bluuebunny/crossref_metadata_2025_split huggingface dataset:
OA03 “I can't stand this pain anymore. Please chop my leg off.” Is amputation a therapeutic option for Complex Regional Pain Syndrome in children and young people?
Authors: Rogers Valerie , Connell Hannah , Davies Karen , Gauntlett-Gilbert Jeremy 
Published: September 2022

Abstract

Introduction/Background
Complex Regional Pain Syndrome (CRPS) is a chronic intensified localised pain condition. Symptoms may include limb pain, allodynia; hyperalgesia; swelling and/or changes in skin colour of the affected limb; dry, mottled skin; hyperhidrosis and trophic changes of the nails and hair. There is no known cure. Management involves analgesic medication and intensive multidisciplinary pain rehabilitation. Some cases however seem intractable, with little or no response to therapy. Some patients feel amputation of the affected limb is the only possible option. We present four cases of children and young adults requesting amputation for CRPS.

Description/Method
Case 1: 13 years, CRPS left leg, with associated gross oedema, ulceration and high risk of sepsis.
Case 2: 16 years, CRPS both feet and ankles, ulceration, fixed dystonic posturing bilaterally, Autistic Spectrum Disorder
Case 3: 17 years, mild cerebral palsy affecting left side, passionate about sport, development of CRPS following surgery for tendon release right hip flexors, fixed flexion contractures at right hip and knee.
Case 4:17 years, high functioning athlete, CRPS initially foot and ankle, contractures ankle, knee and hip; progression to involve other leg.
In each of these cases, prolonged intensive therapeutic interventions have not been successful in restoring function or reducing pain. All have requested amputation of the affected limb(s).

Discussion/Results
The tabloid press has reported the transforming success of amputation for some cases of CRPS, describing individuals who have had CRPS which has not responded to treatment finally having the affected limb amputated, and subsequently achieving great things - including winning medals in the Para-Olympics. Unsurprisingly such success stories provide hope for some enduring CRPS and becomes part of their ‘evidence’ for requesting amputation.
The scientific literature however is sparce, and virtually non-existent in children and young people. The risks of ongoing pain in the stump; recurrence of CRPS in the amputated limb - or elsewhere; phantom limb pain and inability to tolerate a prosthesis are well documented in the adult literature that is available.

Key learning points/Conclusion
Despite this, for the child/young person experiencing not only pain, but disgust, shame and body perception disturbance, the argument for amputation can be strong, and has to be considered. We present the evidence to date and share the complexity of such discussions, focusing on the experience gained from these cases.
P02 ThinkJIA: A campaign to raise awareness that children and young people get arthritis, enabling families and frontline health professionals to recognise symptoms and refer to specialist services
Authors: Beesley Richard 
Published: September 2022

Abstract

Introduction/Background
Awareness that Children and Young People (CYP) may develop arthritis (Juvenile Idiopathic Arthritis, JIA) is low within the general population and amongst non-rheumatology frontline health professionals. This can lead to delays in diagnosis and reduced access to treatment, with consequential risk of joint damage and permanent eye complications through undiagnosed JIA-associated uveitis. The aim of this work was to develop a campaign to enable frontline health professionals and families to be aware that CYP can develop arthritis, to know the main signs and symptoms, and to pursue early referral to speciality services.

Description/Method
An awareness-raising campaign was developed by UK charity Juvenile Arthritis Research working with parents of CYP with JIA, adults with JIA, teachers, campaigners, paediatric rheumatologists, ophthalmologists, and other interested lay and professional individuals. The campaign, called #ThinkJIA, includes postcards with key messages and a supporting website, www.thinkjia.org
Resources are aimed at both the general population, to help improve recognition of the signs and symptoms and encourage early attendance at primary care health services; and at non-rheumatology frontline health professionals to support recognition of JIA symptoms and the need for prompt referral. Whilst the key messaging is common to both audiences, the language used differs to reflect the lay and medical professional expectations.
Once draft campaign resources were developed, they were shared with clinical professionals and tested by parents and school teachers.

