Gender differences in risk factors and psychosocial functioning in children with psychogenic nonepileptic seizures.

OBJECTIVE: Female predominance is evident in childhood-onset psychogenic nonepileptic seizures (PNES). Understanding gender-specific vulnerability to PNES may provide a unique insight into its cause and management. We aimed to investigate gender differences in demographic characteristics, triggering factors and psychosocial functioning in children and youth with PNES. METHODS: We retrospectively reviewed patients who were evaluated in the PNES clinic at Children's Healthcare of Atlanta from July 2019 to March 2020 and completed questionnaires to assess adverse life events, psychosocial function (Pediatric Symptom Checklist-17 [PSC-17]), and somatic symptoms (Children's Somatic Symptom Inventory-8, [CSSI-8]). RESULTS: Forty-nine consecutive patients (38 girls, 11 boys) with a median age of 15.0 (9-19) years were included in the study. We performed univariate analysis and evaluated significant variables related to PNES according to sex. Majority of both genders experienced daily to weekly PNES, came from dysfunctional families, scored high on PSC-17, were treated for neuropsychiatric illnesses, and experienced bothersome somatic symptoms. The variables significantly different between genders were suicidal thoughts, history of trauma, and learning disability. Suicidal thoughts and trauma, particularly sexual abuse, were significantly more prevalent in girls (p = 0.03) whereas learning disability was more common in boys (p = 0.03). CONCLUSION: Females predominated in our PNES clinic (F:M = 3.5:1). Gender differences in predisposing factors were sexual abuse in females and learning disability in males. Our data highlight the gender-specific risk factors and vulnerability to PNES. The awareness of gender difference may guide more targeted intervention for children and youth with PNES.

Myths and truths about pediatric psychogenic nonepileptic seizures.

Psychogenic nonepileptic seizures (PNES) is a neuropsychiatric condition that causes a transient alteration of consciousness and loss of self-control. PNES, which occur in vulnerable individuals who often have experienced trauma and are precipitated by overwhelming circumstances, are a body's expression of a distressed mind, a cry for help. PNES are misunderstood, mistreated, under-recognized, and underdiagnosed. The mindbody dichotomy, an artificial divide between physical and mental health and brain disorders into neurology and psychiatry, contributes to undue delays in the diagnosis and treatment of PNES. One of the major barriers in the effective diagnosis and treatment of PNES is the dissonance caused by different illness perceptions between patients and providers. While patients are bewildered by their experiences of disabling attacks beyond their control or comprehension, providers consider PNES trivial because they are not epileptic seizures and are caused by psychological stress. The belief that patients with PNES are feigning or controlling their symptoms leads to negative attitudes of healthcare providers, which in turn lead to a failure to provide the support and respect that patients with PNES so desperately need and deserve. A biopsychosocial perspective and better understanding of the neurobiology of PNES may help bridge this great divide between brain and behavior and improve our interaction with patients, thereby improving prognosis. Knowledge of dysregulated stress hormones, autonomic nervous system dysfunction, and altered brain connectivity in PNES will better prepare providers to communicate with patients how intangible emotional stressors could cause tangible involuntary movements and altered awareness.

Neonatal lupus presenting as a non-specific rash in primary care.

Neonatal lupus erythematosus is a rare autoimmune disease caused by passive transplacental acquisition of maternal autoantibodies manifesting in cutaneous, cardiac, haematological and hepatobiliary abnormalities. The hallmark dermatological finding is erythematous annular lesions with a predilection for photo-exposed areas of the skin. We present a case of a female infant born to a mother with Sjogren's syndrome, who initially presented to an ambulatory care setting with non-specific erythematous papules involving the face and scalp. Within 6 days the rash changed in appearance, consisting of widespread erythematous annular and polycyclic lesions with central violaceous clearing and atrophy. Serological tests revealed asymptomatic anemia and leukopenia, elevated liver enzymes, and positive antinuclear antibodies (ANA) and anti-SSb/La antibodies. Further cardiac evaluation was normal. She was managed conservatively in the outpatient setting with topical steroids, avoidance of sunlight and fluorescent light exposure, and primary care, rheumatological and dermatological follow-up.

EWS-FLI1 low Ewing sarcoma cells demonstrate decreased susceptibility to T-cell-mediated tumor cell apoptosis.

