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Orphanet Journal of Rare Diseases Apr 2024Microtia is a congenital ear malformation that can occur as isolated microtia or as part of a syndrome. The etiology is currently poorly understood, although there is...
BACKGROUND
Microtia is a congenital ear malformation that can occur as isolated microtia or as part of a syndrome. The etiology is currently poorly understood, although there is strong evidence that genetics has a role in the occurrence of microtia. This systematic review aimed to determine the genes involved and the abnormalities in microtia patients' head and neck regions.
METHODS
We used seven search engines to search all known literature on the genetic and phenotypic variables associated with the development or outcome of microtia. The identified publications were screened and selected based on inclusion and exclusion criteria and assessed for methodological quality using the Joanna Briggs Institute (JBI) critical appraisal tools. We found 40 papers in this systematic review with phenotypic data in microtia involving 1459 patients and 30 articles containing genetic data involved in microtia.
RESULT
The most common accompanying phenotype of all microtia patients was external ear canal atresia, while the most common head and neck abnormalities were the auricular, mental, and oral regions. The most common syndrome found was craniofacial microsomia syndrome. In the syndromic microtia group, the most common genes were TCOF1 (43.75%), SIX2 (4.69%), and HSPA9 (4.69%), while in the non-syndromic microtia group, the most frequently found gene was GSC exon 2 (25%), FANCB (16.67%), HOXA2 (8.33%), GSC exon 3 (8.33%), MARS1 (8.33%), and CDT1 (8.33%).
CONCLUSIONS
Our systematic review shows some genes involved in the microtia development, including TCOF1, SIX2, HSPA9, GSC exon 2, FANCB, HOXA2, GSC exon 3, MARS1, and CDT1 genes. We also reveal a genotype-phenotype association in microtia. In addition, further studies with more complete and comprehensive data are needed, including patients with complete data on syndromes, phenotypes, and genotypes.
Topics: Humans; Congenital Microtia; Homeodomain Proteins; Ear; Phenotype; Syndrome; Genetic Association Studies
PubMed: 38594752
DOI: 10.1186/s13023-024-03142-9 -
PloS One 2024Basic Life Support (BLS) is a sequence of care provided to patients who are experiencing respiratory arrest, cardiac arrest, or airway obstruction. Its main purpose is... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Basic Life Support (BLS) is a sequence of care provided to patients who are experiencing respiratory arrest, cardiac arrest, or airway obstruction. Its main purpose is to maintain the airway, breathing, and circulation through CPR. This review aimed to estimate the pooled prevalence of Health Professionals' knowledge and practice on basic life support in Ethiopia.
METHOD
Eligible primary studies were accessed from international database (PubMed, Google Scholar, Hinari databases) and grey literatures found in online repositories. The required data were extracted from those studies and exported to Stata 17 for analysis. A weighted inverse-variance random-effects model and Der Simonian-Laird estimation method were used to compute the overall pooled prevalence of Health Professional's knowledge, practice of basic life support and its predictors. Variations across the included studies were checked using forest plot, funnel plot, I2 statistics, and Egger's test.
RESULT
A total of 5,258 Health Professionals were included from 11 studies. The pooled prevalence of knowledge and practice outcomes on basic life support in Ethiopia were 47.6 (95% CI: 29.899, 65.300, I2: 99.21%) and 44.42 (95% CI: 16.42, 72.41, I2: 99.69) respectively. Educational status of the Professional's was significantly associated with knowledge outcome. Those who had degree and above were 1.9 times (AOR: 1.90 (1.24, 2.56)) more likely knowledgeable on basic life support than under degree.
CONCLUSION
The overall pooled estimates of Health Professionals knowledge and practice on basic life support was considerably low. The educational status of the Health Professionals was significantly associated with knowledge outcome. The Health Professionals and responsible stakeholders should focus on the basic life support at Health Institutions. The professionals should advance their knowledge and skill on basic life support for the patients.
