bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clubfoot: Review on Assessment, Treatment, Challenges, and Engineering Aspects
Authors: Singh Shamsher , Mali Harlal Singh 
Published: April 2022

ABSTRACT

Introduction
Clubfoot, mainly CTEV, is a complex three-dimensional deformity challenging physicians from ancient times. Among a thousand babies born, one to six suffer from clubfoot deformity; that is, in India alone, around 35,000 need treatment for clubfoot annually. This review summarizes the various practices of clubfoot assessment, noninvasive treatment, and engineering aspects of clubfoot treatment. The shortcomings of assessment methods and challenges with implementation of treatment methods are also presented.

Methods
A narrative review of all related research papers available to authors was carried out.

Results
Various clubfoot assessment methods have been developed to help physicians understand the severity of the problem and predict treatment parameters. The Pirani score and Dimeglio score are used predominantly for the assessment of deformity. However, these methods suffer from implementation-related limitations. Noninvasive methods, namely, Kite's method, functional physiotherapy method, and Ponseti method, are used in practice for treatment. The Ponseti method is the most popular with parents and physicians and is considered the gold standard for the treatment of clubfoot. However, it also suffers from various implementation issues, like treatment cost and lack of experts, particularly in low- and middle-income countries (LMICs). Engineers are using various technologies like computer-aided design (CAD)/computer-aided engineering (CAE) and additive manufacturing for modeling and analysis of clubfoot. Engineers have also attempted to develop corrective and maintenance orthosis for CTEV treatment.

Conclusions
Because newer and newer technologies are becoming accessible for interdisciplinary use, there is a need to apply contemporary technologies, especially to develop a corrective orthosis so that the current challenges of clubfoot assessment and treatment are addressed. The corrective orthosis should be based on Dr Ponseti's insights into clubfoot biomechanics.

Clinical Relevance
There is a need to explore contemporary technologies like data acquisition and CAD/CAE to address CTEV assessment–related issues. A corrective orthosis based on the understanding of the foot biomechanics for treatment can solve the various implementation-related challenges of the Ponseti method, particularly in low-resource settings.
Construction and validation of a robust ferroptosis-associated gene signature predictive of prognosis in lung adenocarcinoma
Authors: Zhou Mi , Zhu Xin 
Published: April 2022

Abstract
To construct and validate a ferroptosis-associated signature predictive of prognosis in lung adenocarcinoma (LUAD), and systematically evaluate the underlying molecular connections in cancer biology.
We retrieved mRNAs sequencing profiles of LUAD from the cancer genome atlas (TCGA) data portal and clinical information from the cBio Cancer Genomics Portal. The differentially expressed ferroptosis-associated genes (DEFAGs) were screened between normal samples and LUAD by packages “limma” in R. Then the total TCGA cohort was randomly divided into training set and testing set. Based on the training set, a DEFAG signature was built and further validated in the test set, the total TCGA cohort and other independent cohorts from the gene expression omnibus data portal. A nomogram was constructed and validated, and the correlation between high-risk group and cancer biology was further evaluated.
We initially identified 68 DEFAGs from TCGA cohort. A 6 DEFAG signature was built and further validated in the test set, the total TCGA cohort and other 2 independent cohorts including GSE31210 and GSE72094 from gene expression omnibus data portal. Further exploration indicated that high-risk group combined with TP53 mutation harbored the most unfavorable prognosis while low-risk group with TP53 wild-type status had the most favorable survival advantage over other groups. Moreover, high-risk group was associated with higher cancer stemness, tumor mutation burden, and CD274 (programmed cell death 1 ligand 1) expression.
We constructed a robust ferroptosis-associated gene signature and a nomogram predictive of prognosis in LUAD, and provided a new perspective on associations between ferroptosis and cancer.
Correlation between polymorphism of vitamin D receptor TaqI and susceptibility to tuberculosis
Authors: Li Bin , Wen Fei , Wang Zhaofen 
Published: April 2022

Abstract

Background:
To investigate the association between TaqI polymorphism of the vitamin D receptor gene and tuberculosis (TB).

Methods:
A systematic search was performed in PubMed, Embase, Web of Science, Elsevier Science Direct, Cochrane Library, Chinese National Knowledge Infrastructure, Wanfang, and Chongqing VIP databases for case-control study on TaqI gene polymorphism and TB susceptivity. Quality assessment of studies was performed using the Newcastle–Ottawa Scale for the methodological assessment of case-control studies, and R 4.0.5 software was used for the meta-analysis.

