bluuebunny/crossref_metadata_2025_split huggingface dataset:
Dissecting pathophysiology of a human dominantly inherited disease, familial amyloidotic polyneuropathy, by using genetically engineered mice
Authors: Li Zhenghua , Yamamura Kenichi 
Published: May 2022

Abstract
Familial amyloidotic polyneuropathy (FAP) is a type of systemic amyloidosis characterized by peripheral and autonomic neuropathy. Although FAP is a typical autosomal dominant disorder caused by a point mutation in the TTR gene, the average age at onset varies significantly among different countries. This discrepancy clearly suggests that a combination of intrinsic factors as well as extrinsic (environmental) factors shapes the development of FAP. However, these factors are difficult to analyze in humans, because detailed pathologic tissue analysis is only possible at autopsy. Thus, mouse models have been produced and used to disentangle these factors. This review covers the mouse models produced thus far and how these models are applied to analyze intrinsic and extrinsic factors involved in disease development and to test drug efficacy.
Challenges and improvement in management of neonates born to mothers with COVID-19 in China
Authors: Yang Jie , Ren Zhuxiao , Zeng Lingkong , Xia Shiwen , Wang Lin , Miao Jiayu , Zhao Zhe , Yang Chuanzhong , Cheng Xiuyong , Zhang Huayan , Zhu Yuanfang , Liu Li , Gao Xirong , Yi Bin , Lin Zhenlang , Liu Wei , Zhou Xiaoyu , Wu Benqing , Chen Ling , Li Zhankui , Zhang Xuefeng , Lu Wei , Zhang Lianhong , Chen Xiao , Zhong Xiaoyun , Xu Falin , Wu Jinhui , Ji Yong , Pan Jiahua , Chen Yanxiang , Ronsmans Carine , Feng Zhichun 
Published: May 2022

Abstract

Objective
China was the first country suffering from the SARS-CoV-2 pandemic and one of the countries with stringent mother-neonate isolation measure implemented. Now increasing evidence suggests that coronavirus disease 2019 (COVID-19) should not be taken as an indication for formula feeding or isolation of the infant from the mother.

Methods
We conducted a retrospective cohort study in 44 hospitals from 14 provinces in China to investigate the management of neonates whose mothers have confirmed or suspected COVID-19. In addition, 65 members of Chinese Neonatologist Association (CNA) were invited to give their comments and suggestions on the clinical management guidelines for high-risk neonates.

Results
There were 121 neonates born to 118 mothers suspected with COVID-19 including 42 mothers with SARS-CoV-2 positive results and 76 mothers with SARS-CoV-2 negative results. All neonates were born by caesarean section, isolated from their mothers immediately after birth and were formula-fed. Five neonates were positive for SARS-CoV-2 at initial testing between 36 and 46 h after birth. Regarding the confusion on the clinical management guidelines, 58.78% of the newborns were put into isolation, 32.22% were subject to PCR tests, and 5.16% and 2.75% received breastfeeding and vaccination, respectively.

Conclusion
The clinical symptoms of neonates born to mothers with confirmed SARS-CoV-2 were mild, though five neonates might have been infected in utero or during delivery. Given the favorable outcomes of neonates born to COVID-confirmed mothers, full isolation may not be warranted. Rather, separation of the mother and her newborn should be assessed on a case-by-case basis, considering local facilities and risk factors for adverse outcomes, such as prematurity and fetal distress.
Point of care ultrasonography in patients with haemophilia and acute haemarthrosis: a physiotherapist and sonographer inter-professional agreement pilot study
Authors: Strike Karen , Chan Anthony KC , Maly Monica R , Stein Nina , Farrell Lynne , Solomon Patricia 
Published: May 2022

Abstract

Background
Haemophilia treatment centres (HTCs) around the world are increasingly adopting point-of-care ultrasonography (POCUS) for the assessment of acute haemarthrosis and to monitor joint health. POCUS is in large part administered by physiotherapists in most comprehensive care teams. Appropriate implementation of haemophilia-specific POCUS requires an educational foundation and training to ensure competency and optimal outcomes. Inter-professional agreement and evaluation of image quality are important measures of competency and acceptable use of POCUS.

Aims
To determine the level of agreement between physiotherapist and sonographer-performed POCUS scans and to compare the quality of the ultrasound images obtained by physiotherapists to those obtained by the sonographer.