Topics: Humans; Ethiopia; Health Personnel; Prevalence
PubMed: 38593136
DOI: 10.1371/journal.pone.0297430 -
Cureus Apr 2024Childhood pneumonia is a major contributor to illness and death in children under the age of five globally. Despite advancements in medical science, the burden of... (Review)
Review
Childhood pneumonia is a major contributor to illness and death in children under the age of five globally. Despite advancements in medical science, the burden of pediatric community-acquired pneumonia (CAP) remains high, particularly in low- and middle-income countries. This systematic review aims to synthesize existing literature on the prevalence, risk factors, and healthcare-seeking behaviors associated with pediatric CAP to inform the development of targeted community-based interventions. An extensive search of various databases such as Medline, EMBASE, Web of Science, Cochrane, PubMed, PubMed Central, Helinet, SpringerLink, Google Scholar, and Biomed Central was performed, resulting in 65 potentially relevant studies. After a thorough evaluation process, 25 studies were selected for the final analysis. These selected studies offered valuable information on the epidemiology, risk factors, and healthcare-seeking behaviors associated with childhood pneumonia. The review revealed that environmental factors such as indoor air pollution, overcrowding, and exposure to tobacco smoke are significant risk factors for pediatric pneumonia. Additionally, socioeconomic factors, including poverty and a lack of access to clean water and sanitation, contribute to the vulnerability of children to this disease. Poor healthcare-seeking behaviors, driven by limited knowledge and awareness of pneumonia symptoms and treatment, further exacerbate the situation. The review also highlighted the critical role of vaccination, particularly against type b (Hib) and pneumococcus, in preventing pneumonia. However, gaps in vaccination coverage and challenges in accessing healthcare services remain barriers to effective pneumonia control. In light of these findings, the review recommends the implementation of community-based interventions that address the multifaceted determinants of pediatric pneumonia. These interventions should focus on improving environmental conditions, enhancing access to preventive measures such as vaccination, and promoting better healthcare-seeking behaviors through education and awareness campaigns. It is essential for healthcare providers, policymakers, and community members to collaborate in developing and implementing culturally appropriate and sustainable interventions. This cooperation aims to lessen the impact of pneumonia on children and their families.
PubMed: 38586234
DOI: 10.7759/cureus.57636 -
Frontiers in Pediatrics 2024Asthma is a common chronic respiratory disease in children. Alongside pharmacological interventions, inspiratory muscle training (IMT) emerges as a complementary...
BACKGROUND
Asthma is a common chronic respiratory disease in children. Alongside pharmacological interventions, inspiratory muscle training (IMT) emerges as a complementary therapeutic approach for asthma management. However, the extent of its efficacy in pediatric populations remains uncertain when compared to its benefits in adults. This systematic review aims to evaluate the effectiveness of IMT with threshold loading in children with asthma.
METHODS
Randomized controlled trials (RCTs) evaluating the efficacy of inspiratory muscle training in pediatric asthma patients were identified through June 2023 across various literature databases, including PubMed, Embase, Cochrane Central Register of Controlled Trials (CENTRAL), Cumulative Index to Nursing and Allied Health Literature (CINAL), Web of Science, China Knowledge Resource Integrated Database (CNKI), Wei Pu Database, Wan Fang Database, and Chinese Biomedical Database (CBM). These trials compared inspiratory muscle training against sham inspiratory muscle training and conventional care. Eligible studies were assessed in terms of risk of bias and quality of evidence. Where feasible, data were pooled and subjected to meta-analysis, with results reported as mean differences (MDs) and 95% confidence intervals (CIs).
RESULTS
Six trials involving 333 patients were included in the analysis. IMT demonstrated significant improvements in maximum inspiratory pressure (MIP) (MD 25.36, 95% CI 2.47-48.26, = 0.03), maximum expiratory pressure (MEP) (MD 14.72, 95% CI 4.21-25.24, = 0.006), forced vital capacity in percent predicted values [FVC(% pred)] (MD 3.90, 95% CI 1.86-5.93, = 0.0002), forced expiratory volume in the first second in percent predicted values [FEV(% pred)] (MD 4.96, 95% CI 2.60-7.32, < 0.0001), ratio of forced expiratory volume in 1 s to forced vital capacity (FEV/FVC) (MD 4.94, 95% CI 2.66-7.21, < 0.0001), and asthma control test (ACT) (MD = 1.86, 95% CI: 0.96-2.75, < 0.0001).
CONCLUSIONS
Findings from randomized controlled trials indicate that inspiratory muscle training enhances respiratory muscle strength and pulmonary function in pediatric asthma patients.
SYSTEMATIC REVIEW REGISTRATION
www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42023449918, identifier: CRD42023449918.
PubMed: 38562138
DOI: 10.3389/fped.2024.1367710 -
EClinicalMedicine May 2024Aggressive care near patients' end-of-life (EOL) entails limited therapeutic values, high costs, and compromised quality of life (QoL). In this study, we aimed to...
