bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical features of plastic bronchitis in children after congenital heart surgery
Authors: Zhu Li-Min , Li Chun-Xiang , Gong Xiao-Lei , Xu Zhuo-Ming , Liu Jin-Long , Zhang Hai-Bo 
Published: April 2024

Abstract
Background
Plastic bronchitis (PB) can occur in patients who have undergone congenital heart surgery (CHS). This study aimed to investigate the clinical features of PB in children after CHS.

Methods
We conducted a retrospective cohort study using the electronic medical record system. The study population consisted of children diagnosed with PB after bronchoscopy in the cardiac intensive care unit after CHS from May 2016 to October 2021.

Results
A total of 68 children after CHS were finally included in the study (32 in the airway abnormalities group and 36 in the right ventricular dysfunction group). All children were examined and treated with fiberoptic bronchoscopy. Pathogens were detected in the bronchoalveolar lavage fluid of 41 children, including 32 cases in the airway abnormalities group and 9 cases in the right ventricular dysfunction group. All patients were treated with antibiotics, corticosteroids (intravenous or oral), and budesonide inhalation suspension. Children with right ventricular dysfunction underwent pharmacological treatment such as reducing pulmonary arterial pressure. Clinical symptoms improved in 64 children, two of whom were treated with veno-arterial extracorporeal membrane oxygenation (ECMO) due to recurrent PB and disease progression.

Conclusions
Children with airway abnormalities or right ventricular dysfunction after CHS should be alerted to the development of PB. Pharmacological treatment such as anti-infection, corticosteroids, or improvement of right ventricular function is the basis of PB treatment, while fiberoptic bronchoscopy is an essential tool for the diagnosis and treatment of PB. ECMO assistance is a vital salvage treatment for recurrent critically ill PB patients.
“Quality of Life in Epidermolysis Bullosa” and “Epidermolysis Bullosa Burden of Disease”: Italian translation, cultural adaptation, and pilot testing of two disease-specific questionnaires
Authors: El Hachem May , Diociaiuti Andrea , Zambruno Giovanna , Samela Tonia , Ferretti Francesca , Carnevale Claudia , Linertová Renata , Bodemer Christine , Murrell Dédée F. , Abeni Damiano 
Published: April 2024

Abstract
Background
Inherited epidermolysis bullosa (EB) is a clinically and genetically heterogeneous group of skin fragility disorders characterized by blister formation following minor trauma. Four major types are distinguished based on the level of cleavage within the skin. Most EB forms present severely disabling cutaneous and systemic signs and symptoms. Management relies on daily time-consuming and distressing topical medications, and symptomatic treatment of systemic findings. Disease manifestations, symptoms, and daily care strongly affect patient and caregiver quality of life (QoL). To date, there are two validated EB-specific questionnaires, the “Quality of Life in Epidermolysis Bullosa” (QOLEB) and the “Epidermolysis Bullosa Burden of Disease” (EB-BoD) for the evaluation of patient and family disease burden, respectively. The aim of our study was to develop an Italian translation of the two questionnaires and to pilot-test them.

Methods
The guidelines for translation and cross-cultural adaptation of health-related QoL measures were followed. Initially, two separate translations were generated for each questionnaire, and subsequently reconciled by an expert committee. This was followed by a back-translation process. The original texts and all translations underwent revision by the expert committee, resulting in definitive versions. The final versions were then tested in a pilot study involving cognitive debriefing in a group of 17 families, representative of all EB major types.

Results
The translation and reconciliation process led to minor changes to obtain semantic/idiomatic/cultural equivalence of the Italian versions with the original ones and to reconcile the questions with the answer options. The cognitive debriefing process showed a good understanding and did not require text modifications.

