tissue defect was between 3/3 cm and 16/21 cm. The average period of fixation ranged from 4 to 22.74 months. The most used reconstruction methods were 80 free flaps and 73 pedicled flaps out of 249 interventions. Complete flap loss appeared only in 3/283 patients. Regarding the bone union, the percentage of non-union was low, and in all cases, it was achieved after reintervention. A low rate of major complications was observed. Conclusions: The orthoplastic team is the key to successfully treating the high-energy traumatism of the distal tibia (with or without a middle third). The Ilizarov external fixator can be used as a definitive limb-salvage treatment (secondary to the standard primary methods of fixation) in combination with a flap to cover the defects because it does not damage the pedicle, and it helps stabilize the soft tissues and bones around the flap to lower the complications. Full article

Introduction: Polymethylmethacrylate (PMMA) is the most widely used denture base material due to its favourable properties. Several studies have tested the incorporation of anti-infective agents into PMMA as a strategy to prevent biofilm growth on the denture surface. This systematic review aims to evaluate the efficacy of incorporating inorganic antimicrobial particles into denture base resins in preventing antimicrobial growth, thereby identifying the most effective agents for enhancing PMMA’s antimicrobial properties. Materials and methods: This systematic review followed the PRISMA guidelines, and the research protocol was registered in PROSPERO. The search was performed by using Medical Subject Headings and free text combined with Boolean operators in PubMed/Medline^® and in Cochrane^® and a free text combination in Web of Science^® Core Collection. Data regarding the inorganic particles studied, their antimicrobial effect, and the type of samples produced were collected and analysed. Results: After screening, a total of fifteen studies were included in this review. Most samples were disk-shaped and of varying sizes, and the most tested microbial strain was Candida albicans. Silver was the most used antimicrobial particle, followed by gold, titanium, and copper. Conclusions: Overall, incorporating inorganic particles into PMMA has produced promising antimicrobial results, depending on the concentration. Due to the high heterogeneity observed in the samples, more studies are recommended, particularly clinical trials. Full article

Virtual reality (VR) technology has made remarkable progress in recent years and will be widely used in the future. As a bridge for information exchanges between users and VR systems, the interaction interface is pivotal for providing users with a good experience and has emerged as a key research focus. In this review, we conducted a comprehensive search of the Web of Science and CNKI databases from 2011 to 2023 to identify articles dedicated to VR interaction interface design. Through a meticulous analysis of 438 articles, this paper offers a substantial contribution to the emerging field of VR interactive interface research, providing an in-depth review of the principal research advancements. This review revealed that the majority of studies are centered on practical case analyses within specific application scenarios, employing empirical evaluation methods to assess objective or subjective metrics. We then concentrated on elucidating the foundational principles of interface design and their evaluation methodologies, providing a reference for future research endeavors. Additionally, the limitations, challenges, and future directions in VR interaction interface design research were discussed, highlighting the need for further research in design evaluation to continuously refine the development of standards and guidelines for VR interactive interface design. According to the findings of this review, there is a necessity to enhance research on information design for multi-channel interactive interfaces. Furthermore, it is essential to focus on the diverse characteristics of users to propose more inclusive design solutions. Adopting interdisciplinary approaches could lead to breakthroughs in the creation of personalized and adaptive VR interaction interfaces. Full article

Background: This review focuses on the prognostic role of resting-state functional magnetic resonance imaging (fMRI) in disorders of consciousness (DOCs). Several studies were conducted to determine the diagnostic accuracy in DOC patients to identify prognostic markers and to understand the neural correlates of consciousness. A correct diagnosis of consciousness in unresponsive or minimally responsive patients is important for prognostic and therapeutic management. Functional connectivity is considered as an important tool for the formulation of cerebral networks; it takes into account the primary sensorimotor, language, visual and central executive areas, where fMRI studies show damage in brain connectivity in the areas of frontoparietal networks in DOC patients. Methods: The integration of neuroimaging or neurophysiological methods could improve our knowledge of the neural correlates of clinical response after an acquired brain injury. The use of MRI is widely reported in the literature in different neurological diseases. In particular, fMRI is the most widely used brain-imaging technique to investigate the neural mechanisms underlying cognition and motor function. We carried out a detailed literature search following the relevant guidelines (PRISMA), where we collected data and results on patients with disorders of consciousness from the studies performed. Results: In this review, 12 studies were selected, which showed the importance of the prognostic role of fMRI for DOCs. Conclusions: Currently there are still few studies on this topic. Future studies using fMRI are to be considered an added value for the prognosis and management of DOCs. Full article