Discussion/Results
Of the pilot group of parents and teachers receiving draft resources, 100% of parents and 68% of teachers agreed that they would feel confident in seeking medical attention if their child, or a child in their school, started showing signs of JIA. Following feedback from recipients in the pilot group resources have been updated with clearer imagery. These are also supported by posters for use in health clinics and general circulation.

Key learning points/Conclusion
The development of the #ThinkJIA awareness-raising resources has helped ensure frontline health professionals and the general population can have access to information about JIA. This can help facilitate early referral and improved access to treatment. The broad rollout of awareness resources is essential to appropriately support CYP with JIA and ensure they receive the treatment they require promptly.

Acknowledgements
Thanks to Prof Helen Foster for her vital role in the initial development of these resources, and to all those involved in the development and pilot testing.
P49 The Barbara Ansell National Network for Adolescent Rheumatology – 10 years on
Authors: Yorke Sarah , Harbottle Victoria , McErlane Flora , Tattersall Rachel , McDonagh Janet 
Published: September 2022

Abstract

Introduction/Background
Over the last 10 years since BANNAR was established, there has been increasing awareness of the specific age and developmental needs of adolescents and young adults (AYA) with rheumatic disease. The aim of this study was to describe the current BANNAR membership to ensure representativeness and determine clinical and research capacity to inform future strategies.

Description/Method
An online survey on Microsoft Forms (version 2021) was developed, piloted with the BANNAR leadership team and then disseminated to existing members in addition to any new members as part of the membership process.

Discussion/Results
As of May 2022, there are 118 members. The BANNAR membership includes a wide variety of roles, from healthcare professionals reflecting the multidisciplinary team to charity representatives, academics, and researchers. Of the clinically active professionals (n = 93) there are more members working in the paediatric (n = 57) compared to the adult care setting (n = 34). There are 15 members in training posts, 16 members in research-only roles, and 9 charity representatives. Membership is predominantly female with a gender ratio of members F104: M14.
Members are predominantly White British (n = 81, 69%) and include representation from all regions of England and the devolved nations in addition to 1 researcher based in Australia. 27/116 (23.3%) members reported being currently involved in AYA research with 2 non-respondents. This research was wide ranging and involved both basic science as well as clinical research. Of the NHS clinicians (excluding the clinical academics), 34/79 (43%) had research in their job description and/or job plan.
Of 56 paediatric clinicians, 34 had access (61%) to a dedicated adolescent clinic in the paediatric setting. 37/93 NHS clinicians (40%) had access to a young adult clinic in adult services.
Of the 93 clinically active members, only 15 (16%) members reported regular in-service training for team members in AYA topics. 23/93 (25%) clinician members used/signposted team members to the Adolescent Health e-learning project (www.e-lfh.org.uk) but 55/93 (59%) reported that they were unaware of this resource.

Key learning points/Conclusion
BANNAR is a national network with membership reflecting the multidisciplinary team in addition to researchers and charity representatives. As such, along with its youth advisory panel – Your Rheum – it is an excellent resource to support future AYA rheumatology research. However, there remain challenges for clinicians in contributing to research, with less than half having formal provision for research in their job descriptions. Furthermore, even in this committed group of AYA-focussed individuals, there was not universal access to adolescent and/or young adult clinics and there are still areas for development in AYA rheumatology training.
P65 A patient-led survey into the benefits and limitations of telemedicine appointments for assessing children and young people with rheumatic conditions: Comparing European and Canadian cohorts
Authors: Beesley Richard , Angevare Saskya , Wilson Jennifer , Costello Wendy 
Published: September 2022

Abstract

Introduction/Background
During the COVID-19 (coronavirus) pandemic, some provision of healthcare shifted to remote, technology-assisted appointments (telemedicine). Whilst parents/carers of children and young people with rheumatic conditions have reported benefits of telemedicine, concerns remain.
This patient and parent-led project sought to understand the views of parents/carers about telemedicine, identifying the benefits and limitations of remote technology-assisted appointments, and comparing views between Canadian and European cohorts.

