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Background: Mitochondrial DNA (mtDNA) depletion syndromes (MDDS) are genetically and clinically variable disorders resulting from a reduction in mtDNA content in the cells, tissues, and organ systems, leading to symptoms related to energy deficits. Deficiency of the mitochondrial succinyl-CoA ligase/synthetase enzyme secondary to pathogenic variations in the SUCLG1 and SUCLA2 genes is a subtype of MDDS that presents with neurological manifestations and a specific biochemical profile. Methods: This cross-sectional series describes five patients with MDDS secondary to pathogenic variations in the SUCLG1 and SUCLA2 genes from two tertiary care centers in Canada and India. Clinical data concerning the course, investigations, and outcome were gathered through chart reviews. Results: All subjects presented in early infancy with neurological manifestations, including movement disorder, psychomotor regression, developmental delay, hearing loss, behavioral issues, or a combination thereof. Elevated methylmalonic acid metabolites, an abnormal acylcarnitine profile, and lactic acidemia were noted in the biochemical profile of each patient (n = 5/5, 100%). Molecular genetic testing disclosed the presence of pathogenic homozygous mutations in four subjects and compound heterozygosity in one subject. Conclusion: MDDS associated with SUCLG1 and SUCLA2 genes can be detected biochemically by the presence of methylmalonic aciduria besides the elevation of lactate, C3, C4DC, and C5-OH acylcarnitine. Conducting metabolic workups including MMA and acylcarnitine profiles in patients with heterogeneity of clinical symptoms associated with the presence of this biochemical marker may potentially reduce the time to diagnosis and management. [ABSTRACT FROM AUTHOR]

Background: The coronavirus disease 2019 (COVID-19) pandemic has highlighted the need to improve the safety of the environments where we care for older adults in Canada. After providing assistance during the first wave, many Ontario hospitals formally partnered with local congregate care homes in a "hub and spoke" model during second pandemic wave onward. The objective of this article is to describe the implementation and longitudinal outcomes of residents in one hub and spoke model composed of a hospital partnered with 18 congregate care homes including four long-term care and 14 retirement or other congregate care homes. Intervention: Homes were provided continuous seven-day per week access to hospital support, including infection prevention and control (IPAC), testing, vaccine delivery and clinical support as needed. Any COVID-19 exposure or transmission triggered a same-day meeting to implement initial control measures. A minimum of weekly on-site visits occurred for long-term care homes and biweekly for other congregate care homes, with up to daily on-site presence during outbreaks. Outcomes: Case detection among residents increased following implementation in context of increased testing, then decreased post-immunization until the Omicron wave when it peaked. After adjusting for the correlation within homes, COVID-related mortality decreased following implementation (OR=0.51, 95% CI, 0.30-0.88; p=0.01). In secondary analysis, homes without pre-existing IPAC programs had higher baseline COVID-related mortality rate (OR=19.19, 95% CI, 4.66-79.02; p<0.001) and saw a larger overall decrease during implementation (3.76% to 0.37%-0.98%) as compared to homes with pre-existing IPAC programs (0.21% to 0.57%-0.90%). Conclusion: The outcomes for older adults residing in congregate care homes improved steadily throughout the COVID-19 pandemic. While this finding is multifactorial, integration with a local hospital partner supported key interventions known to protect residents. [ABSTRACT FROM AUTHOR]

Objective: Epilepsy associated with tuberous sclerosis complex (TSC) can be challenging to treat and is associated with significant disease burden. Our objective was to better understand the state of epilepsy care of TSC amongst pediatric neurologists in Canada, identify gaps in care and determine whether access to a dedicated TSC clinic has an impact on epilepsy management. Methods: A survey was developed after a literature review and discussion amongst two pediatric epileptologists and one nurse practitioner with expertise in TSC about the state of epilepsy care of TSC patients in Canada. Canadian pediatric neurologists were asked to participate in sharing their experiences via an anonymous web‐based survey through the Canadian League Against Epilepsy (CLAE) and the Canadian Neurological Sciences Federation (CNSF). Results: Fifty‐seven responses were received. Access to a dedicated TSC clinic was reported by 25% (n = 14). Sixty percent (n = 34) reported performing serial EEG monitoring in infants with TSC and 57% (n = 33) started prophylactic antiseizure therapy when EEG abnormalities were detected, regardless of whether there was access to a TSC clinic (P =.06 and P =.29, respectively). While 52% (n = 29) did not feel comfortable prescribing mTORi for epilepsy, 65% (n = 36) indicated they would consider it with additional training. Epilepsy surgery was offered in 93% (n = 13) of centers with a dedicated TSC clinic but only 45% of centers without a TSC clinic (n = 19) (P =.002). Significance: Our findings demonstrate the variability in neurological care of pediatric patients with TSC as it pertains to epilepsy management. There is a need for the establishment of epilepsy practice guidelines and a national network to support clinical practice, research, and education. [ABSTRACT FROM AUTHOR]

