Leading well and staying psychologically healthy: the role of resources and constraints for managers in the healthcare sector.

PURPOSE: Maintaining a healthy and productive workforce is a challenge for most organizations. This is even truer for health organization, facing staff shortages and work overload. The aim of this study is to identify the resources and constraints that influence managers' mental health and better understand how they are affected by them. DESIGN/METHODOLOGY/APPROACH: A qualitative approach was chosen to document the resources, the constraints as well as their consequences on managers in their day-to-day realities. The sample included executive-, intermediate- and first-level managers from a Canadian healthcare facility. A total of 62 semi-structured interviews were conducted. The coding process was based on the IGLOO model of Nielsen et al. (2018) to which an employee-related level was added (IGELOO). FINDINGS: Results highlight the importance of considering both resources as well as constraints in examining managers' mental health. Overarching context, organizational constraints and the management of difficult employees played important roles in the stress experienced by managers. PRACTICAL IMPLICATIONS: The results offer a better understanding of the importance of intervening at different levels to promote better organizational health. Results also highlight the importance of setting up organizational resources and act on the various constraints to reduce them. Different individual strategies used by managers to deal with the various constraints and maintain their mental health also emerge from those results. ORIGINALITY/VALUE: In addition to addressing the reality of healthcare managers, this study supplements a theoretical model and suggests avenues for interventions promoting more sustainable organizational health.

Organizational culture and leadership behaviors: is manager's psychological health the missing piece?

Background: In a context where organizations struggle to attract and retain highly qualified workers, organizations need to prioritize the psychological health of employees as a retention factor. To do so, they need to provide a healthy work environment. As an integral part of the employee experience, managers are an important factor in employee retention. In past studies, researchers have focused on the importance of leadership in boosting employees' health without, however, considering factors encouraging such behavior in managers. Recently, some scholars have become interested in managers' health as a resource allowing them to adopt good leadership behavior. Indeed, these studies reveal interesting links between managers' emotional state and their behavior as leaders. Other studies, underscore the importance of considering the organizational context to better understand managers' psychological health that may influence their leadership behaviors. This study proposes to examine the complex process by which organizational culture influences managers' psychological health, which acts as a resource favoring the adoption of good leadership behaviors that are known to be constructive and have positive effects on employee. Methods: Path analyses with the CALIS procedure SAS software, version 9.4 were conducted on a sample of 522 managers in three healthcare facilities in the province of Quebec, Canada. Results: The results revealed that group culture is associated with the two indicators of managers' psychological health at work. The results also demonstrated that managers' psychological distress at work is positively related to transactional and laissez-faire leadership styles whereas psychological well-being at work is positively related to transformational and transactional leadership. Concerning indirect associations, there is a significant and positive indirect association between group culture and transformational leadership and there is also a significant and negative association between group culture and laissez-faire leadership. Finally, there is also an indirect association between hierarchical culture and transactional leadership. Conclusion: Our study provides a more in-depth understanding of the relationship between organizational culture and leadership styles. More specifically, our findings highlight the benefits of implementing a group organizational culture to enhance psychological well-being, reduce psychological distress symptoms and promote good leadership behaviors.

TUFM variants lead to white matter abnormalities mimicking multiple sclerosis.

BACKGROUND AND PURPOSE: Defects in the mitochondrial respiratory chain (MRC) can lead to combined MRC dysfunctions (COXPDs) with heterogenous genotypes and clinical features. We report a patient carrying heterozygous variants in the TUFM gene who presented with clinical features compatible with COXPD4 and radiological findings mimicking multiple sclerosis (MS). METHODS: A 37-year-old French Canadian woman was investigated for recent onset of gait and balance problems. Her previous medical history included recurrent episodes of hyperventilation associated with lactic acidosis during infections, asymptomatic Wolff-Parkinson-White syndrome, and nonprogressive sensorineural deafness. RESULTS: Neurological examinations revealed fine bilateral nystagmus, facial weakness, hypertonia, hyperreflexia, dysdiadochokinesia, dysmetria, and ataxic gait. Brain magnetic resonance imaging (MRI) showed multifocal white matter abnormalities in cerebral white matter as well as cerebellar hemispheres, brainstem, and middle cerebellar peduncles, some of which mimicked MS. Analysis of native-state oxidative phosphorylation showed a combined decrease in CI/CII, CIV/CII, and CVI/CII. Exome sequencing detected two heterozygous TUFM gene variants. Little clinical progression was noted over a 5-year follow-up. Brain MRI remained unchanged. CONCLUSIONS: Our report broadens the phenotypic and radiological spectrum of TUFM-related disorders by adding milder, later onset forms to the previously known early onset, severe presentations. The presence of multifocal white matter abnormalities can be misinterpreted as due to acquired demyelinating diseases, and thus TUFM-related disorders should be added to the list of mitochondrial MS mimickers.