Methods
This single blind, prospective, pilot study recruited patients with haemophilia A and B who presented to clinic with a suspected acute haemarthrosis of the elbow, knee, or ankle and consented to participate. POCUS scans were performed by one trained physiotherapist and one sonographer in the haemophilia ambulatory clinic at patient presentation, one-week follow-up, and two-week follow-up. The physiotherapist participated in formal training consisting of 12 hours of online didactic modules and a two-day, 12-hour practical module with instructor-led hands-on training. For the primary objective, the outcome of interest was the binary decision on the presence or absence of blood within the joint. For the secondary objective, image quality was evaluated by the radiologist post hoc and rated as optimal, acceptable, or sub-optimal.

Results
Thirteen participants with haemophilia consented to the study. The results indicated an excellent level of agreement (k=0.80) with an observed agreement of 91.7%, a specific positive agreement of 94.1%, and a specific negative agreement of 85.7% for the detection of blood within the joint space. The quality of the ultrasound images obtained by the physiotherapist were rated by the radiologist as optimal (84.6%) and acceptable (15.4%). None of the images were rated as sub-optimal.

Conclusion
Optimal image quality and a high level of agreement between the physiotherapist and sonographer-performed POCUS for the assessment of acute hemarthrosis in people with haemophilia A and B was observed. These results suggest that, with a short formal training programme, physiotherapists can be proficient in the performance, acquisition, and interpretation of POCUS scans in patients with haemophilia.
Processes and experiences of satellite haemophilia clinic set-ups in Uganda – a short report
Authors: Kasirye Philip , Kisakye Agnes , Nakalyango Annette , Barnabas Atwiine 
Published: May 2022

Abstract
Haemophilia knowledge and care are largely missing in much of sub-Saharan Africa and there is a need for concerted efforts to ensure access to care services by affected persons. Haemophilia Foundation Uganda, supported by the global haemophilia community and working with the Uganda Ministry of Health, has set up eight satellite haemophilia treatment centres (HTCs) as part of a wider initiative to raise awareness and improve haemophilia care. Setting up the HTCs has involved a six-step process involving stakeholders in government, healthcare and the community, and ranging from securing initial support to an ongoing follow-up programme of mentorship and training. Over 1,700 healthcare professionals have been trained and 186 patients have been registered at these peripheral facilities over the past five years. This is helping to improve access to care, but there are still shortcomings around diagnostic capacity, available healthcare personnel, and facilities to procure recombinant factor products. We will continue and further our advocacy for budgetary inclusion of haemophilia at political and facility levels. We also propose a continued strengthening of the haemophilia care teams through mentorship, networking, and mobilisation for diagnostic support at large public hospitals.
Lärares iscensättning av stöttning vid nyanlända elevers digitala textproduktion
Authors: Hell Anna Irma Gertrud 
Published: May 2022

Previous studies describe scaffolding as fundamental for newly arrived students to understand and create texts. In this article, scaffolding students’ multilingual palette (the full range of multilingual resources) is vital for their meaning-making. This study aims to make linguistic diversity visible when scaffolding newly arrived students during digital text activities in narrative and retelling text in Swedish as a second language. The following research question guides the study: How do scaffolding processes during digital text activities contribute to opportunities for newly arrived students with varying backgrounds and experiences of the Swedish language to use their multilingual palettes? The data consists of ethnographic fieldwork in three primary schools (year 3), focusing on scaffolding during nine newly arrived students’ text activities. Through an iterative analysis process, scaffolding emerged to have the following functions: 1) to promote multilingualism, 2) to develop textual competence, 3) to support student space. Scaffolding of newly arrived students who did not yet speak Swedish contributed to all students being able to create text within the text types. However, the scaffolding did not support their use of multilingual resources during individual text activities. For students to develop multilingual resources, scaffolding must focus on translanguaging and non-verbal means of expression, even during individual activities. For this, using digital technology is advantageous.
Ta inte de grundläggande begreppen inom naturvetenskapen för givna
Authors: Nilsson Gunilla Åkesson 
Published: May 2022