Description/Method
An online survey was developed, translated into multiple languages and shared via social media and patient organisations, targeted at parents of children and young people with rheumatic, autoimmune and autoinflammatory conditions. Fieldwork took place in April 2021 in Europe and May 2021 in Canada. Consent was provided during enrolment.

Discussion/Results
A total of 290 responses were received (133 Europe; 157 Canada; 73% female, median age 12).
Over half of respondents (53%) in Europe reported travelling over an hour to in-person appointments with their paediatric rheumatologist, compared to a significantly higher proportion of respondents in Canada (87%). Consequently, in-person appointments represent a greater time burden amongst Canadian caregivers, though both groups report appointments taking over three hours in total (51% Europe, 69% Canada).
Prior to COVID-19, most had never had a telemedicine appointment (92% Europe, 95% Canada). Since March 2020, the majority (71% Europe, 82% Canada) had at least one telemedicine appointment.
Table 1 shows the scores (1 worst, 5 best) given by parents about their telemedicine experience. Overall, most aspects scored positively (p&lt;.05). However, parents felt telemedicine was not as good as in-person appointments.

Key learning points/Conclusion
Overall respondents said they would prefer the next appointment to be in-person (82% Europe, 62% Canada, p&lt;.05), although 31% from Canada were amenable to a combination of in-person and telemedicine-based care.
There are advantages to telemedicine, notably saving time and making appointments accessible. Families from Canada tended to view telemedicine more favourably than those from Europe, although the majority from both cohorts reported concerns about the ability to assess their child. There may be value in providing training to parents to enhance the accuracy of home-based assessments, particularly when the disease is stable. However, parents continue to report the value of in-person appointments.
Post–cardiac arrest Sedation Promotes Electroencephalographic Slow-wave Activity and Improves Survival in a Mouse Model of Cardiac Arrest
Authors: Ikeda Takamitsu , Amorim Edilberto , Miyazaki Yusuke , Kato Risako , Marutani Eizo , Silverman Michael G. , Malhotra Rajeev , Solt Ken , Ichinose Fumito 
Published: September 2022

Background
Patients resuscitated from cardiac arrest are routinely sedated during targeted temperature management, while the effects of sedation on cerebral physiology and outcomes after cardiac arrest remain to be determined. The authors hypothesized that sedation would improve survival and neurologic outcomes in mice after cardiac arrest.

Methods
Adult C57BL/6J mice of both sexes were subjected to potassium chloride–induced cardiac arrest and cardiopulmonary resuscitation. Starting at the return of spontaneous circulation or at 60 min after return of spontaneous circulation, mice received intravenous infusion of propofol at 40 mg · kg–1 · h–1, dexmedetomidine at 1 µg · kg–1 · h–1, or normal saline for 2 h. Body temperature was lowered and maintained at 33°C during sedation. Cerebral blood flow was measured for 4 h postresuscitation. Telemetric electroencephalogram (EEG) was recorded in freely moving mice from 3 days before up to 7 days after cardiac arrest.

Results
Sedation with propofol or dexmedetomidine starting at return of spontaneous circulation improved survival in hypothermia-treated mice (propofol [13 of 16, 81%] vs. no sedation [4 of 16, 25%], P = 0.008; dexmedetomidine [14 of 16, 88%] vs. no sedation [4 of 16, 25%], P = 0.002). Mice receiving no sedation exhibited cerebral hyperemia immediately after resuscitation and EEG power remained less than 30% of the baseline in the first 6 h postresuscitation. Administration of propofol or dexmedetomidine starting at return of spontaneous circulation attenuated cerebral hyperemia and increased EEG slow oscillation power during and early after sedation (40 to 80% of the baseline). In contrast, delayed sedation failed to improve outcomes, without attenuating cerebral hyperemia and inducing slow-wave activity.

Conclusions
Early administration of sedation with propofol or dexmedetomidine improved survival and neurologic outcomes in mice resuscitated from cardiac arrest and treated with hypothermia. The beneficial effects of sedation were accompanied by attenuation of the cerebral hyperemic response and enhancement of electroencephalographic slow-wave activity.