BACKGROUND
Aggressive care near patients' end-of-life (EOL) entails limited therapeutic values, high costs, and compromised quality of life (QoL). In this study, we aimed to estimate the global prevalence of aggressive care in patients with cancer and explore potential subgroup differences.
METHODS
We searched PubMed, Embase, and the Cochrane Library from database inception to Feb 16, 2024. Eligible studies reported the prevalence of aggressive EOL care using at least one quantifiable measure. Random-effects models were used to derive the pooled prevalence and subgroup analyses were performed to investigate differences in the prevalence of aggressive care across regions, the country's level of economic development, tumor types, ages, and sample sizes. This review is registered with PROSPERO, number CRD42023467839.
FINDINGS
A total of 129 studies were included in this systematic review, of which 118 (91.5%) were from high-income countries. Studies were mostly conducted in the Americas (60, 46.5%), Europe (34, 26.4%), and Western Pacific (31, 24.0%). Measures of aggressive care were inconsistent across studies, with the most commonly used measure being the use of chemotherapy in the last 14 days of life (DOLs) (n = 87, 67.4%) and intensive care unit (ICU) stay in the last 30 DOLs (n = 87, 67.4%). The prevalence of the five claims-based measures of aggressive care, i.e., chemotherapy in the last 14 DOLs, ICU stay in the last 30 DOLs, repeated hospital admission in the last 30 DOLs, repeated emergency room (ER) visit in the last 30 DOLs, and hospice care <3 days before death were 11.6% (95% CI, 9.8%-13.4%), 14.4% (95% CI, 11.8%-17.0%), 17.9% (95% CI, 14.4%-21.4%), 14.8% (95% CI, 12.0%-17.6%), and 14.4% (95% CI, 11.2%-17.6%), respectively. Regional differences were statistically significant in the prevalence of ICU stay and repeated hospital admission in the last 30 DOLs (p < 0.01; p = 0.03). Patients with hematologic malignancies were more likely to receive aggressive care than those with solid tumors, as seen in their higher rates of chemotherapy in the last 14 DOLs (21.7% versus 11.6%; p = 0.03), ICU stay in the last 30 DOLs (25.5% versus 10.8%; p < 0.01), and hospice care <3 days before death (26.7% versus 14.2%; p < 0.01). In addition, the prevalence of chemotherapy in the last 14 DOLs (26.2%; p < 0.01) and repeated hospital admission in the last 30 DOLs (31.4%; p < 0.01) were highest among pediatric patients with cancer.
INTERPRETATION
This meta-analysis found that aggressive EOL care was common in patients with cancer, regardless of the definition used, and varied by regions and populations. It is necessary to be aware of the global burden of aggressive care for patients with cancer near their EOL and take prompt action to address it.
FUNDING
National Natural Science Foundation of China (Grant No. 72274004).
PubMed: 38549585
DOI: 10.1016/j.eclinm.2024.102561 -
Journal of Pediatric... 2024Pediatric oncology patients receive multiple modalities of therapy to treat their malignancies. These modalities have the potential for acute toxicity and late effects.... (Review)
Review
Evidence-Based Recommendations for Education Provided to Patients and Families Regarding the Adverse Events of ALK and MEK Inhibitors: A Systematic Review From the Children's Oncology Group.
Pediatric oncology patients receive multiple modalities of therapy to treat their malignancies. These modalities have the potential for acute toxicity and late effects. In the last decade, a new modality known as targeted biological therapy, has become an integral part of treatment for pediatric cancers. As targeted therapy use has increased, adverse events specific to these targeted agents have emerged, requiring a new effort focused on providing education to patients and families regarding how best to report, monitor, and manage these adverse events. A clinical question was developed to guide the systematic literature review. Anaplastic lymphoma kinase (ALK) and mitogen-activated protein kinase kinase (MEK) inhibitors were selected for review due to their frequency of use in pediatric oncology. The search was conducted to identify relevant articles published between January 1, 2000 and May 5, 2020. Articles were screened by two team members for inclusion/exclusion criteria using the web-based systematic review tool, Rayyan. Twenty-seven articles met the eligibility criteria for inclusion and were evaluated using the Grading of Recommendations, Assessment, Development, and Evaluation criteria. Adverse events for ALK and MEK inhibitors included manifestations of the gastrointestinal, hematologic, dermatologic, musculoskeletal, neurological, cardiovascular, and ocular systems. Recommendations for patient/family education were made for ALK and MEK inhibitors based on the reported adverse events. Adverse events of ALK and MEK inhibitors differ from the more common adverse events experienced with conventional treatment modalities used in pediatric oncology. It is important for nurses to include information regarding potential adverse events in patient/family education for children receiving these targeted agents.