Förståelsen för att materia och organismer består av mindre partiklar samt massans bevarande är grunden för att lära sig naturvetenskap. Vidare är det inom naturvetenskapen betydelsefullt att förstå hur de olika fysikaliska egenskaperna kan uppkomma och förändras i en produkt. Det är därför viktigt att känna till skillnaden mellan begreppen rena ämne och blandningar, eftersom rena substanser har ofta precisa och beständiga fysikaliska egenskaper, vilket en blandning inte har. Men att lära sig dessa begrepp har visat sig vara besvärligt.
Syftet med denna studie var att låta studenter som läser kemi 1 på tekniskt basår genomföra olika praktiska övningar om materiens partikelnatur för att se om denna undervisningsform skulle kunna öka deras förståelse för massans bevarande. Vidare utvecklades en modell ”Produktens rotsystem av ämnen” för att träna på att särskilja begreppen rent ämne och blandning. Övningar gjordes i början av kursen. I slutet av kursen fick de svara på fem frågor om massans bevarande med vardaglig koppling.
Det var ca 50 % som fick alla rätt på de fem frågorna, vilket är något bättre än 34 % hos gruppen som inte fick någon särskild undervisning om materiens partikelnatur och massans bevarande. En viss ökning i förståelse för skillnaden mellan begreppen rent ämne och blandning kunde urskiljas i deras tentamenssvar, men detta behöver undersökas i större omfattning för att kunna bekräftas.
Det är viktigt att undervisa studenter inom natur- och ingenjörsvetenskapliga utbildningar om begreppen materiens partikelnatur, massans bevarande, rent ämne och blandning. Annars kan dessa kunskapsbrister begränsa deras möjligheter att kunna lära sig mer avancerade koncept inom naturvetenskapen. Detta är särskilt viktigt för lärosäten som satsar på breddad rekrytering eftersom undervisningen inom naturvetenskap kan begränsas eller prioriteras bort för elever som följer en anpassad timplan till förmån för ämnen som krävs för gymnasiebehörighet.
Remote patient monitoring in spine surgery
Authors: Farias Frederico A. C. , Falavigna Vincenzo F. , Viapiana Maria Eduarda L. , Falavigna Asdrubal 
Published: May 2022

Objectives:
Remote monitoring or telemonitoring is an innovative strategy to improve patient care. This study aimed to present the authors’ experience of a remote monitoring platform for post-operative care of patients who have undergone spine surgery.

Methods:
A telemonitoring system for patients undergoing spine surgery, centered on a smartphone application named Wippe Track, (Brazilian Telemedicine Company BR HomMed, São Paulo, Brazil) compatible with both Android and iOS smartphones. All patients had two Bluetooth devices connected to the smartphone application: Digital scale and wristband activity tracker. The outcomes evaluated were based on patient-related outcome measures (PROMs) using specific disease-oriented questionnaires.

Results:
A preliminary study to refine the system based on patient/provider input was performed with 30 patients. The adherence and interaction with the platform were 94%. Patients have provided all the PROMs data inputs and have consistently interacted with monitoring center professionals and the surgeon through the platform. Weight loss was observed in 76% of overweight patients using remote nutritionist support and specific exercises.

Conclusion:
The patients were satisfied and felt secure. The remote monitoring could detect and help manage events and warnings related to post-operative issues and reduce the need to travel for non-scheduled care.
Brain overgrowth associated with megalencephaly-capillary malformation syndrome causing progressive Chiari and syringomyelia
Authors: Deleu Tom , Jansen Katrien , Calenbergh Frank Van 
Published: May 2022

Background:
Megalencephaly-capillary malformation (M-CM) syndrome is a rare overgrowth syndrome characterized by macrocephaly, port-wine stains, asymmetric brain growth, hydrocephalus, and developmental delay. Cerebellar tonsil herniation is often seen, but rarely with syringomyelia.

Case Description:
A newborn with M-CM syndrome developed a progressive Chiari malformation type I (CM-I) with syringomyelia. At 4 months, he was treated for subdural hematomas, while at 10 months, he required a shunt for hydrocephalus. At 16 years of age, he newly presented a left hemiparesis and ataxia. Notably, successive volumetric measurements of the posterior fossa/cerebellum showed disproportionate cerebellar growth over time that correlated with the appearance of a CM-I. Following a suboccipital craniectomy with C1-laminectomy and duraplasty, he neurologically improved.

