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Neuro-oncology Jul 2024Treatment of childhood medulloblastoma has evolved to reduce neurotoxicity while improving survival. However, the impact of evolving therapies on late neurocognitive...
BACKGROUND
Treatment of childhood medulloblastoma has evolved to reduce neurotoxicity while improving survival. However, the impact of evolving therapies on late neurocognitive outcomes and adult functional independence remains unknown.
METHODS
Adult survivors of childhood medulloblastoma (n=505; median[minimum-maximum] age, 29[18-46] years) and sibling controls (n=727; 32[18-58] years) from the Childhood Cancer Survivor Study completed surveys assessing neurocognitive problems and chronic health conditions (CHCs). Treatment exposures were categorized as historical (craniospinal irradiation [CSI]≥30 Gy, no chemotherapy), standard-risk (CSI>0 to <30 Gy +chemotherapy) and high-risk (CSI≥30 Gy +chemotherapy) therapy. Latent class analysis identified patterns of functional independence using employment, independent living, assistance with routine/personal care needs, driver's license, marital/partner status. Multivariable models estimated risk of neurocognitive impairment in survivors versus siblings and by treatment exposure group, and associations between neurocognitive impairment, CHCs, and functional independence.
RESULTS
Survivors in each treatment exposure group had 4- to 5-fold elevated risk of impaired memory and task efficiency compared to siblings. Contemporary risk-based therapies did not confer lower risk compared to historical therapy. Survivors treated in the 1990s had higher risk of memory impairment (relative risk [RR] 2.24, 95% confidence interval [CI] 1.39-3.60) compared to survivors treated in the 1970s. Sensorimotor, hearing problems and seizures were associated with 33%-34%, 25-26% and 21%-42% elevated risk of task efficiency and memory impairment, respectively. Treatment-related CHCs and neurocognitive impairment were associated with non-independence.
CONCLUSIONS
Despite treatment changes, long-term survivors of childhood medulloblastoma remain at risk for neurocognitive impairment, which was associated with CHCs. Neurocognitive surveillance after contemporary regimens is imperative.
PubMed: 38963825
DOI: 10.1093/neuonc/noae119 -
Endocrinology Jul 2024Vitamin D signals through the vitamin D receptor (VDR) to induce its end-organ effects. Hepatic stellate cells control development of liver fibrosis in response to...
Vitamin D signals through the vitamin D receptor (VDR) to induce its end-organ effects. Hepatic stellate cells control development of liver fibrosis in response to stressors and vitamin D signaling decreases fibrogenesis. VDR expression in hepatocytes, however, is low in healthy liver, and the role of VDR in hepatocyte proliferation is unclear. Hepatocyte-VDR null mice (hVDR) were used to assess the role of VDR and vitamin D signaling in hepatic regeneration. hVDR mice have impaired liver regeneration and impaired hepatocyte proliferation associated with significant differential changes in bile salts. Notably, mice lacking hepatocyte VDR had significant increases in expression of conjugated bile acids after partial hepatectomy, consistent with failure to normalize hepatic function by the 14-day time point tested. Real-time PCR of hVDR and control livers showed significant changes in expression of cell cycle genes including cyclins D1 and E1 and cyclin-dependent kinase 2. Gene expression profiling of hepatocytes treated with vitamin D or control showed regulation of groups of genes involved in liver proliferation, hepatitis, liver hyperplasia/hyperproliferation and liver necrosis/cell death. Together these studies demonstrate an important functional role for VDR in hepatocytes during liver regeneration. Combined with the known profibrotic effects of impaired VDR signaling in stellate cells, the studies provide a mechanism whereby vitamin D deficiency would both reduce hepatocyte proliferation and permit fibrosis, leading to significant liver compromise.
PubMed: 38963813
DOI: 10.1210/endocr/bqae077 -
The American Journal of Case Reports Jul 2024BACKGROUND Morvan fibrillary chorea (Morvan syndrome) is a rare disorder marked by a collection of neurological symptoms such as myokymia, peripheral nerve excitability,...
BACKGROUND Morvan fibrillary chorea (Morvan syndrome) is a rare disorder marked by a collection of neurological symptoms such as myokymia, peripheral nerve excitability, neuromyotonia, autonomic instability, memory impairment, and delirium. Morvan syndrome is suspected to occur through antibodies directed against voltage gated potassium channels (VGKC), and has been linked with several autoimmune conditions and hematologic malignancies. We present a case of Morvan syndrome in association with monoclonal B cell lymphocytosis. Upon our literature review, we believe this to be the first documented case of Morvan syndrome associated with monoclonal B cell lymphocytosis. CASE REPORT The present case report describes a 75-year-old man with Morvan's syndrome. The patient had a diverse neurologic presentation with encephalopathy, progressive neuropathic pain, muscle fasciculations, myokymia, sensory deficits, and Bell's palsy. Ultimately, a paraneoplastic antibody panel revealed a positive titer of contactin-associated protein-like IgG (CASPR) and VGKC antibody. Flow cytometry showed a small population of abnormal lambda-restricted B cells. Given his symptoms, positive CASPR antibody, and flow cytometry findings, he was diagnosed with Morvan syndrome associated with monoclonal B cell lymphocytosis. He was treated with IV methylprednisolone and IVIG, with immediate improvement in neurologic symptoms. CONCLUSIONS Morvan syndrome presents with a spectrum of neurologic symptoms and is associated with autoantibodies against VGKC through anti-CASPR2 antibodies. Classically, Morvan syndrome presents as a paraneoplastic disease secondary to thymomas. Our case demonstrates that there is an association between B cell lymphoproliferative disorders and Morvan syndrome.