Metastatic and relapsed Ewing sarcoma typically afflicts the adolescent population and is largely fatal. These bone tumors are most commonly driven by the fusion oncoprotein EWS-FLI1. Ewing tumors demonstrate significant intra-tumoral heterogeneity, and individual tumor cells can express highly variable and dynamic levels of EWS-FLI1. Recent studies revealed that the EWS-FLI1 oncoprotein level (high versus low expression) greatly influences the behavior of Ewing tumor cells. As compared to cells with high EWS-FLI1, Ewing cells in the EWS-FLI1 low state demonstrate an increased propensity for metastasis. In light of these observations, we sought to determine how tumor cell EWS-FLI1 level influences the anti-tumor cell immune response. Since ICAM-1, which can promote tumor cell/T-cell interaction and T-cell activation, is highly expressed on EWS-FLI1 low cells, we hypothesized that EWS-FLI1 low cells would be more susceptible to T-cell mediated tumor cell apoptosis as compared to cells with high EWS-FLI1. Unexpectedly, we found that EWS-FLI1 low cells are more resistant to T-cell mediated apoptosis than EWS-FLI1 high cells. We investigated the potential mechanisms by which EWS-FLI1 level might influence the T-cell anti-tumor response, and discovered that low EWS-FLI1 expression results in upregulation of PD-L1 and PD-L2, both important ligands for the PD-1 immune checkpoint receptor on T-cells. We demonstrated that blocking PD-1 results in a greater increase of T-cell mediated killing of EWS-FLI1 low tumor cells as compared to cells with higher EWS-FLI1 expression. Our studies suggest that Ewing cells in the EWS-FLI1 low expression state may serve as a niche of tumor immune-evasion.

A demonstration project on the impact of safety culture on infection control practices in hemodialysis.

BACKGROUND: Infections among hemodialysis patients continues to be major causes of morbidity and mortality despite advances in the science of infection prevention. Many infections are potentially preventable, yet research suggests that evidence-based interventions are not uniformly practiced in dialysis settings. The purpose of the project was to reduce the risk of infection among hemodialysis patients in an outpatient dialysis clinic in upstate New York through the development of an enhanced patient safety culture. METHODS: A survey was used to assess the safety culture of a large outpatient dialysis program. A Comprehensive Unit-based Safety Program was instituted to enhance infection prevention practices. Evidence-based checklists and audit tools were used to track staff adherence to protocols. RESULTS: Scores on the survey were strongly correlated with bloodstream infection rates. Adherence to infection control standards improved when the End Stage Renal Disease Safety Program was implemented, with audits improving from 27%-82% of procedures performed correctly. Bloodstream infection rates decreased from 2.33-1.07 events per 100 patient months, and the standardized infection ratios decreased from 1.960-0.985 in the 12-months after implementation. CONCLUSIONS: The Comprehensive Unit-based Safety Program model and implementation of the safety program may be effective in improving the culture of safety and adherence to evidence-based practices in hemodialysis. Enhanced patient safety culture is correlated with improved patient outcomes.

Creation of a competency-based professional development program for infection preventionists guided by the APIC Competency Model: steps in the process.

BACKGROUND: Infection Preventionists have varying levels of educational preparation. Many have no prior experience in IP. The diversity makes design of professional development programs challenging. Recent surveys suggest that only about half of practicing IPs are board certified. There is an urgent need to employ competent IP's to drive improvement in patient outcomes. METHODS: This is a project that utilized the APIC Competency Model to create a professional development program characterizing three career stages. Methods included a review of literature on professional development; a survey of IP competence; an assessment of job descriptions and performance evaluations; and a crosswalk of IP competencies. RESULTS: The professional development program includes competency - based IP job descriptions and performance evaluations for each career stage; a professional portfolio; and a toolkit for supervisors. DISCUSSION: Participants agreed that application of the model resulted in tools which are more closely aligned with current roles for IPs; and increased satisfaction and motivation with the new program. CONCLUSION: Competent and knowledgeable IP's are crucial to optimizing efficacy of IPC programs. A professional development program has the potential to guide staff orientation, improve satisfaction and retention, improve patient outcomes and promote a positive trajectory in advancing practice.