Conclusion:
M-CM with CM-I and syringomyelia rarely present together. Here, we treated an infant with M-CM who developed a progressive CM-I malformation and syringomyelia reflecting disproportionate growth of the cerebellum/posterior fossa over a 16-year period.
Clinical outcome and safety of stem cell therapy for ischemic stroke: A systematic review and meta-analysis
Authors: Permana Andhika Tomy , Bajamal Abdul Hafid , Parenrengi Muhammad Arifin , Suroto Nur Setiawan , Lestari Pudji , Fauzi Asra Al 
Published: May 2022

Background:
Several reports on stem cell administration have emerged proving it to be an ideal therapeutic approach for improving neurological functions in ischemic stroke patients. However, some studies also show disappointing results, with some reporting no statistically significant improvements among several different parameters. Several challenges also arise relating to safety and nonscientific aspects, such as ethics.

Methods:
We performed a systematic review and meta-analysis to evaluate the effect of stem cell therapy on the clinical outcomes of ischemic stroke patients. A systematic review and meta-analysis were performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines. A thorough literature search was conducted on PubMed, Scopus, and Cochrane databases. Articles were selected systematically based on the PRISMA protocol and reviewed completely. A total of 19 publications pertaining to stem cell therapy on the ischemic route were included and reviewed. Efficacy outcomes were measured with the National Institutes of Health Stroke Scale, modified Rankin Scale, or Barthel Index.

Results:
The results of the meta-analysis indicate that the efficacy outcomes suggest favorable results after stem cell therapy, although not all study results are statistically significant. Stem cell therapy in stroke cases showed a better outcome than standard conservative therapy alone, although our analysis shows that many factors can influence this outcome, and significant effects can only be seen after several months.

Conclusion:
The results of this study show promising and satisfying efficacy and a relatively low rate of serious adverse events.
STRUCTURE OF TRAUMATIC INJURIES AND EXPERIENCE IN THE TREATMENT OF THE WOUNDED PATIENTS, AS A RESULT OF HOSTILITIES IN THE FIRST DAYS OF RUSSIA’S ATTACK ON UKRAINE
Authors: DENYSIUK M.V , DUBROV S. , CHERNIAIEV S. , SEREDA S. , ZAIKIN YU.M 
Published: May 2022

Introduction. All the gunshot wounds are of high-energy. Gunshot and blast injuries, in most cases, are multiple, varying in depth and area of the wound surface. From the first day of the full-scale invasion of Russian troops (February 24, 2022), many victims of hostilities on the right bank of the Dnipro river in Kyiv were hospitalized in our hospital, which is one of the clinical bases of the Department of Anesthesiology and Intensive Care of the Bogomolets National Medical University. Dozens of wounded patients needed simultaneous interventions, and the presence of only 5 operating tables in the facility did not prevent the provision of appropriate qualified medical care to 6-8 critical patients at the same time.
The goal of the work. To determine the structure and severity of traumatic injuries received as a result of hostilities.
Materials and methods. In the study a prospective analysis of 89 patients who were injured in hostilities for the period from February 24 and March 3 2022, and were hospitalized in one of the clinical bases of the Department of Anesthesiology and Intensive Care of the Bogomolets National Medical University was conducted.
Results and discussion. Lesions of one anatomical area occurred in 54 people (60.7%), lesions of two or more anatomical areas – in 35 people (39.3%). Most injured patients – 61 (68.5%), required from 2 to 6 surgeries, which was due to damage to various anatomical areas or the need for phased surgical interventions, including the use of damage-control surgery. Of the 66 patients with limb and pelvic bone injuries, mine explosions or gunshot fractures of the long bones of arms and legs, 41 were injured, and all were treated on an orthopedic basis. Damage-control surgery for injuries of abdominal organs was used in 5 wounded patients. In two patients, damage-control surgery was used for injuries of the pleural cavity. 9 patients (10.1%) of the 89 hospitalized with traumatic injuries received as a result of hostilities died.
Conclusion. Extremities and pelvic bones injuries dominate in the structure of combat injuries – 66 (43.7%) patients; chest injuries were in 31 (20.5%) patients; head and neck injuries in 27 (17.9%) patients; abdominal solid organs and pelvic organs injuries in 19 (12.6%) patients; facial injuries in 8 (5.3%) patients. Damage of two or more anatomical areas occurred in 39.3% of patients affected by the hostilities. Most of the wounded patients – 68.5%, required from 2 to 6 surgeries.
PREVENTION OF PULMONARY COMPLICATIONS IN THORACIC ANESTHESIOLOGY
Authors: SYDIUK O.E. , SYDIUK A 
Published: May 2022

