Utilization of the 2017 diagnostic criteria for hEDS by the Toronto GoodHope Ehlers-Danlos syndrome clinic: A retrospective review.

The new 2017 diagnostic criteria for hypermobile Ehlers-Danlos Syndrome (hEDS) provide a framework for diagnosing hEDS but are more stringent than the previous Villefranche criteria. Our clinical experience at the GoodHope EDS clinic was that the 2017 criteria left many highly symptomatic patients without a diagnosis of hEDS. We conducted a retrospective cohort study to confirm our clinic experience and assess the accuracy of the 2017 diagnostic criteria for hEDS in patients who had a previous hEDS diagnosis based on the Villefranche criteria. Our study found that 15% (n = 20 of 131) of patients with a prior diagnosis of hEDS met the 2017 diagnostic criteria, and many of the traits used to distinguish hEDS were not significantly more frequent in patients who met 2017 criteria versus those who did not. In both groups objective systemic manifestations were found less frequently than subjective systemic manifestations. Beighton score (BS) as assessed by primary care practitioner was found to be higher than assessment by EDS practitioner in 81% (n = 74 of 91) of cases. Generalized joint hypermobility was confirmed in only 46% (n = 51 of 111) of patients who had a previous diagnosis of hEDS. Higher BS did not correlate with increased number of systemic manifestations in our cohort. Common comorbidities of hEDS were found with similar frequency in those who met 2017 criteria and those who did not. Based on our cohort, the 2017 hEDS diagnostic criteria require refinement to improve its diagnostic accuracy.

Exercise and Rehabilitation in People With Ehlers-Danlos Syndrome: A Systematic Review.

Objective: To conduct a systematic review examining the effect of exercise and rehabilitation in people with Ehlers-Danlos syndrome (EDS). Data Sources: The following databases were systematically searched: MEDLINE, MEDLINE In-Process/ePubs, Embase, Cochrane Central Register of Controlled Trials, PsycINFO, and Cumulative Index to Nursing and Allied Health. The final time point captured by the search is November 27, 2020. Study Selection: Eligible study designs included case-control, case-series, prospective cohort, retrospective cohort, and intervention studies of structured exercise or rehabilitation interventions. Eligible populations included adults (18 years or older) with EDS (all subtypes) and hypermobility spectrum disorders. The search was restricted to articles published in English. Data Extraction: Data were extracted by 2 independent reviewers. Risk of bias was assessed using the Physiotherapy Evidence Database (PEDro) scale for randomized controlled trials (RCTs) and Risk Of Bias In Nonrandomized Studies of Interventions (ROBINS-I) for non-RCTs. Reporting quality of RCTs was assessed using the Consolidated Standards for Reporting of Trials statement with the harms extension. Reporting was guided by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Data Synthesis: The search yielded 10 eligible studies including 330 participants. The study designs included 5 RCTs, 1 cohort, 2 single-arm interventions, 1 retrospective, and 1 feasibility study. All studies showed some improvement in a physical and/or psychological outcome after the intervention period. One adverse event (nonserious) potentially related to the intervention was reported. Of the 5 RCTs, 2 were rated as high quality with low risk of bias using PEDro, and the majority of non-RCTs were rated as critical risk of bias by ROBINS-I. Conclusions: The results suggest that exercise and rehabilitation may be beneficial for various physical and psychological outcomes. Adequately powered and rigorous RCTs of exercise and rehabilitation interventions for people with EDS are needed.

Replacing Synperonic® N in the physical developer fingermark visualisation process: Pseudo-operational trial and parameter studies.

A reformulated physical developer (PD) solution has been devised to replace the use of Synperonic® N for environmental reasons. The performance of the replacement solution has proved promising in laboratory trials using planted fingermarks [1] however; this may not always represent how a reagent works on real world samples. This paper therefore explores the effectiveness of the decaethylene glycol monododecyl ether (DGME)-based PD formulation through a pseudo-operational trial. A range of naturally handled, porous substrates were processed, which totalled over 600 samples that had been previously treated with amino acid reagents (1,2-indandione (IND) or 1,8-diazafluoren-9-one (DFO) and ninhydrin). The trial was representative of the operational use of PD at the end of a processing sequence for porous exhibits. The results from the trial establish that DGME is an effective replacement detergent for Synperonic® N in PD solutions and demonstrated the added benefit of using PD as a sequential treatment. Planted mark studies to assess the parameters of the DGME-based PD formulation are also included in this paper. These studies explored the preparation, processing and storage temperature required for the solution as well as the shelf life. The effectiveness of DGME-based PD on items that have been previously wetted was also investigated. These studies show the formulation is suitable for use in an operational laboratory and is therefore an effective replacement formulation for the Synperonic® N-based PD.

