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Annals of Medicine and Surgery (2012) Feb 2024Hyper-IgE syndrome (HIES), also known as Job syndrome, is a rare primary immunodeficiency disorder characterized by elevated serum IgE levels, recurrent infections, and...
INTRODUCTION AND IMPORTANCE
Hyper-IgE syndrome (HIES), also known as Job syndrome, is a rare primary immunodeficiency disorder characterized by elevated serum IgE levels, recurrent infections, and various clinical features. Early diagnosis, prompt management of infections, and supportive care are essential in improving outcomes for individuals with HIES. Genetic testing, including STAT3 gene sequencing, plays a crucial role in confirming the diagnosis. Further research is needed to enhance our understanding of HIES and develop targeted therapies to improve the quality of life for affected individuals.
CASE PRESENTATION
This case report presents the clinical features and management of a 37-year-old male with HIES, diagnosed at the age of 2 due to recurrent cold abscesses caused by Staphylococcal infections.
CLINICAL DISCUSSION
The patient exhibited typical symptoms of HIES, including recurrent eczema, frequent bacterial infections, mucocutaneous candidiasis, and various physical abnormalities. Diagnostic markers such as elevated IgE levels and eosinophilia supported the HIES diagnosis, which was further confirmed by the identification of a STAT3 gene mutation. Treatment primarily involved supportive measures and antibiotics for infections. The patient's blood test results and imaging findings revealed abnormalities such as low red blood cell count, elevated erythrocyte sedimentation rate, and pulmonary nodules.
CONCLUSION
This case report highlights the importance of early diagnosis, prompt management of infections, and the need for ongoing research to improve our understanding and treatment of HIES.
PubMed: 38333292
DOI: 10.1097/MS9.0000000000001670 -
Behavioral Sciences (Basel, Switzerland) Jul 2023Burnout syndrome is caused by a number of factors, including personal, organizational, and professional problems. The purpose of this study was to investigate burnout...
Burnout syndrome is caused by a number of factors, including personal, organizational, and professional problems. The purpose of this study was to investigate burnout and satisfaction levels among emergency department staff in the context of professional activity issues. We studied a sample of 184 participants working in the emergency department. Participants signed an informed consent form, completed a socio-demographic questionnaire and the MBI-HSS (MP) questionnaire to assess burnout, a JSS (Job Satisfaction Survey) to assess their professional satisfaction, and the AWS (AWS of work survey) questionnaire on work aspects. The questionnaires were completed between November 2022 and March 2023. The study group was aged between 24-64 years old, most of the subjects being female. The study found that 30.2% of emergency medical staff are at high risk of burnout. Emotional exhaustion is indirectly proportional to workload, interpersonal relationships, and rewards. Emotional exhaustion is a direct result of work experience. While participants expressed ambivalence and dissatisfaction with the work environment, they were satisfied with the nature of their work. This study found that job-related factors such as social support and feedback are significant predictors of employee well-being and reducing the risk of burnout. Emotional exhaustion was negatively correlated with job satisfaction, while personal accomplishment was positively correlated with job satisfaction. Depersonalization was associated with job dissatisfaction with operating conditions and coworkers. The study also identified differences in burnout and related constructs among healthcare professionals, with resident physicians reporting higher levels of personal accomplishments and paramedics reporting relatively low levels of emotional exhaustion. The findings suggest that tailored interventions addressing job demands and resources are critical in improving employee well-being and reducing burnout.