Results:
Among the 243 selected articles, 27 in the meta-analysis. The meta-analysis showed that the TaqI gene polymorphism allene gene model (t vs T, odds ratio [OR]: 1.12, 95% confidence interval [CI]: 0.99–1.27); dominant model (tt + tT vs TT, OR: 1.12, 95% CI: 0.98–1.29); recessive model (tt vs tT + TT, OR: 1.25, 95% CI: 1.03–1.51); codominant A (tt vs TT, OR: 1.37, 95% CI: 1.00–1.87); codominant B (tT vs TT, OR: 1.09, 95% CI: 0.99–1.19). And subgroup dominant model (tt + tT vs TT, OR: 1.27, 95% CI: 1.03–1.55) in Indianas, recessive model (tt vs tT + TT, OR: 1.49, 95% CI: 1.05–2.11) in Iranians, co-dominant B (tT vs TT, OR: 1.28, 95% CI: 1.03–1.59; OR: 1.42, 95% CI: 1.05–1.93) in Indianas and Iranians.

Conclusion:
This meta-analysis suggests a significant association between TB and the risk of TaqI in Iranians and Indians, but the vitamin D receptor polymorphism TaqI was not associated with Chinese. Thus, validation studies will be required to confirm these findings.
Clinical features and predictive factors related to liver injury in SARS-CoV-2 Delta and Omicron variant-infected patients
Authors: Deng Haohui , Lin Haowei , Mai Yuzhen , Liu Huiyuan , Chen Weilie 
Published: April 2022

Background
Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) Delta and Omicron variants have become the dominant variants worldwide, and studies focused on liver injury in these patients are limited.

Materials and methods
In this study, 157 SARS-CoV-2-infected patients were enrolled, including 77 Delta variant-infected patients and 80 Omicron variant-infected patients. Liver injury data and clinical data were summarized and compared between patients infected with the two variants, additionally, patients with or without liver injury were also compared and multivariate analysis was performed to explore the predictive factors related to liver injury in SARS-CoV-2-infected patients.

Results
Liver injury was found in 18 (23.4%)/15 (18.8%) in Delta/Omicron variant-infected patients on admission, and 4 (5.2%)/1 (1.3%) in Delta/Omicron variant-infected patients during hospitalization, respectively. The ratios of liver injury did not differ between the two groups (χ
2 = 1.571; P = 0.210). Among these patients, 17 (77.3%) and 12 (75.0%) Delta and Omicron variant-infected patients were considered to be related to SARS-CoV-2 infection, the biomarkers of liver function were mildly elevated, dominated by the parameter of cholangiocyte injury: 76.5% (13/17) and 83.3% (10/12) in Delta and Omicron variant-infected patients, and most of these patients recovered to normal during follow-up. Multivariate analysis showed that male sex [odds ratio (OR), 4.476; 95% confidence interval (CI), 1.235–16.222; P = 0.023] and high levels of peak viral load in the nasopharynx (OR, 3.022; 95% CI, 1.338–6.827; P = 0.008) were independent factors related to liver injury.

Conclusion
Cholangiocyte injury biomarkers are dominated in Delta and Omicron variant-infected patients, male sex and high levels of peak viral load in the nasopharynx are predictive factors related to liver injury in SARS-CoV-2-infected patients.
The relation of esophagogastroduodenoscopy time and novel upper gastrointestinal quality measures
Authors: Romańczyk Marcin , Romańczyk Tomasz , Lesińska Magdalena , Barański Kamil , Hartleb Marek , Waluga Marek 
Published: April 2022

Objectives
Various measures of esophagogastroduodenoscopy (EGD) quality have been proposed so far and the examination time was one of the first. The aim of the study was to compare the procedure time with novel novel quality measures – composite detection rate (CDR) and endoscopist biopsy rate (EBR).

Methods
It was prospective observational study. A total of 880 diagnostic EGDs conducted from 01.2019 to 07.2019 have been enrolled in the study.

Results
Median EGD time was 4.2 min. Procedures of longer duration were marked with higher CDR (26.3% vs. 11.8%; P < 0.0001), higher EBR (44.9% vs. 12.3%; P < 0.0001), and better upper gastrointestinal neoplasm (UGN) detection (1.8% vs. 0%; P = 0.004) in comparison with procedures of shorter duration. The procedures were divided into 4 groups based on the time quartiles (group 1 <3.3 min; group 2 3.3–4.2 min; group 3 4.2–5.3 min; group 4 >5.3 min). The odds ratios of groups 2, 3, and 4 for biopsy rate were 2.42 (95% CI, 1.33–4.55), 4.33 (95% CI, 2.46–7.94), and 5.51 (95% CI, 3.18–10.03), respectively, in comparison with group 1. The odds ratios of groups 2, 3, and 4 for CDR were 3.18 (95% CI, 2.03–4.97), 5.46 (95% CI, 3.51–8.50), and 23.44 (95% CI, 14.3–38.4), respectively, in comparison with group 1.