Topics: Child; Humans; Anaplastic Lymphoma Kinase; Protein Kinase Inhibitors; Antineoplastic Agents; Neoplasms; Mitogen-Activated Protein Kinase Kinases
PubMed: 38549368
DOI: 10.1177/27527530231206101 -
Cancer Nursing Mar 2024Interactive features of computerized cognitive training (CCT) may enhance adherence to training, providing a relatively low-cost intervention. A robust systematic review...
BACKGROUND
Interactive features of computerized cognitive training (CCT) may enhance adherence to training, providing a relatively low-cost intervention. A robust systematic review on the effectiveness of CCT for improving working memory (WM) among pediatric survivors with cancer is lacking.
OBJECTIVE
To summarize the available evidence and determine the effectiveness of CCT for WM among pediatric survivors with cancer.
INTERVENTIONS/METHODS
Five databases were searched. The Effective Public Health Practice Project was used to assess the study quality. ReviewerManager was used. The primary outcome was WM performance. Secondary outcomes included processing speed, attention, intervention adherence, and number of adverse events.
RESULTS
Six studies were included. Regarding overall quality, 1 study was weak, and 5 studies were moderate. Five studies reported a significant improvement of WM postintervention (P < .05). The meta-analysis of Cogmed interventions on symbolic WM revealed a significant difference between groups (vs placebo), with an overall pooled effect size of 0.71 (95% confidence interval, 0.02-1.41; P = .04). Two and 4 studies investigated the effects of CCT on processing speed and attention, respectively, with conflicting results. Four studies reported adherence of 80% or greater. Two studies reported no adverse events.
CONCLUSIONS
Computerized cognitive training using Cogmed has a significant positive effect on WM. The effects of CCT on processing speed and attention remain inconclusive.
IMPLICATIONS FOR PRACTICE
More rigorous trials should be conducted to elucidate the cognitive effects of CCT, particularly processing speed and attention, in the pediatric population with cancer. Further studies should consider combining CCT with other existing interventions to strengthen their effectiveness.
PubMed: 38527112
DOI: 10.1097/NCC.0000000000001348 -
Frontiers in Immunology 2024Inflammatory bowel disease (IBD) poses a growing global burden, necessitating the discovery of reliable biomarkers for early diagnosis. The clinical significance of... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Inflammatory bowel disease (IBD) poses a growing global burden, necessitating the discovery of reliable biomarkers for early diagnosis. The clinical significance of dysregulated expression of long noncoding RNAs (lncRNAs) and circular RNAs (circRNAs) in diagnosing IBD has not been well established. Thus, our study aimed to investigate the diagnostic value of lncRNAs and circRNAs for IBD based on currently available studies.
METHODS
A comprehensive search was carried out in diverse electronic databases, such as PubMed, Embase, Scopus, Science Direct and Wiley Online Library to retrieve articles published until October 30, 2023. Stata 17.0 software was employed to determine pooled sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), diagnostic ratio (DOR), and area under the curve (AUC). Heterogeneity, subgroup analysis, and meta-regression were explored, and publication bias was assessed using Deeks' funnel plot. Fagan's nomogram and likelihood ratio scattergram were employed to evaluate the clinical validity.
RESULT
A total of 11 articles encompassing 21 studies which involved 1239 IBD patients and 985 healthy controls were investigated. The findings revealed lncRNAs exhibit high level of pooled sensitivity 0.94 (95% CI: 0.87-0.97) and specificity 0.99 (95% CI: 0.89-1.00), along with PLR, NLR, DOR, and AUC values of 64.25 (95% CI: 7.39-558.66), 0.06 (95% CI: 0.03-0.13), 1055.25 (95% CI: 70.61-15770.77), and 0.99 (95% CI: 0.97-0.99), respectively. Conversely, CircRNAs showed moderate accuracy in IBD diagnosis, with sensitivity of 0.68 (95% CI: 0.61-0.73), specificity of 0.73 (95% CI: 0.65-0.79), PLR of 2.47 (95% CI: 1.94-3.16), NLR of 0.45 (95% CI: 0.38-0.53), DOR of 5.54 (95% CI: 3.88-7.93), and AUC value of 0.75 (95% CI: 0.71-0.79). Moreover, findings from subgroup analysis depicted heightened diagnostic efficacy when employing lncRNA H19 and a large sample size (≥100), with notable efficacy in diagnosing both ulcerative colitis (UC) and Crohn's disease (CD).