Toward a new model of human resource management practices: construction and validation of the High Wellbeing and Performance Work System Scale.

Introduction: The integrated mutual gains model suggests five provisional sets of human resource management (HRM) practices that should benefit both employees and organizations and, as such, be explicitly designed to have a positive impact on wellbeing, which, in turn, can affect performance. Methods: An extensive review of the literature on scales that used a high-performance work system to assess HRM practices, as well as an extraction of items related to the theoretical dimensions of the integrated mutual gains model, were performed. Based on these preliminary steps, an initial scale with the 66 items found most relevant in the literature was developed and assessed regarding its factorial structure, internal consistency, and reliability over a two-week period. Results: Exploratory factorial analysis following test -retest resulted in a 42-item scale for measuring 11 HRM practices. Confirmatory factor analyses resulted in a 36-item instrument for measuring 10 HRM practices and showed adequate validity and reliability. Discussion: Even though the five provisional sets of practices were not validated, the practices that emerged from them were assembled into alternative sets of practices. These sets of practices reflect HRM activities that are considered conducive to employees' wellbeing and, consequently, their job performance. Consequently, the "High Wellbeing and Performance Work System Scale" was created. Nonetheless, future research is necessary to evaluate the predictive capacity of this new scale.

One down, fifty to go: managers' perceptions of their workload and how they cope with it to maintain their psychological health.

Background: Like many other countries, healthcare services in Canada face numerous organizational changes with the main objective of doing more with less. The approach taken within different healthcare networks has brought about a reform in healthcare facilities in Quebec, leading to several mergers and eliminating over 1,000 managerial positions. As a result, this has placed a progressively heavier workload on the shoulders of the remaining managers. Research on mental health in the workplace has mainly focused with the workforce and generally neglects managers. However, studies have shown that workload is a risk factor for managers. Therefore, the objectives of our study are to (1) better understand the elements that make up a manager's workload and the factors that influence it and (2) identify the coping strategies used by managers to deal with their workloads. Methods: Employing a qualitative approach, we analyzed 61 semistructured interviews through an abductive method, utilizing diverse frameworks for data analysis. The participants came from the same Quebec healthcare establishment. Results: Our findings align with the notion that workload is a multifaceted phenomenon that warrants a holistic analysis. The workload mapping framework we propose for healthcare network managers enables pinpointing those factors that contribute to the burden of their workload. Ultimately, this workload can detrimentally impact the psychological wellbeing of employees. Conclusion: In conclusion, this study takes a comprehensive look at workload by using a holistic approach, enabling a more comprehensive understanding of this phenomenon. It also allows for the identification of coping strategies used by managers to deal with their workloads. Finally, our results can provide valuable guidance for the interventions aimed at addressing workload issues among healthcare network managers in Quebec by utilizing the specific elements we have identified.

Management behaviors during the COVID-19 pandemic: The case of healthcare middle managers.

Background: The spread of COVID-19 has disrupted the lifestyles of the world's population. In the workplace, the pandemic has affected all sectors and has changed the way work is organized and carried out. The health sector has been severely impacted by the pandemic and has faced enormous challenges in maintaining healthcare services while providing care to those infected by the virus. At the heart of this battle, healthcare managers were key players in ensuring the orchestration of operations and the physical and mental availability of employees during the crisis. Although few studies have been conducted to identify organizational practices or leadership skills to be adopted in a crisis context, the concrete behaviors of managers have not been documented yet. Therefore, this study aims at filling this gap by studying middle managers' behaviors facing COVID-19 crisis in the healthcare sector. Methods: Using a qualitative approach, eight focus groups were conducted online during the pandemic with 37 middle managers from the healthcare community of a Quebec health establishment (Canada) from April to June 2020. Thematic analyses were conducted, and a mixed-methods approach was used to analyse the data based on Viitala's hierarchical model of management skills. Results: Based on the six managerial skills proposed in the model of Viitala, 21 specific management behaviors were identified as having been deployed by middle managers at the beginning of the pandemic. Considering that the health sector has been profoundly shaken by this health crisis, in addition to being an environment likely to experience other crises, managers need to develop practical skills in various crisis management situations. Thus, the results guide practitioners by highlighting the importance of team-oriented management behaviors (leadership, supervisory competencies), especially in a crisis context.

Intermittent neurologic decompensation: An underrecognized presentation of tyrosine hydroxylase deficiency.