Conclusions
The procedure time is related to novel metrics – CDR and EBR. It is also related to UGN. Based on our findings it could be concluded that EGD should not last less than 4.2 min.
Multicentre, retrospective study to assess long-term outcomes of chelator based treatment with trientine in Wilson disease patients withdrawn from therapy with d-penicillamine
Authors: Weiss Karl Heinz , Kruse Carlot , Manolaki Nina , Zuin Massimo , Ferenci Peter , van Scheppingen Daphne , Wijnberg Larissa , de Koning Constance E. , Dhawan Anil 
Published: April 2022

Objectives
Trientine dihydrochloride (TETA-2HCl) has been used for the treatment of Wilson disease for over 30 years. The current study was designed to systematically evaluate existing data to further define the long-term outcome of the efficacy and tolerability of TETA-2HCl in Wilson disease patients.

Methods
Medical records of 77 Wilson disease patients were reviewed to collect data on hepatic and neurologic symptoms, copper (Cu) homeostasis and adverse events. Data were collected for 48 months after initiation of TETA-2HCl after withdrawal of D-penicillamine treatment.

Results
Mean duration of TETA-2HCl treatment was 8 years (range 5 months–32.5 years). Over the course of TETA-2HCl treatment, 35% of patients had no hepatic symptoms whereas in 49.4% of patients, hepatic symptoms improved. They remained unchanged in 10.4% of patients and worsened in 5.2% of patients. No patients progressed to acute hepatic failure or necessity of a liver transplant. During TETA-2HCl treatment, 46.7% of patients had no neurologic symptoms; in 14.3% of patients, neurologic symptoms improved whereas in 36.4% of patients, they remained stable and worsened in 2.6% of patients. During the evaluation period, 12 patients discontinued TETA-2HCl treatment due to: anemia (N = 1), inadequate hepatic response (N = 2), switch to zinc treatment (N = 8) and patient’s decision to withdraw from treatment (N = 1). Treatment-emergent adverse events were reported by 24.7% of the patients of which gastrointestinal disorders (9.1%) and nervous system disorders (5.2%) were most reported.

Conclusions
TETA-2HCl is well-tolerated and effective in Wilson disease patients following the withdrawal of treatment with D-penicillamine. ClinicalTrials.gov
Identifier: NCT02426905.
Threshold of Quantitative Cytomegalovirus DNA PCR for Preemptive Treatment in Pediatric Hematopoietic Stem Cell Transplant Recipients
Authors: Ahn Bin , Yun Ki Wook , Hong Kyung Taek , Choi Jung Yoon , Kang Hyoung Jin , Seong Moon-Woo , Kim Taek Soo , Ahn Sung Jin , Choi Eun Hwa 
Published: April 2022

Background:
Consensus cytomegalovirus (CMV) DNA viral load thresholds for intervention in hematopoietic stem cell transplant (HSCT) recipients have not been established, especially in children. This study aimed at obtaining viral load thresholds of CMV DNA to guide preemptive management in pediatric HSCT recipients.

Materials and Methods:
A total of 465 blood samples from 177 children who received HSCT between 2015 and 2019 were included in a single center in Korea. The samples were analyzed for CMV infection by both antigenemia assay and quantitative DNA polymerase chain reaction. The 2 assay results were compared for the 233 samples which were collected when antiviral treatment has not been initiated. We determined the viral loads corresponding to the antigenemia of 5 pp65-positive cells/2×105 white blood cells (WBCs) as the level for initiating preemptive therapy.

Results:
Sixty percent of the samples were collected within 100 days (39.7% in 0 to 50 d, 60.2% in 0 to 100 d) from the graft infusion. The correlation between CMV DNA viral load and CMV antigenemia level increased significantly after 50 days from the graft infusion (r=0.71 vs. r=0.93, P<0.0001). The correlation was greater in the antiviral treatment-naive group than the treatment group (r=0.75 vs. r=0.66, P<0.0001). Under receiver operating characteristic curve analysis of the treatment-naive group, the estimated threshold CMV DNA viral loads corresponding to 5 pp65-positive cells/2×105 WBCs was 898 IU/mL.