PubMed: 37504022
DOI: 10.3390/bs13070575 -
Journal of Clinical Immunology Dec 2023Genetic variants in IL6ST encoding the shared cytokine receptor for the IL-6 cytokine family GP130 have been associated with a diverse number of clinical phenotypes and... (Review)
Review
Genetic variants in IL6ST encoding the shared cytokine receptor for the IL-6 cytokine family GP130 have been associated with a diverse number of clinical phenotypes and disorders. We provide a molecular classification for 59 reported rare IL6ST pathogenic or likely pathogenic variants and additional polymorphisms. Based on loss- or gain-of-function, cytokine selectivity, mono- and biallelic associations, and variable cellular mosaicism, we grade six classes of IL6ST variants and explore the potential for additional variants. We classify variants according to the American College of Medical Genetics and Genomics criteria. Loss-of-function variants with (i) biallelic complete loss of GP130 function that presents with extended Stüve-Wiedemann Syndrome; (ii) autosomal recessive hyper-IgE syndrome (HIES) caused by biallelic; and (iii) autosomal dominant HIES caused by monoallelic IL6ST variants both causing selective IL-6 and IL-11 cytokine loss-of-function defects; (iv) a biallelic cytokine-specific variant that exclusively impairs IL-11 signaling, associated with craniosynostosis and tooth abnormalities; (v) somatic monoallelic mosaic constitutively active gain-of-function variants in hepatocytes that present with inflammatory hepatocellular adenoma; and (vi) mosaic constitutively active gain-of-function variants in hematopoietic and non-hematopoietic cells that are associated with an immune dysregulation syndrome. In addition to Mendelian IL6ST coding variants, there are common non-coding cis-acting variants that modify gene expression, which are associated with an increased risk of complex immune-mediated disorders and trans-acting variants that affect GP130 protein function. Our taxonomy highlights IL6ST as a gene with particularly strong functional and phenotypic diversity due to the combinatorial biology of the IL-6 cytokine family and predicts additional genotype-phenotype associations.
Topics: Humans; Cytokine Receptor gp130; Cytokines; Interleukin-11; Interleukin-6; Job Syndrome; Receptors, Cytokine; STAT3 Transcription Factor
PubMed: 38133879
DOI: 10.1007/s10875-023-01603-7 -
Occupational Medicine (Oxford, England) Feb 2024The apparent functional impact of post-COVID-19 syndrome has workability implications for large segments of the working-age population.
BACKGROUND
The apparent functional impact of post-COVID-19 syndrome has workability implications for large segments of the working-age population.
AIMS
To understand obstacles and enablers around self-reported workability of workers following COVID-19, to better guide sustainable workplace accommodations.
METHODS
An exploratory online survey comprising quantitative and qualitative questions was disseminated via social media and industry networks between December 2020 and February 2021, yielding usable responses from 145 workers. Qualitative data were subjected to content analysis.
RESULTS
Over half of the sample (64%) were from the health, social care, and education sectors. Just under 15% had returned to work, and 53% and 50% reported their physical and psychological workability respectively as moderate at best. Leading workability obstacles were multi-level, comprising fatigue, the interaction between symptoms and job, lack of control over job pressures, inappropriate sickness absence management policies, and lack of COVID-aware organizational cultures. Self-management support, modified work, flexible co-developed graded return-to-work planning, and improved line management competency were advocated as key enablers.
CONCLUSIONS
Assuming appropriate medical management of any pathophysiological complications of COVID-19, maintaining or regaining post-COVID workability might reasonably follow a typical biopsychosocial framework enhanced to cater to the fluctuating nature of the symptoms. This should entail flexible, regularly reviewed and longer-term return-to-work planning addressing multi-level workability obstacles, co-developed between workers and line managers, with support from human resources, occupational health professionals (OHP's), and a COVID-aware organizational culture.
Topics: Humans; Post-Acute COVID-19 Syndrome; COVID-19; Workplace; Occupational Health; Social Support
PubMed: 35968657
DOI: 10.1093/occmed/kqac086 -
Anesthesiology Jan 2024Anesthesiologists are experiencing unprecedented levels of workplace stress and staffing shortages. This analysis aims to assess how U.S. attending anesthesiologist...
BACKGROUND
Anesthesiologists are experiencing unprecedented levels of workplace stress and staffing shortages. This analysis aims to assess how U.S. attending anesthesiologist burnout changed since the onset of the COVID-19 pandemic and target well-being efforts.
METHODS
The authors surveyed the American Society of Anesthesiologists' U.S. attending anesthesiologist members in November 2022. Burnout was assessed using the Maslach Burnout Inventory Human Services Survey with additional questions relating to workplace and demographic factors. Burnout was categorized as high risk for burnout (exhibiting emotional exhaustion and/or depersonalization) or burnout syndrome (demonstrating all three burnout dimensions concurrently). The association of burnout with U.S. attending anesthesiologist retention plans was analyzed, and associated factors were identified.