Conclusions
The Italian versions of the QOLEB and EB-BoD provide valuable tools in everyday clinical practice of reference centers, and they allow the participation in multicenter international real-life observational studies as well as in controlled clinical trials. They enable the identification of disease-specific psychological and socioeconomic challenges for EB patients and their families, guiding targeted interventions to ensure appropriate and timely care.
Treatment of COVID-19-associated ARDS with umbilical cord-derived mesenchymal stromal cells in the STROMA-CoV-2 multicenter randomized double-blind trial: long-term safety, respiratory function, and quality of life
Authors: Sitbon Alexandre , Hauw-Berlemont Caroline , Mebarki Miryam , Heming Nicholas , Mayaux Julien , Diehl Jean-Luc , Demoule Alexandre , Annane Djillali , Marois Clémence , Demeret Sophie , Weiss Emmanuel , Voiriot Guillaume , Fartoukh Muriel , Constantin Jean‐Michel , Mégarbane Bruno , Plantefève Gaëtan , Boucher-Pillet Hélène , Churlaud Guillaume , Cras Audrey , Maheux Camille , Pezzana Chloé , Diallo Mamadou Hassimiou , Lebbah Said , Ropers Jacques , Salem Joe-Elie , Straus Christian , Menasché Philippe , Larghero Jérôme , Monsel Antoine ,   APHP STROMA–CoV‐2 Collaborative Research Group, Benchetrit Déborah , Bonvallot Harold , Charbonnier-Beaupel Fanny , Dhib-Charfi Meriem , Delmotte Pierre Romain , Kone Assitan , Le Corre Marine , Metz Carole , Puybasset Louis , Vezinet Corinne 
Published: April 2024

Abstract
Background
The STROMA-CoV-2 study was a French phase 2b, multicenter, double-blind, randomized, placebo-controlled clinical trial that did not identify a significant efficacy of umbilical cord-derived mesenchymal stromal cells in patients with SARS-CoV-2-induced acute respiratory distress syndrome. Safety on day 28 was found to be good. The aim of our extended study was to assess the 6- and 12-month safety of UC-MSCs administration in the STROMA-CoV-2 cohort.

Methods
A detailed multi-domain assessment was conducted at 6 and 12 months following hospital discharge focusing on adverse events, lung computed tomography-scan, pulmonary and muscular functional status, and quality of life in the STROMA-CoV-2 cohort including SARS–CoV-2-related early (< 96 h) mild‐to-severe acute respiratory distress syndrome.

Results
Between April 2020 and October 2020, 47 patients were enrolled, of whom 19 completed a 1-year follow-up. There were no significant differences in any endpoints or adverse effects between the UC-MSCs and placebo groups at the 6- and 12-month assessments. Ground-glass opacities persisted at 1 year in 5 patients (26.3%). Furthermore, diffusing capacity for carbon monoxide remained altered over 1 year, although no patient required oxygen or non-invasive ventilatory support. Quality of life revealed declines in mental, emotional and physical health throughout the follow-up period, and the six-minute walking distance remained slightly impaired at the 1-year patient assessment.

Conclusions
This study suggests a favorable safety profile for the use of intravenous UC-MSCs in the context of the first French wave of SARS-CoV-2-related moderate-to-severe acute respiratory distress syndrome, with no adverse effects observed at 1 year.
Treatment for Covid-19 with SARS-CoV-2 neutralizing antibody BRII-196(Ambavirumab) plus BRII-198(Lomisivir): a retrospective cohort study
Authors: Yalan Qin , Lingfang Hao , Xisong Liu , Run Liang , Junjing Zhang , An Zhang’ 
Published: April 2024

Abstract
Background
Monoclonal antibody therapy for Covid-19 springs up all over the world and get some efficiency. This research aims to explore the treating effect of BRII-196(Ambavirumab) plus BRII-198(Lomisivir) on Covid-19.

Methods
In this retrospective cohort research, patients received standard care or plus BRII-196 /BRII-198 monoclonal antibodies. General comparison of clinical indexes and prognosis between Antibody Group and Control Group was made. Further, according to the antibody using time and patients’ condition, subgroups included Early antibody group, Late antibody group, Mild Antibody Group, Mild Control Group, Severe Antibody Group and Severe Control Group.

Results
Length of stay(LOS) and interval of Covid-19 nucleic acid from positive to negative of Antibody Group were 12.0(IQR 9.0–15.0) and 14.0(IQR 10.0–16.0) days, less than those(13.0 (IQR 11.0–18.0) and 15.0 (IQR 12.8–17.0) days) of Control Group(p = 0.004, p = 0.004). LOS(median 10days) of Early Antibody Group was the shortest, significantly shorter than that of Control Group (median 13days)(p < 0.001). Interval(median 12days) of Covid-19 nucleic acid from positive to negative of Early Antibody Group also was significantly shorter than that of Control Group(median 15days) and Late Antibody Group(median 14days)(p = 0.001, p = 0.042). LOS(median 12days) and interval(median 13days) of Covid-19 nucleic acid from positive to negative of Mild Antibody Group was shorter than that of Mild Control Group(median 13days; median 14.5days)(p = 0.018, p = 0.033).