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Local treatment in patients with hereditary breast cancer: decision-making process in low-, moderate-, high-penetrance pathogenic germline mutation carriers
Authors: Sibilio Andrea , Curcio Annalisa , Toesca Antonio , Rossi Elisabetta Maria Cristina , Corso Giovanni 
Published: September 2022

Purpose of review
We summarize recent evidence regarding commonly tested breast cancer susceptibility genes and review indications derived from recently published guidelines regarding management of carriers affected by early breast cancer (BC).

Recent findings
Management of affected women with a known genetic predisposition to BC was matter of debate at the most relevant international conferences, such as St. Gallen International Consensus Conference and San Antonio Breast Cancer Symposium held both in 2021. At the same time, a joint Experts Panel from American Society of Clinical Oncology/American Society for Radiation Oncology/Society of Surgical Oncology (ASCO/ASTRO/SSO) convened to develop recommendations to support clinical decision-making in this specific setting and results about administration of new systemic therapies such as poly adenosine diphosphate-ribose polymerase (PARP) inhibitors became available.

Summary
Population of patients affected by BC and carriers of mutations in susceptibility genes is progressively increasing, but new mutations identified do not always have a clear clinical impact.
To date, we have data to support consideration of different local management choices for affected patients carrying specific mutations, but some issues especially relating to breast-conserving surgery or administration of radiotherapy in these patients, still need to be better addressed. Opinions about the best way to treat these patients are still heterogeneous and information deriving from different sources seems to be conflicting at times. Our purpose is to offer a synopsis of the different evidence available that may be helpful in clinical decision making.
Pragmatic platform trials to improve the outcome of patients with acute kidney injury
Authors: Tallarico Roberta T. , Neto Ary S. , Legrand Matthieu 
Published: September 2022

Purpose of review
There is an important need for improved diagnostic strategies and treatment among patients with acute kidney injury (AKI). Classical randomized clinical trials have generated relevant results in AKI but are associated with shortcomings, such as high costs and sometimes lack of generalizability. In this minireview, we discuss the value and limits of pragmatic trials and platform trials for AKI research.

Recent findings
The implementation of pragmatic and platform trials in critical care settings has generated relevant clinical evidence impacting clinical practice. Pragmatic and platform designs have recently been applied to patients at risk of AKI and represent a crucial opportunity to advance our understanding of optimized treatment and strategies in patients at risk of AKI or presenting with AKI. Trials embedded in electronic health records can facilitate patient enrollment and data collection. Platform trials have allowed for a more efficient study design. Although both pragmatic and platform trials have several advantages, they also come with the challenges and shortcomings discussed in this review.

Summary
Pragmatic and platform trials can provide clinical answers in ‘real-life’ settings, facilitate a significant sample size enrollment at a limited cost, and provide results that can have a faster implementation in clinical practice.
Clinical Application of Allograft Bone of Alveolar Cleft Repair
Authors: Chen Kaige , Zhang Qingsu , Chen Renji , Guo Siyuan , Lin Yunan 
Published: September 2022

Objectives:
To investigate the osteogenic effect of allograft bone (BIO-GENE) and autologous iliac crest bone graft in the alveolar cleft repair.

Materials and Methods:
A total of 131 patients with congenital unilateral alveolar cleft who consulted in our hospital from January 2016 to May 2021 were selected and divided into 3 groups according to the different bone restoration materials used. Group A totaling 43 cases was the autologous bone group; group B totaling 41 cases was the BIO-GENE group; and group C totaling 47 cases was the BMP-2+BIO-GENE group. The preoperative and postoperative cone beam CT data of the same patient were imported into MIMICS 21.0 in DICOM format. The preoperative cleft volume and newly formed bone volume were calculated by 3-dimensional reconstruction to measure their osteogenic rate.

Results:
The differences in osteogenesis rates were not statistically significant in the group B compared with the group A, and in the group C compared with the group B (P>0.05 for both). The differences in osteogenesis rates were statistically significant in the group C compared with the group A (P=0.003).