Replacing Synperonic® N in the Physical Developer fingermark visualisation process: Reformulation.

The Physical Developer solution currently recommended for use in the United Kingdom for fingermark visualisation uses two surfactants: n-dodecylamine acetate (nDDAA) and Synperonic® N. Synperonic® N is covered by the EU directive 82/242/EEC, which sought to phase out chemicals with degradation products more harmful than their precursor. This study explores the replacement of Synperonic® N with alternative detergents and examines their ability to produce clear, stable solutions that are effective at developing fingermarks. The critical properties of the detergents were investigated, such as the critical micelle concentration and the hydrophilic-lipophilic balance, and planted mark comparisons were performed on promising formulations. Tween® 20 was deemed unsuitable due to the production of cloudy solutions and the requirement to age the formulation to improve effectiveness. Brij® C10 produced clear formulations; however, these were too stable causing unacceptably long exhibit processing times, and an additional preparation stage was necessary. Brij® L23, Brij® S10, Igepal® CO-630, Polyoxyethylene (10) tridecyl ether and Tergitol™ 15-S-9 also proved to be unsuccessful alternatives. Decaethylene glycol monododecyl ether (DGME) was found to be a suitable alternative to Synperonic® N and depletion series experiments suggested that a range of DGME and nDDAA detergent quantities were effective at developing marks. The processing time using DGME was similar to Synperonic® N and the most favourable ratio of reagents is proposed in this paper as a reformulated Physical Developer solution.

The GoodHope Exercise and Rehabilitation (GEAR) Program for People With Ehlers-Danlos Syndromes and Generalized Hypermobility Spectrum Disorders.

Introduction: The Ehlers-Danlos Syndromes (EDS) and Generalized Hypermobility Spectrum Disorders (G-HSD) comprise a heterogeneous group of genetic disorders of abnormal synthesis and/or maturation of collagen and other matricellular proteins. EDS is commonly characterized by manifestations such as multi joint hypermobility that can lead to musculoskeletal pains, subluxations and dislocations, fragile skin, organ dysfunction, and chronic significant diffuse pain with fatigue, deconditioning eventuating to poor quality of life. Evidence suggests exercise and rehabilitation interventions may ameliorate symptoms of unstable joints, recurrent subluxations/dislocations, and chronic widespread musculoskeletal pain. To date, there have only been a few reports describing exercise and rehabilitation care strategies for people with EDS. Methods: In this manuscript, we describe the GoodHope Exercise and Rehabilitation (GEAR) program, its overarching principles, as well as the program development and delivery model. The GEAR program aims to decrease functional impairment, reduce pain, increase confidence in symptom self-management, and provide a community of support for people with EDS/G-HSD. To achieve these goals, we detail the model of care that includes exercise and rehabilitation therapy, education for self-management, and support accessing relevant community resources. Strengths and Limitations of the Study: GEAR represents a novel exercise and rehabilitation care model for people with G-HSD and various clinical EDS subtypes, beyond the commonly included hEDS subtype. Systematic collection of data via validated measurements is ongoing and will guide the refinement of GEAR and support the development of emerging exercise and rehabilitation programs for people with EDS.

The GoodHope Ehlers Danlos Syndrome Clinic: development and implementation of the first interdisciplinary program for multi-system issues in connective tissue disorders at the Toronto General Hospital.

