Screening for cognitive impairment in adults with sickle cell disease: A systematic review and meta-analysis.

Neurovascular disease such as symptomatic stroke, silent brain infarcts and vascular cognitive impairment are common complications of sickle cell disease (SCD) that can have devastating consequences on quality of life, employment, and social functioning.  Early recognition of neurovascular disease is a prerequisite for the timely optimization of medical care and to connect patients to adaptive resources. While cognitive impairment has been well described in children, currently available data are limited in adults. As a result, guidance on the optimal cognitive screening strategies in adults is scarce. We conducted a systematic review to identify the different screening tools that have been evaluated in SCD. A meta-analysis was performed to estimate the prevalence of suspected cognitive impairment in this population. In this qualitative synthesis, we present 8 studies that evaluated 6 different screening tools. Patient characteristics that impacted on cognitive screening performance included age, education level, and a prior history of stroke. We report a pooled prevalence of 38% [14-62%] of suspected cognitive impairment. We discuss the relative benefits and limitations of the different screening tools to help clinicians select an adapted approach tailored to their specific patients' needs. Further studies are needed to establish and validate cognitive screening strategies in patients with diverse cultural and educational backgrounds.

Developing a Web Platform to Optimize the Self-Management of People Living with a Chronic Respiratory Disease.

Purpose: The purpose of this article is to describe the development of a new Web platform to optimize self-management after pulmonary rehabilitation (PR) for persons living with a chronic respiratory disease (CRD) and to present data on its usability. Method: The Web platform is informed by a theoretical framework of behaviour changes and concepts of self-management and self-efficacy. It uses breathing exercises and a logbook and is meant to be a self-management tool. Usability was tested for 8 months after PR with a group consisting of five patients with chronic obstructive pulmonary disease and one with pulmonary fibrosis. We evaluated adherence (e.g., number of exercise/weeks), quality of life, dyspnoea, and functional capacity. We measured frequency count for adherence and pre-post differences per patient for clinical outcomes. Results: Four participants' adherence was higher than 50% of completed exercises (72 exercise/weeks). Five of six participants showed maintenance of functional capacity (6-minute walk test) 8 months after PR. Four participants showed maintenance of their quality of life. Four participants showed a deterioration in dyspnoea on the Borg Scale of Perceived Exertion. Conclusions: We developed a new theory-informed Web platform to optimize self-management after PR for persons living with a CRD. The pilot Web platform appears to optimize adherence to self-management techniques and possibly stabilize people's health outcomes.

Objectif : décrire la création d'une nouvelle plateforme en ligne pour optimiser l'autogestion après la réadaptation pulmonaire (RP) des personnes vivant avec une maladie pulmonaire chronique et présenter des données sur sa facilité d'utilisation. Méthodologie : la plateforme repose sur une structure théorique de changements de comportement et de concepts d'autogestion et d'autoefficacité. Elle se veut un outil d'autogestion faisant appel à des exercices respiratoires et à un journal. Les chercheurs en ont évalué la facilité d'utilisation auprès d'un groupe de cinq patients atteints d'une maladie pulmonaire obstructive chronique et d'un patient atteint de fibrose pulmonaire pendant huit mois après la RP. Ils ont évalué l'adhésion (p. ex., nombre d'exercices par semaine), la qualité de vie, la dyspnée et la capacité fonctionnelle. Ils ont également mesuré la fréquence pour déterminer l'adhésion et la différence avant-après de chaque patient pour évaluer les résultats cliniques. Résultats : quatre participants ont présenté une adhésion supérieure à 50 % pour ce qui est des exercices complétés (72 exercices par semaine). Quatre des six participants avaient maintenu leur capacité fonctionnelle (test de marche de six minutes) huit mois après la RP, et quatre participants avaient maintenu leur qualité de vie. Cependant, quatre participants ont présenté une détérioration de leur dyspnée à l'échelle de Borg. Conclusion : les chercheurs ont créé une nouvelle plateforme reposant sur des critères théoriques pour optimiser l'autogestion après une RP chez les personnes vivant avec une maladie pulmonaire chronique. Le projet-pilote de plateforme en ligne semble optimiser l'adhésion aux techniques d'autogestion et pourrait stabiliser l'état de santé des patients.

Risk of adrenal insufficiency following intra-articular or periarticular corticosteroid injections among children with chronic arthritis.

Objectives Intra-articular and/or periarticular corticosteroid injection (IACI) is a common procedure in pediatric rheumatology. Despite many adult studies demonstrating a significant risk of adrenal insufficiency (AI) following the procedure, very little evidence is available in the pediatric literature regarding this risk. The main goal of this study is to evaluate the prevalence of AI in children with chronic arthritis following IACI. Methods This is a retrospective study including children aged 0-18 years who had an IACI from June 2017 to July 2019. An 8:00 morning cortisol (8MC) sample was drawn around two weeks after the injection, and an ACTH 1mcg stimulation test was performed if morning cortisol level was low. AI was defined as an 8MC under 50 nmol/L or an abnormal ACTH stimulation test. Risks factors for AI and its duration were assessed. Results Sixty patients were included in this study. AI prevalence was 30% with 18 of 60 affected patients. The corticosteroid dose injected was statistically associated with the development of AI. Median duration of AI was 181 days for the nine patients who were followed up until resolution of AI. Four patients developed symptoms of AI, namely fatigue (2 of 4), nausea (2 of 4) and abdominal pain (3 of 4). None were hospitalized or died. Conclusions In this cohort of children with chronic arthritis who had an IACI, we found a high prevalence of AI. Monitoring and counseling of such complication is warranted until further evidence is available.

Coronary artery fixation at iso-arterial pressure: impacts on histologic evaluation and clinical management.

Coronary angiography is the standard imaging method for determining the site, extent, and severity of coronary artery disease. Several publications have reported discordance between the degree of coronary artery stenosis determined from post-mortem histologic evaluation and coronary angiography. While the 2-dimensional limitations of coronary angiography are well established, the determination of coronary stenosis based on histologic evaluation of passively fixed samples is also associated with significant biases. In this study, we used patients with chronic coronary artery disease to compare the stenosis severity estimates that were determined using the passive fixation method with those determined using the active fixation method. Our results showed a significant discrepancy between the stenosis in passively fixed coronary arteries when compared with coronary angiography in all major coronary vessels combined (P=.002), and in Cx (P=.045) and CD (P=.026). However, there was no mean difference when compared with perfused (actively fixed) samples when all vessels were combined or examined individually. Iso-physiologic mechanical perfusion (active) fixation yielded significantly reduced coronary artery stenosis means when compared to the passive fixation method in post-mortem evaluations during autopsies. This was evident when all vessels were combined (P=.0001) and assessed individually (Cx (P=.003), LAD (P=.025), LM (P=.056) and RC (P=.007)). Autopsies including cardiac explant patients also showed differences in estimates for all vessels combined (P=.0001) and in Cx (P=.016) and RC (P=.006). In summary, our quantitative histopathology analyses using perfused coronary artery stenosis at physiologic pressure showed significant discrepancies when compared with passive histopathology.