CONCLUSION
LncRNAs exhibit high diagnostic accuracy in distinguishing patients with IBD from healthy controls signifying their possible use as potential biomarkers, while circRNAs showed moderate diagnostic accuracy. Nevertheless, to validate our findings and confirm the clinical utility of lncRNAs and circRNAs in IBD diagnosis, a large pool of prospective and multi-center studies should be undertaken.
SYSTEMATIC REVIEW REGISTRATION
https://www.crd.york.ac.uk/PROSPERO, identifier CRD42023491840.
Topics: Humans; RNA, Circular; RNA, Long Noncoding; Biomarkers, Tumor; Prospective Studies; Inflammatory Bowel Diseases
PubMed: 38524131
DOI: 10.3389/fimmu.2024.1362437 -
Current Drug Research Reviews Mar 2024Pancreatic neoplasm is one of the types of cancer with a high incidence and case-fatality rate. (Meta-Analysis)
Meta-Analysis
BACKGROUND
Pancreatic neoplasm is one of the types of cancer with a high incidence and case-fatality rate.
OBJECTIVES
This study was designed to investigate the relationship between statin intake and the risk of pancreatic cancer with a systematic review and meta-analysis approach.
METHODS
This study was a systematic review and meta-analysis of studies published before 2023 in Cochrane Library, Web of Science (WOS), PubMed, Google Scholar, ScienceDirect, Scopus, and Embase databases. The statistical analyses were conducted using Stata software, version 15. The significance level for this study was set at 0.05.
RESULTS
This meta-analysis included 32 studies and a total of 5,849,814 participants. The risk ratio (RR) of pancreatic cancer in comparison to the non-statin receiving group in statin users in total was equal to 0.75 (95% CI: 0.66-0.86, p-value <0.001), in the cohort studies was obtained to be 0.70 (0.53-0.93), in the randomized clinical trials (RCTs) had a ratio of 0.99 (0.53-1.86), while studies conducted in American countries had a ratio of 0.69 (0.51-0.93), studies in Asian countries had a ratio of 0.73 (0.56-0.97), and studies in European countries had a ratio of 0.88 (0.76-1.02). Furthermore, the study did not detect any signs of publication bias.
CONCLUSION
The study findings suggest a potential connection between using statins and a lower risk of pancreatic cancer. However, it is important to note that controlled clinical trials did not find a statistically significant association between taking statins and the development of pancreatic cancer. Therefore, it is advisable to exercise caution when interpreting the results of this study.
PubMed: 38523536
DOI: 10.2174/0125899775281869240311043637 -
The Lancet. Child & Adolescent Health May 2024Infants born to mothers with tuberculosis disease are at increased risk of developing tuberculosis disease themselves. We reviewed published studies and guidelines on... (Review)
Review
Infants born to mothers with tuberculosis disease are at increased risk of developing tuberculosis disease themselves. We reviewed published studies and guidelines on the management of these infants to inform the development of a consensus practice guideline. We searched MEDLINE, CINAHL, and Cochrane Library from database inception to Dec 1, 2022, for original studies reporting the management and outcome of infants born to mothers with tuberculosis. Of the 521 published papers identified, only three met inclusion criteria and no evidence-based conclusions could be drawn from these studies, given their narrow scope, variable aims, descriptive nature, inconsistent data collection, and high attrition rates. We also assessed a collection of national and international guidelines to inform a consensus practice guideline developed by an international panel of experts from different epidemiological contexts. The 16 guidelines reviewed had consistent features to inform the expert consultation process. Two management algorithms were developed-one for infants born to mothers considered potentially infectious at the time of delivery and another for mothers not considered infectious at the time of delivery-with different guidance for high and low tuberculosis incidence settings. This systematic review and consensus practice guideline should facilitate more consistent clinical management, support the collection of better data, and encourage the development of more studies to improve evidence-based care.
Topics: Infant; Female; Humans; Mothers; Tuberculosis; Consensus
PubMed: 38522446
DOI: 10.1016/S2352-4642(23)00345-0