Tyrosine hydroxylase deficiency (THD) is a treatable inborn error of dopamine biosynthesis caused by mutations in TH. Two presentations are described. Type A, milder, presents after 12 months of age with progressive hypokinesis and rigidity. Type B presents before 12 months as a progressive complex encephalopathy. We report a girl with mild THD who had recurrent episodes of neurological decompensations. Before the first episode, she had normal development except for mild head tremor. Episodes occurred at 12, 19, and 25 months of age. After viral infections or vaccination, she developed lethargy, worsened tremor, language, and motor regression including severe axial hypotonia, recuperating over several weeks of intensive rehabilitation but with residual tremor and mild lower limb spasticity. Basal ganglia imaging was normal. Exome sequencing revealed two missense variants of uncertain significance in TH: c.1147G>T and c.1084G>A. Both have low gnomAD allele frequencies and in silico, are predicted to be deleterious. Cerebrospinal fluid analysis showed low homovanillic acid (HVA, 160 nmol/L, reference 233-938) and low HVA/5-hydroxyindolacetic acid molar ratio (1.07, reference .5-3.5). She responded rapidly to L-Dopa/carbidopa without further episodes. Literature review revealed four other THD patients who had a total of seven episodes of marked hypotonia and motor regression following infections, occurring between ages 12 months and 6 years. All improved with L-Dopa/carbidopa treatment. Intermittent THD is treatable, important for genetic counseling, and should be considered after even a single episode of marked hypotonia with recuperation over weeks, especially in patients with preexisting tremor, dystonia, or rigidity.

Picomolar Sensitivity Analysis of Multiple Bradykinin-Related Peptides in the Blood Plasma of Patients With Hereditary Angioedema in Remission: A Pilot Study.

Background: Hereditary angioedema (HAE) is a rare autosomal dominant disease; the most well understood forms concern the haplodeficiency of C1 esterase inhibitor (C1INH) and a gain of function mutation of factor XII (FXII). The acute forms of these conditions are mediated by an excessive bradykinin (BK) formation by plasma kallikrein. Methods: A validated LC-MS/MS platform of picomolar sensitivity developed for the analysis of eleven bradykinin-related peptides was applied to the plasma of HAE-C1INH and HAE-FXII sampled during remission. Results: In HAE-C1INH plasma, the concentrations of the relatively stable BK1-5 fragment (mean ± S.E.M.: 12.0 ± 4.2 pmol/L), of BK2-9 (0.7 ± 0.2 pmol/L) and of the sums of BK and its tested fragments (18.0 ± 6.4 pmol/L) are significantly greater than those recorded in the plasma of healthy volunteers (1.9 ± 0.6, 0.03 ± 0.03 and 4.3 ± 0.8 pmol/L, respectively), consistent with the previous evidence of permanent plasma kallikrein activity in this disease. Kinin levels in the plasma of HAE-FXII patients did not differ from controls, suggesting that triggering factors for contact system activation are not active during remission. Conclusion: BK1-5, BK2-9 and the sum of BK and its fragments determined by the sensitive LC-MS/MS technique are proposed as potential biomarkers of HAE-C1INH in remission while this was not applicable to HAE-FXII patients.

Variant-specific effects define the phenotypic spectrum of HNRNPH2-associated neurodevelopmental disorders in males.

Bain type of X-linked syndromic intellectual developmental disorder, caused by pathogenic missense variants in HRNRPH2, was initially described in six female individuals affected by moderate-to-severe neurodevelopmental delay. Although it was initially postulated that the condition would not be compatible with life in males, several affected male individuals harboring pathogenic variants in HNRNPH2 have since been documented. However, functional in-vitro analyses of identified variants have not been performed and, therefore, possible genotype-phenotype correlations remain elusive. Here, we present eight male individuals, including a pair of monozygotic twins, harboring pathogenic or likely pathogenic HNRNPH2 variants. Notably, we present the first individuals harboring nonsense or frameshift variants who, similarly to an individual harboring a de novo p.(Arg29Cys) variant within the first quasi-RNA-recognition motif (qRRM), displayed mild developmental delay, and developed mostly autistic features and/or psychiatric co-morbidities. Additionally, we present two individuals harboring a recurrent de novo p.(Arg114Trp), within the second qRRM, who had a severe neurodevelopmental delay with seizures. Functional characterization of the three most common HNRNPH2 missense variants revealed dysfunctional nucleocytoplasmic shuttling of proteins harboring the p.(Arg206Gln) and p.(Pro209Leu) variants, located within the nuclear localization signal, whereas proteins with p.(Arg114Trp) showed reduced interaction with members of the large assembly of splicing regulators (LASR). Moreover, RNA-sequencing of primary fibroblasts of the individual harboring the p.(Arg114Trp) revealed substantial alterations in the regulation of alternative splicing along with global transcriptome changes. Thus, we further expand the clinical and variant spectrum in HNRNPH2-associated disease in males and provide novel molecular insights suggesting the disorder to be a spliceopathy on the molecular level.