RESULTS
Of 24,680 individuals contacted, 2,698 (10.9%) completed the survey, with 67.7% (1,827 of 2,698) at high risk for burnout and 18.9% (510 of 2,698) with burnout syndrome. Most (78.4%, n = 2,115) respondents have experienced recent staffing shortages, and many (36.0%, n = 970) were likely to leave their job within the next 2 yr. Those likely to leave their job in the next 2 yr had higher prevalence of high risk for burnout (78.5% [760 of 970] vs. 55.7% [651 of 1,169], P < 0.001) and burnout syndrome (24.3% [236 of 970] vs. 13.3% [156 of 1,169], P < 0.001) compared to those unlikely to leave. On multivariable analysis, perceived lack of support at work (odds ratio, 9.2; 95% CI, 7.0 to 12.1), and staffing shortages (odds ratio, 1.96; 95% CI, 1.57 to 2.43) were most strongly associated with high risk for burnout. Perceived lack of support at work (odds ratio, 6.3; 95% CI, 3.81 to 10.4) was the factor most strongly associated with burnout syndrome.
CONCLUSIONS
Burnout is more prevalent in anesthesiology since early 2020, with workplace factors of perceived support and staffing being the predominant associated variables. Interventions focused on the drivers of burnout are needed to improve well-being among U.S. attending anesthesiologists.
Topics: Humans; Anesthesiologists; Pandemics; Job Satisfaction; Burnout, Professional; Surveys and Questionnaires
PubMed: 37930155
DOI: 10.1097/ALN.0000000000004784 -
Pharmaceutical Research Oct 2023Impostor phenomenon (IP), grit, and other factors impact job satisfaction for faculty, particularly female faculty.
INTRODUCTION
Impostor phenomenon (IP), grit, and other factors impact job satisfaction for faculty, particularly female faculty.
METHODS
The Impostor Phenomenon Research Collaborative (IPRC) evaluated IP, grit, and job satisfaction in pharmacy faculty. A cross-sectional study was conducted with a convenience sample of faculty using a survey, which included demographic questions and validated instruments: Clance Impostor Phenomenon (CIPS), Short GRIT Scale, and Overall Job Satisfaction Questionnaire. Differences between groups, relationships, and prediction were evaluated using independent t-tests, ANOVA, Pearson correlation, and regression analyses.
RESULTS
A total of 436 participants completed the survey; 380 self-identified as pharmacy faculty. Two hundred and one (54%) reported intense or frequent feelings of IP. The mean CIPS score was above 60, indicating a risk of negative outcomes related to IP. There were no differences in the prevalence of IP or job satisfaction levels when female and male faculty were compared. Female faculty had higher GRIT-S scores. Faculty reporting more IP had lower grit and lower job satisfaction. Job satisfaction in faculty was predicted by IP and grit; however, grit did not provide a unique prediction when combined with IP for male faculty.
CONCLUSION
IP was not more prevalent in female faculty. Female faculty were grittier than male faculty. Higher grit was associated with less IP and higher job satisfaction. IP and grit predicted job satisfaction for female and male pharmacy faculty. Our findings suggest that improving grit may help mitigate IP and impact job satisfaction. Further research on evidence-based IP interventions is needed.
Topics: Humans; Male; Female; Faculty, Pharmacy; Job Satisfaction; Cross-Sectional Studies; Anxiety Disorders
PubMed: 37101082
DOI: 10.1007/s11095-023-03518-9 -
Journal of Medical Internet Research Aug 2023Shift workers are at high risk of developing sleep disorders such as shift worker sleep disorder or chronic insomnia. Cognitive behavioral therapy (CBT) is the... (Clinical Trial)
Clinical Trial
Effect of an Internet-Delivered Cognitive Behavioral Therapy-Based Sleep Improvement App for Shift Workers at High Risk of Sleep Disorder: Single-Arm, Nonrandomized Trial.