Ehlers-Danlos Syndrome (EDS) are a heterogeneous group of genetic connective tissue disorders, and typically manifests as weak joints that subluxate/dislocate, stretchy and/or fragile skin, organ/systems dysfunction, and significant widespread pain. Historically, this syndrome has been poorly understood and often overlooked. As a result, people living with EDS had difficulty obtaining an accurate diagnosis and appropriate treatment, leading to untold personal suffering as well as ineffective health care utilization. The GoodHope EDS clinic addresses systemic gaps in the diagnosis and treatment of EDS. This paper describes a leap forward-from lack of awareness, diagnosis, and treatment-to expert care that is tailored to meet the specific needs of patients with EDS. The GoodHope EDS clinic consists of experts from various medical specialties who work together to provide comprehensive care that addresses the multi-systemic nature of the syndrome. In addition, EDS-specific self-management programs have been developed that draw on exercise science, rehabilitation, and health psychology to improve physical and psychosocial wellbeing and overall quality of life. Embedded into the program are research initiatives to shed light on the clinical presentation, underlying mechanisms of pathophysiology, and syndrome management. We also lead regular educational activities for community health care providers to increase awareness and competence in the interprofessional management of EDS beyond our doors and throughout the province and country.

Mitochondria form contact sites with the nucleus to couple prosurvival retrograde response.

Mitochondria drive cellular adaptation to stress by retro-communicating with the nucleus. This process is known as mitochondrial retrograde response (MRR) and is induced by mitochondrial dysfunction. MRR results in the nuclear stabilization of prosurvival transcription factors such as the nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB). Here, we demonstrate that MRR is facilitated by contact sites between mitochondria and the nucleus. The translocator protein (TSPO) by preventing the mitophagy-mediated segregation o mitochonria is required for this interaction. The complex formed by TSPO with the protein kinase A (PKA), via the A-kinase anchoring protein acyl-CoA binding domain containing 3 (ACBD3), established the tethering. The latter allows for cholesterol redistribution of cholesterol in the nucleus to sustain the prosurvival response by blocking NF-κB deacetylation. This work proposes a previously unidentified paradigm in MRR: the formation of contact sites between mitochondria and nucleus to aid communication.

A validation study of the 1,2-indandione reagent for operational use in the UK: Part 1 - Formulation optimization.

This paper contains details of work carried out to examine the composition of 1,2-indandione formulations and to develop the most effective 1,2-indandione/zinc formulation for use under UK conditions. Previous research into the reactions of 1,2-indandione without zinc ions have concluded that formulations containing methanol produce stable hemiketals, which are less reactive to amino acids, resulting in reduced fluorescence intensity of developed fingermarks. In this study, fingermarks were treated using varying formulations of 1,2-indandione, with and without the presence of methanol and zinc ions. It was found that both were beneficial in producing marks of the highest fluorescence intensity, although too much methanol could have a detrimental effect on the quality the mark due to diffusion of ridge detail. Therefore the 1,2-indandione formulation recommended for further trials has been modified to contain both zinc ions and methanol.

A validation study of the 1,2-indandione reagent for operational use in the UK: Part 2 - Optimization of processing conditions.

This paper contains details of work carried out to identify the most effective processing conditions for the optimized 1,2-indandione/zn formulation developed for use under UK conditions. Using direct measurements of fluorescence taken from test spots of amino acids and eccrine sweat during oven processing, complemented with experiments on real fingermarks, it was established that processing temperatures above 120°C in the oven were detrimental to the fluorescence of the developed mark. Alternative methods of development to oven processing were found to be effective, but less controllable. High levels of humidification were also found to be detrimental to the fluorescence of 1,2-indandione developed marks, and oven processing at 100°C and 0% relative humidity is therefore recommended for further studies. It has also been shown that 1,2-indandione can develop fingermarks at temperatures as low as 20°C, making it a candidate for use at crime scenes.

Fingermark visualisation with iron oxide powder suspension: The variable effectiveness of iron (II/III) oxide powders, and Tween® 20 as an alternative to Triton™ X-100.