Conclusion
The neutralizing antibody therapy, BRII-196 plus BRII-198 could shorten LOS and interval of Covid-19 nucleic acid from positive to negative. However, it didn’t show efficacy for improving clinical outcomes among severe or critical cases.
Nutritional status of children with neurodevelopmental disorders: a cross-sectional study at a tertiary-level hospital in northern Bangladesh
Authors: Khatun Rabeya , Bin Siddique Md. Kaoser , Khatun Mst. Reshma , Benzir Maskura , Islam Md. Rafiqul , Ahmed Sohel , Muurlink Olav 
Published: April 2024

Abstract
Background
Malnutrition in children with neurodevelopmental disorders (NDDs) is a significant global public health issue. Nutritional assessment combined with management or advice are essential to produce optimal outcomes.

Objectives
The objective of this study was to assess nutritional status and the sociodemographic profile of children with neurodevelopmental disorders in Bangladesh.

Methods
A cross-sectional study was conducted from December to April 2020 among the population of children with NDDs who presented to the pediatric department of the TMSS Medical College and Rafatullah Community Hospital in Bogura during this period. Socio-demographic data along with anthropometric measurements of the children were taken. Assessment of nutritional status were made using metrics such as z-scores for weight-for-age (WAZ), height-for-age (HAZ), and body mass index-for-age (BAZ). Descriptive statistics (number and percentage) and analytical statistics (chi-square and logistic regression) were included.

Results
58.6% of children displayed malnutrition, with 47.8% showing undernutrition (WHZ / BAZ − 1 SD-≤-3 SD), and 10.8% overnutrition (BAZ > 2SD). Significant negative associations were found between malnutrition and parental education level, urban residency, and monthly family income. Children diagnosed with cerebral palsy exhibited twice the likelihood to be malnourished (AOR 2.39, 95% CI 0.83–6.87). Furthermore, residing in rural regions was associated with an increased risk of experiencing malnutrition, as indicated by an adjusted odds ratio of 1.60 (95% CI 0.12–3.09).

Conclusions
While the results are cross-sectional, over half of children with NDDs were found to be malnourished, suggesting that children with NDD in Bangladesh are vulnerable to developing any form of malnutrition. Therefore, regular assessments and timely nutritional support may improve their situation.
Detection of and response to gender-based violence: a quality improvement project across three secondary mental health services in London
Authors: Keynejad Roxanne C. , Boardman-Pretty Theo , Barber Sarah , Tweed John , Forshall Emily , Edwards Alice , Shotton Joshua , Wilson Claire A. 
Published: April 2024

Aims and method
Our team of core and higher psychiatry trainees aimed to improve secondary mental health service detection of and response to gender-based violence (GBV) in South East London. We audited home treatment team (HTT), drug and alcohol (D&A) service and in-patient ward clinical records (n = 90) for female and non-binary patients. We implemented brief, cost-neutral staff engagement and education interventions at service, borough and trust levels before re-auditing (n = 86), completing a plan–do–study–act cycle.

Results
Documented enquiry about exposure to GBV increased by 30% (HTT), 15% (ward) and 7% (D&A), post-intervention. We identified staff training needs and support for improving GBV care. Up to 56% of records identified psychiatric symptoms related to GBV exposure.

Clinical implications
Moves to make mental healthcare more trauma-informed rely on services first being supportive environments for enquiry, disclosure and response to traumatic stressors. Our collaborative approach across clinical services increased GBV enquiry and documentation. The quality of response is more difficult to measure and requires concerted attention.
Ganienkeh, Out of the City and Away from the Reservation: The Making of an Indigenous Space, 1974–1979
Authors: Johnson Amanda 
Published: April 2024

Abstract
In 1974, a group of Kahnawá:ke Mohawk families claimed a New York State–run campsite with the intent of starting a “traditional” Indigenous community they named Ganienkeh. Residents ultimately secured a permanent space in upstate New York for the community to grow in 1979. Using newspapers, interviews, and organizational newsletters, this article argues that the sources of this takeover depended on Ganienkeh people who exercised sovereignty on their own innovative terms. Using the power of gender, kinship, and family, they maintained support from outside groups and successfully fought against and capitalized on the cultural tensions of the decade.
Improving access to gene therapy for rare diseases
Authors: Fox Thomas A. , Booth Claire 
Published: April 2024