Conclusions:
The CMV DNA levels that corresponded to 5 pp65-positive cells/2×105 WBCs was 900 IU/mL in the HSCT group. The proposed viral load thresholds can be used to guide preemptive therapy in pediatric HSCT recipients, especially in the preengraftment period.
Shwachman-Diamond Syndrome With Congenital Myogenic Ptosis: Case Report of a Rare Association?
Authors: Wang Nai-Wei , Georgara Natalia , Khan Haji S. 
Published: April 2022

Background:
Shwachman-Diamond syndrome (SDS) is a multisystem disorder characterized by exocrine pancreatic insufficiency and bone marrow failure. There is considerable variation in the phenotypes of SDS. We present a case of an infant presenting with SDS and left-sided ptosis.

Observation:
We report a case of an infant who presented with 2 episodes of severe sepsis and cytopenia, without overt symptoms of exocrine pancreatic deficiency or skeletal abnormalities. Persistent left-sided ptosis was noted in both presentations. Genetic testing confirmed the diagnosis of SDS. The left-sided ptosis was diagnosed as congenital myogenic ptosis.

Conclusion:
The association of ptosis and other congenital bone marrow failure syndromes is well established, but this is the first description of SDS with ptosis. This association may expand our understanding of SDS phenotypes if similar cases are reported in the future.
Family Caregivers’ Experiences of Caring for Advanced Cancer Patients
Authors: Zhu Yuxuan , Pei Xianbo , Chen Xiaoli , Li Tong 
Published: April 2022

Background
Advanced cancers qualify as severe stressors to family caregivers (FCGs), which can negatively impact caregivers’ psychological and physical well-being because of their association with high symptom burden, distress, and poor prognosis.

Objective
This review aims to synthesize FCGs’ experiences of caring for advanced cancer patients using a qualitative systematic review method.

Methods
A comprehensive search was conducted in 7 databases from inception until July 2020. Two reviewers independently screened and assessed each study using Joanna Briggs Institute instruments and subsequently undertook the meta-aggregation approach to synthesize findings.

Results
A total of 26 studies were included, refined to 37 findings, and integrated into 9 categories and 5 synthesized findings. When a loved one with advanced cancer faced deterioration near the end of their life, FCGs showed a tremendous sense of responsibility for care and concerted great efforts to alleviate their loved one’s suffering while lacking effective professional support. Cultural beliefs had a great impact on FCGs’ responsibility and role recognition. Ultimately, the caregiving helped FCGs achieve personal transcendence inherent in their unique experience.

Conclusions
Caring for advanced cancer patients is a unique, culture-specific experience marked by struggle. Effective professional support, including early palliative care, should be considered to improve the FCGs’ experience of caring for advanced cancer patients. Cultural beliefs should be considered to understand and develop appropriate strategies to support FCGs.

Implications for Practice
Healthcare providers need to ensure that individualized, multifaceted interventions considering FCGs’ needs are delivered at the optimal time with the appropriate approach.
Peripheral and Central/Intraosseous Vasoactive Infusions During and After Pediatric Critical Care Transport: Retrospective Cohort Study of Extravasation Injury*
Authors: Peshimam Niha , Bruce-Hickman Kara , Crawford Katrina , Upadhyay Gaurang , Randle Elise , Ramnarayan Padmanabhan , Jones Andrew J. 
Published: April 2022

OBJECTIVES:
To compare the prevalence of adverse events related to vasoactive drug infusions administered via a peripheral venous catheter versus a central venous or intraosseous catheter.

DESIGN:
Retrospective observational study.

SETTING:
A pediatric critical care transport team, and the PICUs and regional hospitals within the North Thames and East Anglia regions of the United Kingdom.

PATIENTS:
Children (up to 18 yr old) transported by the Children’s Acute Transport Service receiving an infusion of a vasoactive drug (epinephrine, dobutamine, dopamine, norepinephrine, and vasopressin).

INTERVENTIONS:
None.

MEASUREMENTS AND MAIN RESULTS:
The medical records of all children transported between April 2017 and May 2020 receiving a vasoactive drug infusion were reviewed and cross-referenced with the service critical incident database. The outcome measure was anatomic catheter-related adverse events (including extravasation) reported during transport or in the first 24 hours on the PICU. During the study period, the service undertook 3,836 transports. Vasoactive drugs were administered during 558 patient transports (14.5%). During 198 of 558 transports (35.5%), vasoactive drugs were administered via a peripheral venous catheter, with seven of 198 (3.5%) adverse events. One extravasation event resulted in tissue necrosis. The median time to injury after the infusion was commenced was 60 minutes (interquartile range, 30–60 min). During 360 of 558 transports (64.5%), vasoactive infusions were administered by central venous or intraosseous catheter, with nine of 360 (2.5%) adverse events.