Introduction. Pulmonary complications are one of the most common after surgery on the thoracic cavity. Although the incidence of pulmonary complications is gradually decreasing, recent studies have shown that they still occur in 26% -38% of cases. Pulmonary complications are also a major cause of hospital mortality and may be an independent risk factor for worse long-term survival [1, 2, 3].
The goal of the work. Therefore, the aim of the work was to improve the results of treatment of patients after thoracic surgery by developing an algorithm for anesthesia for perioperative prevention of pulmonary complications.
Materials and methods. The study was performed on 192 patients with diseases of the thoracic cavity (esophagus, lungs, mediastinum), underwent surgery in the thoracoabdominal department of the Shalimov’s National Institute of Surgery and Transplantation. Retrospective control group – 96 patients after thoracic surgery, which used conventional methods of perioperative management. The study group – 96 patients after thoracic surgery, which used perioperative anesthesia algorithm for the prevention of pulmonary complications. Statistical software EZR v. 1.54 was used for statistical calculations (graphical user interface for statistical software R version 4.0.3, R Foundation for Statistical Computing, Vienna, Austria).
Results and discussion. One-factor analysis revealed a relationship (p = 0.001) of the risk of complications with the method of prevention of pulmonary complications. When conducting a multifactor analysis, it was found that the proposed method of prevention of pulmonary complications can reduce (p = 0.001) the risk of development, HR = 0.27 (95% CI 0.13-0.58) compared with the control group (with standardization by sex, weight, ASA of the patient). Pulmonary complications developed in 33 (34.4%) patients of the control group and in 13 (13.5%) patients of the study group, the difference was statistically significant, p = 0.001. Thus, the use of the proposed technique reduces (p = 0.001) the risk of complications, HR = 0.39 (95% CI 0.22 - 0.70) compared to traditional methods.
Conclusions. Thus, the developed algorithm for the prevention of pulmonary complications after thoracic surgery has significantly improved the results of surgical treatment of patients by reducing the number and reducing the risk of these complications by 2.5 times (from 34.4% in patients in the control group to 13.5% in patients in the study group).
Assessment of mortality and hospital admissions associated with confirmed infection with SARS-CoV-2 Alpha variant: a matched cohort and time-to-event analysis, England, October to December 2020
Authors: Dabrera Gavin , Allen Hester , Zaidi Asad , Flannagan Joe , Twohig Katherine , Thelwall Simon , Marchant Elizabeth , Aziz Nurin Abdul , Lamagni Theresa , Myers Richard , Charlett André , Capelastegui Fernando , Chudasama Dimple , Clare Tom , Coukan Flavien , Sinnathamby Mary , Ferguson Neil , Hopkins Susan , Chand Meera , Hope Russell , Kall Meaghan ,   on behalf of the  COG-UK Consortium
Published: May 2022

Background
The emergence of the SARS-CoV-2 Alpha variant in England coincided with a rapid increase in the number of PCR-confirmed COVID-19 cases in areas where the variant was concentrated.

Aim
Our aim was to assess whether infection with Alpha was associated with more severe clinical outcomes than the wild type.

Methods
Laboratory-confirmed infections with genomically sequenced SARS-CoV-2 Alpha and wild type between October and December 2020 were linked to routine healthcare and surveillance datasets. We conducted two statistical analyses to compare the risk of hospital admission and death within 28 days of testing between Alpha and wild-type infections: a matched cohort study and an adjusted Cox proportional hazards model. We assessed differences in disease severity by comparing hospital admission and mortality, including length of hospitalisation and time to death.

Results
Of 63,609 COVID-19 cases sequenced in England between October and December 2020, 6,038 had the Alpha variant. In the matched cohort analysis, we matched 2,821 cases with Alpha to 2,821 to cases with wild type. In the time-to-event analysis, we observed a 34% increased risk in hospitalisation associated with Alpha compared with wild type, but no significant difference in the risk of mortality.

Conclusion
We found evidence of increased risk of hospitalisation after adjusting for key confounders, suggesting increased infection severity associated with the Alpha variant. Rapid assessments of the relative morbidity in terms of clinical outcomes and mortality associated with emerging SARS-CoV-2 variants compared with dominant variants are required to assess overall impact of SARS-CoV-2 mutations.