BACKGROUND
Shift workers are at high risk of developing sleep disorders such as shift worker sleep disorder or chronic insomnia. Cognitive behavioral therapy (CBT) is the first-line treatment for insomnia, and emerging evidence shows that internet-based CBT is highly effective with additional features such as continuous tracking and personalization. However, there are limited studies on internet-based CBT for shift workers with sleep disorders.
OBJECTIVE
This study aimed to evaluate the impact of a 4-week, physician-assisted, internet-delivered CBT program incorporating machine learning-based well-being prediction on the sleep duration of shift workers at high risk of sleep disorders. We evaluated these outcomes using an internet-delivered CBT app and fitness trackers in the intensive care unit.
METHODS
A convenience sample of 61 shift workers (mean age 32.9, SD 8.3 years) from the intensive care unit or emergency department participated in the study. Eligible participants were on a 3-shift schedule and had a Pittsburgh Sleep Quality Index score ≥5. The study comprised a 1-week baseline period, followed by a 4-week intervention period. Before the study, the participants completed questionnaires regarding the subjective evaluation of sleep, burnout syndrome, and mental health. Participants were asked to wear a commercial fitness tracker to track their daily activities, heart rate, and sleep for 5 weeks. The internet-delivered CBT program included well-being prediction, activity and sleep chart, and sleep advice. A job-based multitask and multilabel convolutional neural network-based model was used for well-being prediction. Participant-specific sleep advice was provided by sleep physicians based on daily surveys and fitness tracker data. The primary end point of this study was sleep duration. For continuous measurements (sleep duration, steps, etc), the mean baseline and week-4 intervention data were compared. The 2-tailed paired t test or Wilcoxon signed rank test was performed depending on the distribution of the data.
RESULTS
In the fourth week of intervention, the mean daily sleep duration for 7 days (6.06, SD 1.30 hours) showed a statistically significant increase compared with the baseline (5.54, SD 1.36 hours; P=.02). Subjective sleep quality, as measured by the Pittsburgh Sleep Quality Index, also showed statistically significant improvement from baseline (9.10) to after the intervention (7.84; P=.001). However, no significant improvement was found in the subjective well-being scores (all P>.05). Feature importance analysis for all 45 variables in the prediction model showed that sleep duration had the highest importance.
CONCLUSIONS
The physician-assisted internet-delivered CBT program targeting shift workers with a high risk of sleep disorders showed a statistically significant increase in sleep duration as measured by wearable sensors along with subjective sleep quality. This study shows that sleep improvement programs using an app and wearable sensors are feasible and may play an important role in preventing shift work-related sleep disorders.
INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID)
RR2-10.2196/24799.
Topics: Humans; Adult; Mobile Applications; Sleep; Sleep Duration; Sleep Initiation and Maintenance Disorders; Cognitive Behavioral Therapy; Internet
PubMed: 37606971
DOI: 10.2196/45834 -
Critical Reviews in Clinical Laboratory... Jun 2024No standard tool to measure pathologist workload currently exists. An accurate measure of workload is needed for determining the number of pathologists to be hired,... (Review)
Review
No standard tool to measure pathologist workload currently exists. An accurate measure of workload is needed for determining the number of pathologists to be hired, distributing the workload fairly among pathologists, and assessing the overall cost of pathology consults. Initially, simple tools such as counting cases or slides were used to give an estimate of the workload. More recently, multiple workload models, including relative value units (RVUs), the Royal College of Pathologists (RCP) point system, Level 4 Equivalent (L4E), Work2Quality (W2Q), and the University of Washington, Seattle (UW) slide count method, have been developed. There is no "ideal" model that is universally accepted. The main differences among the models come from the weights assigned to different specimen types, differential calculations for organs, and the capture of additional tasks needed for safe and timely patient care. Academic centers tend to see more complex cases that require extensive sampling and additional testing, while community-based and private laboratories deal more with biopsies. Additionally, some systems do not account for teaching, participation in multidisciplinary rounds, quality assurance activities, and medical oversight. A successful workload model needs to be continually updated to reflect the current state of practice.Awareness about physician burnout has gained attention in recent years and has been added to the World Health Organization's International Classification of Diseases (World Health Organization, WHO) as an occupational phenomenon. However, the extent to which this affects pathologists is not well understood. According to the WHO, burnout syndrome is diagnosed by the presence of three components: emotional exhaustion, depersonalization from one's work (cynicism related to one's job), and a low sense of personal achievement or accomplishment. Three drivers of burnout are the demand for productivity, lack of recognition, and electronic health records. Prominent consequences of physician burnout are economic and personal costs to the public and to the providers.Wellness is physical and mental well-being that allows individuals to manage stress effectively and to thrive in both their professional and personal lives. To achieve wellness, it is necessary to understand the root causes of burnout, including over-work and working under stressful conditions. Wellness is more than the absence of stress or burnout, and the responsibility of wellness should be shared by pathologists themselves, their healthcare organization, and governing bodies. Each pathologist needs to take their own path to achieve wellness.