The effectiveness of the current UK iron oxide powder suspension formulation, 'C-IOPS-09' (Triton X-100 based), for fingermark or latent fingerprint visualization is shown to be affected by variations between batches of the recommended iron oxide powder from Fisher Scientific (I/1100/53). When incorporated into the C-IOPS-09 formulation, a 2015 powder batch resulted in the detection of ∼19% fewer fingermarks, of broadly reduced contrast, when compared to powder suspension prepared with a 2008 batch of the same product. Furthermore, the 2015 powder batch was found to be unsuitable in experimental reduced-surfactant concentration powder suspension, because it caused surface-wide or background staining. The studies in this paper also investigated the use of Tween 20 surfactant as an alternative to the currently utilised Triton X-100, in preparation for the potential unavailability of Triton X-100 in the future. Powder suspensions prepared with Tween 20 surfactant solutions of 4% and 40% were shown to offer similar effectiveness to the currently recommended C-IOPS-09 formulation, when compared using the same batch of Fisher Scientific iron oxide powder (2008 or 2015). The difference between the 2008 and 2015 iron oxide batches was hence also evident with these alternative surfactant solutions. Particle size distribution analysis of the iron oxide powders in Tween 20 and Triton X-100 based surfactant solutions show that the more effective powder exhibits a higher sub-micrometre particle population than the less effective powder. This work leads to an improved specification for powder suspension formulations. This is demonstrated with an example powder suspension formulation which uses a 10% Tween 20 surfactant solution and iron oxide nanopowder (50-100nm) from Sigma Aldrich, which was shown to visualise 27% more fingermarks than the C-IOPS-09 formulation prepared with the 2015 Fisher Scientific powder batch, in a comparative study.

To Be or Not to Be a Cardiorespiratory Physiotherapist: Factors That Influence Career Choice in a Sample of Canadian Physiotherapists.

Purpose: This study explored the factors that influence choosing or not choosing a career in cardiorespiratory physiotherapy (CRP) from the perspective of a group of currently practising, experienced physiotherapists in Canada. Methods: A modified Dillman approach was used to distribute a cross-sectional, self-administered, online questionnaire to all eligible members of the cardiorespiratory and orthopaedic divisions of the Canadian Physiotherapy Association. A total of 438 participants-21 CRP and 417 non-CRP therapists-completed the survey. The survey response rate was 9.4%. Results: A narrow scope of practice (61.9%) and a lack of interest in CRP subject matter (50.1%) were the most influential factors deterring the respondents from making CRP their career choice. Interest in CRP (81.0%), mentorship (76.2%), access to physical resources (76.2%), and inter-professional practice (71.4%) were the most influential factors in pursuing a career in CRP. Conclusion: Increasing the awareness of the scope of practice for CRP, exposure to positive mentors, and rich practice settings are key factors in promoting physiotherapists' specialisation in CRP.

Objectif : explorer les facteurs qui influent sur le choix ou le rejet d'une carrière en physiothérapie cardiorespiratoire (PCR) d'après le point de vue d'un groupe de physiothérapeutes expérimentés qui exercent déjà au Canada. Méthodologie : les chercheurs ont utilisé la méthode Dillman modifiée pour distribuer un questionnaire virtuel autoadministré transversal à tous les membres admissibles de la division cardiorespiratoire et de la division orthopédique de l'Association canadienne de physiothérapie. Au total, 438 participants, soit 21 PCR et 417 non-PCR, ont participé au sondage. Le taux de réponse s'élevait à 9,4 %. Résultats : un champ de pratique étroit (61,9 %) et le manque d'intérêt pour la PCR (50,1 %) étaient les facteurs qui dissuadaient le plus les répondants à opter pour une carrière en PCR. L'intérêt pour la PCR (81 %), le mentorat (76,2 %), l'accès aux ressources physiques (76,2 %) et la pratique interprofessionnelle (71,4 %) étaient les facteurs qui favorisaient le plus la poursuite d'une carrière en PCR. Conclusion : une plus grande sensibilisation au champ de pratique de la PCR, l'exposition à des mentors positifs et des milieux de pratique enrichissants sont des facteurs clés pour inciter les physiothérapeutes à se spécialiser en PCR.

Enhancement of the conductivity of Ba(2)In(2)O(5) through phosphate doping.

In this communication, we demonstrate the successful incorporation of phosphate into Ba(2)In(2)O(5), which leads to the conversion from an orthorhombic to a cubic unit cell. The resulting increased oxygen vacancy disorder leads to an enhancement in the oxide ion conductivity at low temperatures. In addition, in wet atmospheres, significant proton conduction is observed.