ABSTRACT
Effective gene therapy approaches have been developed for many rare diseases, including inborn errors of immunity and metabolism, haemoglobinopathies and inherited blindness. Despite successful pre-clinical and clinical results, these gene therapies are not widely available, primarily for non-medical reasons. Lack of commercial interest in therapies for ultra-rare diseases, costs of development and complex manufacturing processes required for advanced therapy medicinal products (ATMPs) are some of the main problems that are restricting access. The complexities and costs of navigating the regulatory environments in different jurisdictions for treatments that affect small numbers of patients is a problem unique to ATMPS for rare and ultra-rare diseases. In this Perspective, we outline some of the challenges and potential solutions that, we hope, will improve access to gene therapy for rare diseases.
Application of Artificial Intelligence to Automate the Reconstruction of Muscle Cross-Sectional Area Obtained by Ultrasound
Authors: DA SILVA DEIVID GOMES , DA SILVA DIEGO GOMES , ANGLERI VITOR , SCARPELLI MAÍRA CAMARGO , BERGAMASCO JOÃO GUILHERME ALMEIDA , NÓBREGA SANMY ROCHA , DAMAS FELIPE , CHAVES TALISSON SANTOS , CAMARGO HELOISA DE ARRUDA , UGRINOWITSCH CARLOS , LIBARDI CLEITON AUGUSTO 
Published: April 2024

ABSTRACT

Purpose
Manual reconstruction (MR) of the vastus lateralis (VL) muscle cross-sectional area (CSA) from sequential ultrasound (US) images is accessible, is reproducible, and has concurrent validity with magnetic resonance imaging. However, this technique requires numerous controls and procedures during image acquisition and reconstruction, making it laborious and time-consuming. The aim of this study was to determine the concurrent validity of VL CSA assessments between MR and computer vision–based automated reconstruction (AR) of CSA from sequential images of the VL obtained by US.

Methods
The images from each sequence were manually rotated to align the fascia between images and thus visualize the VL CSA. For the AR, an artificial neural network model was utilized to segment areas of interest in the image, such as skin, fascia, deep aponeurosis, and femur. This segmentation was crucial to impose necessary constraints for the main assembly phase. At this stage, an image registration application, combined with differential evolution, was employed to achieve appropriate adjustments between the images. Next, the VL CSA obtained from the MR (n = 488) and AR (n = 488) techniques was used to determine their concurrent validity.

Results
Our findings demonstrated a low coefficient of variation (CV) (1.51%) for AR compared with MR. The Bland–Altman plot showed low bias and close limits of agreement (+1.18 cm2, −1.19 cm2), containing more than 95% of the data points.

Conclusions
The AR technique is valid compared with MR when measuring VL CSA in a heterogeneous sample.
Process- and Product-Oriented Fundamental Movement Skills in Early Childhood as Predictors of Later Health-Related Fitness
Authors: KASANEN MARIA , SÄÄKSLAHTI ARJA , NIEMISTÖ DONNA , TOLVANEN ASKO , LUUKKAINEN NANNE-MARI , MEKLIN ELINA , LAUKKANEN ARTO 
Published: April 2024

ABSTRACTIntroductionThe skill domains of fundamental movement skills (FMS), emphasizing gross motor movements, including locomotor skills (LMS) and object control skills (OCS), along with process- and product-oriented measures of FMS, may predict cardiorespiratory fitness (CRF) and muscular fitness (MF) differently. This study investigates the longitudinal relationship from early childhood FMS, focusing on process-oriented LMS and OCS and product-oriented FMS, to CRF and MF in late childhood.MethodsThe study involved 441 Finnish children (49.9% female; mean age at baseline, 5.5 yr) over a 6-yr period. FMS was evaluated using the Test of Gross Motor Development, third version, for process-oriented LMS and OCS, and the Körperkoordinationstest Für Kinder (KTK) was used to evaluate the product-oriented FMS. CRF was assessed through the total number of laps completed in the 20-m shuttle run test, whereas MF was measured via repetitions of curl-ups and push-ups. Employing a two-level cross-classified regression analysis and Cholesky decomposition, this study aimed to determine the contributions of product-oriented KTK and process-oriented LMS and OCS. Adjustments for variations in age, measurement intervals, and maturation were achieved through residualization. In addition, gender and body mass index were incorporated as covariates in the analysis.ResultsThe analysis revealed that process-oriented LMS (CRF: ΔR2= 0.016; MF: ΔR2= 0.014) significantly predicted later health-related fitness, whereas OCS did not. However, KTK exhibited a better ability to predict both CRF (ΔR2= 0.092) and MF (ΔR2= 0.032), overshadowing process-oriented measures.ConclusionsIn conclusion, the findings suggest that KTK, which potentially encompasses a broader spectrum of fitness elements along with FMS, more effectively predicts health-related fitness components than process-oriented FMS.
Innovative and transdisciplinary educational approach through the EDICULA toolkit
Authors: Roumeliotis Stergios , Lampropoulos Kyriakos 
Published: April 2024