Topics: Humans; Workload; Burnout, Professional; Pathologists
PubMed: 38809116
DOI: 10.1080/10408363.2023.2285284 -
Journal of Women's Health (2002) Apr 2024Women now make up more than half of the physician workforce, but they are disproportionately plagued by burnout. Medicine is a fast-paced stressful field, the practice...
Women now make up more than half of the physician workforce, but they are disproportionately plagued by burnout. Medicine is a fast-paced stressful field, the practice of which is associated with significant chronic stress due to systems issues, crowding, electronic medical records, and patient case mix. Hospitals and health care systems are responsible for mitigating system-based burnout-prone conditions, but often their best efforts fail. Physicians, particularly women, must confront their stressors and the daily burden of significant system strain when this occurs. Those who routinely exceed their cumulative stress threshold may experience burnout, career dissatisfaction, and second victim syndrome and, ultimately, may prematurely leave medicine. These conditions affect women in medicine more often than men and may also produce a higher incidence of health issues, including depression, substance use disorder, and suicide. The individual self-care required to maintain health and raise stress thresholds is not widely ingrained in provider practice patterns or behavior. However, the successful long-term practice of high-stress occupations, such as medicine, requires that physicians, especially women physicians, attend to their wellness. In this article, we address one aspect of health, resilience, and review six practices that can create additional stores of personal resilience when proactively integrated into a daily routine.
Topics: Humans; Female; Burnout, Professional; Physicians, Women; Resilience, Psychological; Job Satisfaction; Stress, Psychological
PubMed: 37843899
DOI: 10.1089/jwh.2022.0502 -
BMC Surgery Sep 2023STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad...
BACKGROUND
STAT3 hyperimmunoglobulin E syndrome (STAT3-HIES) also referred to as autosomal dominant HIES (AD-HIES) is an inborn error of immunity characterized by the classic triad of eczema, frequent opportunistic infections, and elevated serum IgE levels. As a consequence of lung sequels due to repeated infections and impaired tissue healing, patients may require interventional pulmonary procedures.
METHOD
Four patients with dominant-negative STAT3 mutations who had received interventional pulmonary procedures were enrolled. The demographic, clinical, and molecular characteristics were gathered through a medical record search. All reported STAT3-HIES patients in the literature requiring pulmonary procedures as part of their treatment were reviewed.
RESULT
Recurrent episodes of pneumonia and lung abscess were the most prevalent symptoms. The most common non-immunological features were scoliosis, failure to thrive, and dental problems such as primary teeth retention and disseminated decays. Bronchiectasis, lung abscess, pneumatocele, and cavitary lesion were the most prevalent finding on high-resolution computed tomography at the earliest recording. All patients underwent pulmonary surgery and two of them experienced complications.
CONCLUSION
Patients with STAT3-HIES have marked pulmonary infection susceptibility which may necessitate thoracic surgeries. Since surgical procedures involve a high risk of complication, surgical options are recommended to be utilized only in cases of drug resistance or emergencies.
Topics: Humans; Lung Abscess; Job Syndrome; Patients; Scoliosis; Lung; STAT3 Transcription Factor
PubMed: 37741967
DOI: 10.1186/s12893-023-02193-2