Conclusions:
Comparative studies found the allograft bone with an ideal artificial material to repair alveolar clefts has become possible. Meanwhile, the addition of BMP-2 in the allograft bone significantly increased the osteogenic rate.
Surgical Approaches and Outcomes of Short Nose Correction: A Descriptive Systematic Review of the Literature
Authors: Alqabbani Almaha A. , Alqabbani Alhanouf , Mulafikh Dhaifallah S. , Alanazi Hani M. , Alhussien Ahmed , Alshehrhi Wala A. , Obeid Amani A. , Alarfaj Ahmed M. 
Published: September 2022

Objectives:
The purpose of this study is to describe all published techniques of short nose correction released by surgeons over the years and summarize their outcomes in a descriptive systematic review of the literature.

Patients and Methods:
A computerized literature search was conducted using 5 major databases. All original studies discussing correction methods and/or outcomes of shortened noses were included. The risk of bias was evaluated blindly by 2 reviewers, and the data were descriptively presented. The study protocol was recorded in the International Prospective Register of Systematic Reviews.

Results:
A total of 24 studies including 1450 patients were included. Lengthening of the nose was performed through a cartilage-only graft in 14 studies, whereas bone graft was used in 3 studies. The mean increase in nasal tip projection in 5 reports ranged from 0.05 to 2.2 mm. The mean nasal length before and after rhinoplasty was reported in 8 studies, with a mean increase ranging from 0.28 to 6.2 mm. Regarding surgical complications, 30 patients had implant deviation or migration, 22 patients experienced postoperative infection, and 12 patients required corrective surgery.

Conclusions:
Augmentation of a short nose is a challenging surgical procedure for rhinoplasty. There are several techniques for short nose correction; however, it is difficult to evaluate the outcomes in the absence of a standard tool of assessment. Further analytical studies are warranted to fully evaluate surgical techniques.
Markovian Structures in Modelling COVID-19
Authors: Cui Elvis Han , Wong Weng Kee 
Published: September 2022

AbstractThe aim of this paper is to model COVID-19 based on Markov chains. First, we introduce basic concepts of Markov chains with examples from different disciplines. Second, we use different types of Markov chains to model COVID-19, including confirmed cases, death and recovered cases and forecasting future confirmed cases. Third, we give conclusions based on these models and ideas for future work.
Supra-second interval timing deficits and abnormal frontal theta oscillations in individuals with bipolar disorder
Authors: Müller Ewald Victόria A. , Trapp Nicholas T. , Sarrett McCall E. , Pace Benjamin D. , Richards Jenny G. , Gala Ilisa K. , Miller Jacob N. , Wessel Jan R. , Magnotta Vincent A. , Wemmie John A. , Boes Aaron D. , Parker Krystal L. 
Published: September 2022

AbstractObjectivesThough widely reported by patients, cognitive symptoms associated with bipolar disorder (BD), including deficits in executive function, memory, attention, and timing are under-studied. Work suggests that individuals with BD show impairments in sub-second interval timing tasks (ITT), however, results have been inconclusive regarding supra-second time perception in BD patients. Additionally, the effects of mood or medication status on time perception in BD patients are debated in the literature.MethodsTo address this, the present work administered a supra-second ITT concurrent with electroencephalographic (EEG) recordings to patients with BD and neuronormative controls. As this task is known to elicit frontal theta oscillations, which can be abnormal in psychiatric populations, signal from the Fz lead was analyzed at rest and during the task.Results & ConclusionsAs hypothesized, results suggest that individuals with BD show impairments in supra-second ITT performance compared to neuronormative controls. Frontal theta power was also reduced compared to controls during the ITT but not during rest. Finally, timing impairments remain detectible in BD patients independent of mood state and use of antipsychotic medication. This suggests that supra-second interval timing deficits are a key characteristic observed in BD. Together with previous work, these findings point to critical timing impairments in BD patients across a wide range of timing modalities and durations.