The EDICULA educational toolkit regards an open source educational platform that addresses key issues in the rehabilitation, protection and sustainability of Cultural Heritage (CH) assets, and disseminates valuable knowhow and experience both to the wide audience as well as to CH stakeholders, scientists and professionals. The fundamental characteristic of this platform is that it promotes a holistic approach for transdisciplinary documentation, based on the experience and know-how of the EDICULA partners and – in the future – of the users as they will begin to utilize it. Both EDICULA toolkit modules are described after the development of the main thematic areas, the development of the toolkit on a moodle platform, and the preparation and uploading of the educational material. The access to the EDICULA educational platform, the layout of the educational platform, the types of educational material uploaded, the issues related to the toolkit-to-user information presentation approach and educational aspects, the complexities encountered and how they were overcome are being analyzed. The issue of users and degree of commonality between EDICULA-4-all and EDICULA+ educational toolkits is described. Emphasis is given on the way the modules are differentiated from one another. The access level is the main issue, which eventually provides a “smooth” transition between the two modules, ad hoc per user, under the supervision of registered and approved administrators or advanced users with editing privileges.
The joint utilization of both the EDICULA-4-all and EDICULA+ toolkits in the educational, advisory or research processes, is at the core of the EDICULA project’s scopes.
Applications of gene modification technologies in the treatment of inherited diseases
Authors: Superson Maciej , Szmyt Katarzyna , Wilk-Trytko Klaudia , Krasnoborska Julia , Samojedny Sylwia , Szymańska Katarzyna , Walczak Kamil 
Published: April 2024

Introduction and Purpose: In last years gene modification technologies such as CRISPR/Cas9 has had a revolutionary impact on the treatment of inherited diseases. Technologies developed from bacterial defense mechanisms, has become a basic tools in scientific research and medical therapies. In our article we provided an overview of applications of gene modifications technologies, directly focusing on CRISPR/Cas9, in genetic disease treatment.

State of Knowledge: New applications of CRISPR/Cas9 are still being explored. Treating inherited diseases such as cystic fibrosis, Duchenne muscular dystrophy, thalassemia, hemophilia, Huntington's disease, Crigler-Najjar syndrome, sickle cell anemia, Marfan syndrome, and phenylketonuria, is feasible with this novel technique. A comparative analysis with other gene editing methods highlights CRISPR/Cas9's efficacy, ease of use, and multiplexing capabilities.

Summary: CRISPR/Cas9 is a groundbreaking technology with broad applications in genetic research and therapy. Its ease of use, cost-effectiveness, and ability to target multiple genes simultaneously position it as a preferred method. However, there are some challenges associated with precision issues and ethical considerations in human embryo gene editing. As CRISPR/Cas9 continues to evolve, responsible application and ethical considerations are important for maximizing its potential in treatment of genetic diseases.
Lymphoedema specialists embedded into community nurse and wound services: impacts and outcomes
Authors: Thomas Melanie , Morgan Karen , Humphreys Ioan 
Published: April 2024

Background:Lymphoedema is a progressive condition causing significant alterations to life, exerting pressures on unscheduled care from complications including cellulitis and wounds. An on the ground education programme (OGEP) was implemented to raise knowledge, competence and confidence in lymphoedema management in community clinical services. The aim of this study was to explore the impact and outcomes of the OGEP intervention.Methods:Data were captured before and after OGEP on 561 lymphoedema patients in the community setting. Data recorded included resource use, costs and outcomes (EQ-5D-5L and LYMPROM).Results:Data demonstrated statistically significant reductions in resource allocations including staff visits (P<0.001), cellulitis admissions (P<0.001), compression consumables and wound dressing costs (P<0.001). Overall, the total mean per patient cost decreased from £1457.10 to £964.40 (including intervention) with outcomes significantly improved in EQ-5D-5L/LYMPROM scores.Conclusion:The analysis suggests the OGEP intervention may offer reductions in resource costs and improvements in patient outcomes. OGEP may therefore provide an innovative solution in future care delivery.