For much of the industrialized world, pollution from manufacturing has been falling despite increased output. We examine how air quality standards---a common environmental regulation---have contributed to this cleanup of manufacturing. We develop a general equilibrium model to show how air quality standards can lead to a cleanup by causing reductions in plant emission intensity, relative changes in plant output, and plant entry and exit. We provide quasi-experimental evidence from Canada to highlight the magnitude of these responses. Our results suggest that air quality standards explain just under 40% of the cleanup of manufacturing. [ABSTRACT FROM AUTHOR]

The article presents an analysis of the ways in which Arab Canadians have fought with the discriminating discourses that have been circulating with different intensities since the late 1960s and early 1970s, when the first wave of Arab immigrants came to Canada, and through the aftermath of the September 11, 2001 terrorist attacks. The Arab Canadians featured in the book "Royal Canadian Jihad," by Patrick Grady, the documentary film "Being Osama," by Mahmoud Kaabour, about the post-9/11 experiences of six Arab Canadians who share the name "Osama," and the film "Sabah: A Love Story," directed by Ruba Nadda, are explored. The author argues that Arab Canadians' lack of alternatives for voicing their discontent led to their confronting Arabness with the experiences of everyday Arabs.

Rigorous sampling and quality assurance protocols are required for the reliable measurement of personal, indoor and outdoor exposures to metals in fine particulate matter (PM2.5). Testing of five co-located replicate air samplers assisted in identifying and quantifying sources of contamination of filters in the laboratory and in the field. A field pilot study was conducted in Windsor, Ont., Canada to ascertain the actual range of metal content that may be obtained on filter samples using low-flow (4Lmin−1) 24-h monitoring of personal, indoor and outdoor air. Laboratory filter blanks and NIST certified reference materials were used to assess contamination, instrument performance, accuracy and precision of the metals determination. The results show that there is a high risk of introducing metal contamination during all stages of sampling, handling and analysis, and that sources and magnitude of contamination vary widely from element to element. Due to the very small particle masses collected on low-flow 24-h filter samples (median 0.107mg for a sample volume of approximately 6m3) the contribution of metals from contamination commonly exceeds the content of the airborne particles being sampled. Thus, the use of field blanks to ascertain the magnitude and variability of contamination is critical to determine whether or not a given element should be reported. The results of this study were incorporated into standard operating procedures for a large multiyear personal, indoor and outdoor air monitoring campaign in Windsor. [Copyright &y& Elsevier]

Background: Multiple sclerosis is the most important cause of non-injury-related disability in young adults. The disease has unknown causes and distresses and affects daily activities. While therapeutic interventions mainly focus on body function and structure to reduce impairment, Cognitive Orientation to Daily Occupational Performance (CO-OP) is a cognitive approach that provides intervention at the level of activity and participation., Purpose: We aim to examine the effects of CO-OP approach on fatigue, quality of life, occupational performance, and satisfaction in people living with multiple sclerosis; and to examine whether they could transfer strategies and skills learned during CO-OP to untrained goals., Methods: A pre-post design was used. Assessment tools included Montreal Cognitive Assessment, Multiple Sclerosis Impact Scale, Fatigue Impact Scale and the Canadian Occupational Performance Measure. Six individuals living with multiple sclerosis participated in 10 CO-OP sessions twice a week. The sessions were held in an environment of the participants' choice, along with an extra session added to determine the goals. The study was registered in the ethics committee of University of Social Welfare and Rehabilitation Sciences (IR.USWR.REC.1399.089)., Results: The performance improved (2-point positive change) in 12 out of 18 trained goals and in three out of six untrained goals (self-report). The improvement was maintained at a 3-month follow-up assessment. There was a statistically significant difference in Canadian Occupational Performance Measure (χ 2  = 11.565, p = 0.003 same for performance and satisfaction scores), Fatigue Impact Scale (χ 2  = 7.000, p = 0.030), and Multiple Sclerosis Impact Scale scores over time (χ 2  = 9.478, p = 0.009) respectively., Conclusion: The CO-OP approach has the potential to improve daily activity performance, reduce pain, and improve the quality of life for people living with multiple sclerosis. A definitive randomised controlled trial is required., (© 2023 Occupational Therapy Australia.)