The challenge of genetically unresolved haemophilia A patients: Interest of the combination of whole F8 gene sequencing and functional assays.

BACKGROUND: The causative variant remains unidentified in 2%-5% of haemophilia A (HA) patients despite an exhaustive sequencing of the full F8 coding sequence, splice consensus sequences, 5'/3' untranslated regions and copy number variant (CNV) analysis. Next-generation sequencing (NGS) has provided significant improvements for a complete F8 analysis. AIM: The aim of this study was to identify and characterize pathogenic non-coding variants in F8 of 15 French and Canadian HA patients genetically unresolved, through the use of NGS, mRNA sequencing and functional confirmation of aberrant splicing. METHODS: We sequenced the entire F8 gene using an NGS capture method. We analysed F8 mRNA in order to detect aberrant transcripts. The pathogenic effect of candidate intronic variants was further confirmed using a minigene assay. RESULTS: After bioinformatic analysis, 11 deep intronic variants were identified in 13 patients (8 new variants and 3 previously reported). Three variants were confirmed to be likely pathogenic with the presence of an aberrant transcript during mRNA analysis and minigene assay. We also found a small intronic deletion in 6 patients, recently described as causing mild HA. CONCLUSION: With this comprehensive work combining NGS and functional assays, we report new deep intronic variants that cause HA through splicing alteration mechanism. Functional analyses are critical to confirm the pathogenic effect of these variants and will be invaluable in the future to study the large number of variants of uncertain significance that may affect splicing that will be found in the human genome.

Measurement of Bradykinin Formation and Degradation in Blood Plasma: Relevance for Acquired Angioedema Associated With Angiotensin Converting Enzyme Inhibition and for Hereditary Angioedema Due to Factor XII or Plasminogen Gene Variants.

Bradykinin (BK)-mediated angioedema (AE) states are rare acquired or hereditary conditions involving localized edema of the subcutaneous and submucosal tissues. Citrated plasma from healthy volunteers or patients with hereditary angioedema (HAE) with normal level of C1-inhibitor (C1-INH) was used to investigate pathways of BK formation and breakdown relevant to AE physiopathology. The half-life of BK (100 nM) added to normal plasma was 34 s, a value that was increased ~12-fold when the angiotensin converting enzyme (ACE) inhibitor enalaprilat (130 nM) was added (enzyme immunoassay measurements). The BK half-life was similarly increased ~5-fold following 2 daily oral doses of enalapril maleate in healthy volunteers, finding of possible relevance for the most common form of drug-associated AE. We also addressed the kinetics of immunoreactive BK (iBK) formation and decline, spontaneous or under three standardized stimuli: tissue kallikrein (KLK-1), the particulate material Kontact-APTT™ and tissue plasminogen activator (tPA). Relative to controls, iBK production was rapid (10-20 min) and very intense in response to tPA in plasma of female heterozygotes for variants in gene F12 coding for factor XII (FXII) (p.Thr328Lys, 9 patients; p.Thr328Arg, one). An increased response to Kontact-APTT™ and an early tPA-induced cleavage of anomalous FXII (immunoblots) were also observed. Biotechnological inhibitors showed that the early response to tPA was dependent on plasmin, FXIIa and plasma kallikrein. Results from post-menopausal and pre-menopausal women with HAE-FXII were indistinguishable. The iBK production profiles in seven patients with the plasminogen p.Lys330Glu variant (HAE-PLG) did not significantly differ from those of controls, except for an unexpected, rapid and lanadelumab-resistant potentiation of KLK-1 effect. This enzyme did not cleave plasminogen or factor XII, suggesting a possible idiosyncratic interaction of the plasminogen pathogenic variant with KLK-1 activity. KLK-1 abounds in salivary glands and human saliva, hypothetically correlating with the clinical presentation of HAE-PLG that includes the swelling of the tongue, lips and contiguous throat tissues. Samples from HAE patients with normal C1-INH levels and F12 gene did not produce excessive iBK in response to stimuli. The ex vivo approach provides physiopathological insight into AE states and supports the heterogeneous physiopathology of HAE with normal C1-INH.

Correction of Tetralogy of Fallot Associated With Anomalous Coronary Artery Without Extracardiac Conduit.

Between 1983 and 2016, we operated on 14 children with tetralogy of Fallot with an anomalous coronary artery crossing the pulmonary infundibulum, which is an anomaly that makes the repair complex. The technique used was the enlargement of the right ventricular outflow tract underneath the mobilized coronary artery. All patients had right ventricular outflow tract relief without coronary artery injury. Only one patient required the use of an extracardiac conduit. There was neither in-hospital mortality nor coronary anomaly requiring reintervention. Mobilizing the anomalous coronary artery in tetralogy of Fallot repair often allows relief of obstruction without using an extracardiac conduit.