CONCLUSIONS:
During pediatric critical care transport, we did not find a difference in prevalence of adverse events following the administration of vasoactive drugs via peripheral venous catheters or via central venous and intraosseous catheters.
Clinical Decision Support in the PICU: Implications for Design and Evaluation*
Authors: Dziorny Adam C. , Heneghan Julia A. , Bhat Moodakare Ashwini , Karavite Dean J. , Sanchez-Pinto L. Nelson , McArthur Jennifer , Muthu Naveen ,   on behalf of the Pediatric Data Science and Analytics (PEDAL) Subgroup of the Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) Network
Published: April 2022

OBJECTIVES:
To assess the current landscape of clinical decision support (CDS) tools in PICUs in order to identify priority areas of focus in this field.

DESIGN:
International, quantitative, cross-sectional survey.

SETTING:
Role-specific, web-based survey administered in November and December 2020.

SUBJECTS:
Medical directors, bedside nurses, attending physicians, and residents/advanced practice providers at Pediatric Acute Lung Injury and Sepsis Network-affiliated PICUs.

INTERVENTIONS:
None.

MEASUREMENTS AND MAIN RESULTS:
The survey was completed by 109 respondents from 45 institutions, primarily attending physicians from university-affiliated PICUs in the United States. The most commonly used CDS tools were people-based resources (93% used always or most of the time) and laboratory result highlighting (86%), with order sets, order-based alerts, and other electronic CDS tools also used frequently. The most important goal providers endorsed for CDS tools were a proven impact on patient safety and an evidence base for their use. Negative perceptions of CDS included concerns about diminished critical thinking and the burden of intrusive processes on providers. Routine assessment of existing CDS was rare, with infrequent reported use of observation to assess CDS impact on workflows or measures of individual alert burden.

CONCLUSIONS:
Although providers share some consensus over CDS utility, we identified specific priority areas of research focus. Consensus across practitioners exists around the importance of evidence-based CDS tools having a proven impact on patient safety. Despite broad presence of CDS tools in PICUs, practitioners continue to view them as intrusive and with concern for diminished critical thinking. Deimplementing ineffective CDS may mitigate this burden, though postimplementation evaluation of CDS is rare.
Increased Fellow Scholarly Publication Rate After Implementation of a Comprehensive Research Curriculum and Milestone-Based Research Timeline for Pediatric Emergency Medicine Fellows
Authors: Schmucker Kyle A. , Zuckerbraun Noel S. , Vitale Melissa A. , Hickey Robert W. , Callaway Clifton W. , Pitetti Raymond D. , Saladino Richard A. , Manole Mionora D. 
Published: April 2022

Objective
Pediatric subspecialty fellows are required to complete a scholarly product during training; however, many do not bring the work to publication. To amplify our fellows’ publication success, our pediatric emergency medicine fellowship program implemented a comprehensive research curriculum and established a milestone-based research timeline for each component of a project. Our objective was to assess whether these interventions increased the publication rate and enhanced the graduated fellows' perceived ability to perform independent research.

Methods
Our study was conducted at a tertiary children's hospital affiliated with an academic university, enrolling 3 fellows each year in its pediatric emergency medicine program. A comprehensive research curriculum and a milestone-based research timeline were implemented in 2011. We analyzed the publication rate of our graduating fellows before (2004–2011) and after (2012–2016) our intervention. In addition, in 2017 we surveyed our previous fellows who graduated from 2004 to 2016 and analyzed factors favoring manuscript publication and confidence with various research skills.

Results
During the study period, 38 trainees completed the fellowship program. Publication rate increased from 26% ± 17% to 87% ± 30 % (P < 0.05). When scoring the importance of various factors, fellows most valued mentorship (5 ± 0 vs 4.3 ± 1.0, P < 0.05, postintervention vs preintervention) for the completion of the fellowship study and manuscript. Fellows after the intervention reported greater confidence in performing an analysis of variance (89% vs 36%, odds ratio, 6.3; 95% confidence interval, 1.4–150.1).

Conclusions
Implementation of a comprehensive research curriculum and a milestone-based research timeline was associated with an increase in the publication rate within 3 years of graduation of our pediatric emergency medicine fellows. After implementation, fellows reported an increased importance of mentorship and greater confidence in performing an analysis of variance. We provide a comprehensive curriculum and a research timeline that may serve as a model for other fellowship programs.