Interferon-gamma release assays (IGRAs) have gained attention for the diagnosis of latent tuberculosis infection (LTBI) due to their higher specificity compared to the tuberculin skin test (TST). However, the IGRA’s performance varies across different populations. This study evaluated the diagnostic performance of three IGRAs (TBF-FIA, TBF-ELISA, and QFT-Plus) in Ghana, comparing them among individuals exposed and unexposed to MTB infection. Conducted in TB clinics across three regions, this prospective and cross-sectional study included healthy individuals with no known TB exposure (unexposed group) and patients with confirmed active TB (exposed group). Blood samples were tested using all three assays as per the manufacturers’ guidelines. The TBF-ELISA showed 3.4% higher sensitivity but 4.6% lower specificity compared to QFT-Plus. The TBF-FIA had sensitivity of 78.5–87.3% and specificity of 82.9–90.0%. These findings indicate that while the three IGRAs offer similar diagnostic accuracy, the variations in specificity and limited data on assays like TBF-FIA require further investigation. Full article

The goal of this study was to examine the predictors of sustainable tourism development during the post-COVID-19 pandemic period in the context of Bangladesh. Quantitative-type research was applied, and this study used a descriptive research design. A standardized questionnaire was used to collect 302 data points from actual indigenous tourists using an online purposive sampling method. A partial least squares structural equation modeling (PLS-SEM) approach was used to evaluate the data and test the hypotheses. The results of the PLS-SEM analysis method demonstrate that environmental integrity, social equity, economic prosperity, and technological adaption had a positive relationship with sustainable tourism development during the post-COVID-19 pandemic period in Bangladesh. This research article provides practical guidelines for tourism authorities (BTB, BPC, and so on) and stakeholders on how to effectively impact environmental integrity, social equity, economic prosperity, and technological adaption on sustainable tourism development during the post-COVID-19 pandemic period in the context of Bangladesh. This study recommends that influential strategic factors be utilized to develop a sustainable tourism business in Bangladesh, positioning it as an emerging tourism destination. Full article

Background and Objectives: Adrenal insufficiency (AI) can be a significant concern in patients with transfusion-dependent homozygous beta thalassemia (bThal) due to the chronic disease burden and frequent blood transfusions that these patients require. The prevalence of AI in this population remains unclear, with studies often lacking control groups for comparison. This meta-analysis aimed to estimate the proportion of patients with transfusion-dependent bThal who exhibit evidence of AI. Materials and Methods: A systematic review following PRISMA guidelines identified 19 studies for analysis. Results: Despite the variability in the diagnostic methods used to ascertain AI, the meta-analysis revealed that approximately one-third of patients had evidence of AI, with the prevalence rising to 50% in studies focused on adults with bThal. Conclusions: These findings suggest an increased risk of AI in patients with bThal compared to the general population. Clinicians should consider tailored management strategies, including glucocorticoid coverage during surgical procedures, to mitigate the risk of adrenal crises in this vulnerable patient group. Further research is needed to optimize adrenal surveillance and management in patients with bThal. Full article

Baiyangdian Lake, recognized as the largest freshwater body in northern China, plays a vital role in maintaining the regional eco-environment. Prior studies have pointed out the contamination of sediments with heavy metals, raising concerns about eco-environmental challenges. Therefore, it is imperative to evaluate the current pollution levels and ecological threats related to heavy metals found in the sediments of Baiyangdian Lake as well as in its inflow rivers. In May 2022, surface sediments with a depth of less than 20 cm were analyzed for Cu, Zn, Pb, Cr, Ni, As, Cd, and Hg to determine the pollution status, identify sources of pollution, and evaluate potential ecological risks. A range of evaluation methods used by predecessors such as geo-accumulation index (I_geo), enrichment factor (EF), ecological risk index (RI), sediment quality guidelines (SQGs), positive matrix factorization (PMF), absolute principal component score-multiple linear regression model (APCS-MLR), chemical mass balance (CMB), and UNMIX model were analyzed. After comparison, multi-methods including the geo-accumulation index (Igeo), absolute principal component score-multiple linear regression model (APCS-MLR), ecological risk index (RI), and sediment quality guidelines (SQGs) were utilized this time, leading to a better result. Findings reveal that pollution levels are generally low or non-existent, with only 1.64% of sampling sites showing close to moderate pollution levels for Cu, Pb, and Zn, and 4.92% and 1.64% of sites exhibiting close to moderate and moderate pollution levels for Cd, respectively. The main contributors to heavy metal presence are pinpointed as industrial wastewater discharge, particularly Cu, Zn, Pb, Cd, and Hg. The ecological risks are also relatively low, with 4.92%, 1.64%, and 1.64% of sampling sites demonstrating close to moderate, moderate, and strong risks in the inflow rivers, respectively. Additionally, only one site shows moderate potential biological toxicity, while the rest display non-toxicity. These findings will update our cognition and offer a scientific basis for pollution treatment and ecosystem enhancement for government management. Full article