Topics: Humans; Male; Aged; Lymphocytosis; B-Lymphocytes; Neuroacanthocytosis
PubMed: 38963810
DOI: 10.12659/AJCR.943867 -
Journal of Speech, Language, and... Jul 2024This study examines the effectiveness of automatic speech recognition (ASR) for individuals with speech disorders, addressing the gap in performance between read and...
PURPOSE
This study examines the effectiveness of automatic speech recognition (ASR) for individuals with speech disorders, addressing the gap in performance between read and conversational ASR. We analyze the factors influencing this disparity and the effect of speech mode-specific training on ASR accuracy.
METHOD
Recordings of read and conversational speech from 27 individuals with various speech disorders were analyzed using both (a) one speaker-independent ASR system trained and optimized for typical speech and (b) multiple ASR models that were personalized to the speech of the participants with disordered speech. Word error rates were calculated for each speech model, read versus conversational, and subject. Linear mixed-effects models were used to assess the impact of speech mode and disorder severity on ASR accuracy. We investigated nine variables, classified as technical, linguistic, or speech impairment factors, for their potential influence on the performance gap.
RESULTS
We found a significant performance gap between read and conversational speech in both personalized and unadapted ASR models. Speech impairment severity notably impacted recognition accuracy in unadapted models for both speech modes and in personalized models for read speech. Linguistic attributes of utterances were the most influential on accuracy, though atypical speech characteristics also played a role. Including conversational speech samples in model training notably improved recognition accuracy.
CONCLUSIONS
We observed a significant performance gap in ASR accuracy between read and conversational speech for individuals with speech disorders. This gap was largely due to the linguistic complexity and unique characteristics of speech disorders in conversational speech. Training personalized ASR models using conversational speech significantly improved recognition accuracy, demonstrating the importance of domain-specific training and highlighting the need for further research into ASR systems capable of handling disordered conversational speech effectively.
PubMed: 38963790
DOI: 10.1044/2024_JSLHR-24-00045 -
Telemedicine Journal and E-health : the... Jul 2024Heart failure (HF) is responsible for a high number of hospitalizations, caused by a progressive worsening quality of life. Telemedicine allows for better management of...
Heart failure (HF) is responsible for a high number of hospitalizations, caused by a progressive worsening quality of life. Telemedicine allows for better management of patients' complex conditions, improving the care released. However, the risk of remaining at a testing stage often limits the integration of remote care in daily pathways for HF patients. The aim of this study is to outline the steps needed to integrate telemedicine activities into ordinary HF clinic practices. This methodology is applied to observe activities and trend improvements over a 12-month routine phase. Three steps have been defined for an efficient introduction of remote care services in ordinary activities, integrating them with traditional in-person care: (i) introduction of temporary telemedicine projects, (ii) systematization of telemedicine pathways, and (iii) evaluation of monitoring phase. Observational data have been collected from structured interviews to show the rate of telemedicine activities achieved in clinical practice over the last year. The methodology has been proposed in the HF clinic of the Italian hospital ASST Bergamo Est. After an initial testing phase, in which usability and user experience have been tested, four different remote activities were added: (i) telemonitoring for patients with an implantable device, (ii) follow-up televisits, (iii) nursing telephone support, and (iv) high-intensity telesurveillance pathways for patients after an HF acute event. During the last year, 218 telemonitoring pathways, 75 televisits, 500 telephone calls, and nine telesurveillance pathways have been performed. Success rates were high, and patients gave positive feedback. By integrating multiple telemedicine activities, it has been possible to better manage complex patients, keep track of disease progression, and improve their participation in care.
PubMed: 38963767
DOI: 10.1089/tmj.2024.0067 -
Telemedicine Journal and E-health : the... Jul 2024The COVID-19 pandemic created critical challenges for hospitals and health care providers. Suddenly clinics were forced to close; elective procedures were delayed;...
The COVID-19 pandemic created critical challenges for hospitals and health care providers. Suddenly clinics were forced to close; elective procedures were delayed; scheduled visits were canceled; emergency rooms were overcrowded; hospital beds, equipment, and personal protective equipment (PPE) were in short supply; and staff were faced with rapidly changing circumstances, care protocols, trauma, and personal risk. To better address challenges of the ongoing COVID-19 pandemic and prepare for future pandemics, the National Telemedicine Technology Assessment Resource Center (TTAC) was asked to develop a Pandemic Response Action Plan that would allow its users to address critical issues with available telemedicine and related technologies. The project was constructed in 3 phases. Phase 1-Develop a Pandemic Response Action Plan and a Pandemic Response Action Plan Policy and Regulatory Summary, which identifies the regulatory challenges as well as policy recommendations. Phase 2-Publish the Action Plan and the Policy and Regulatory Summary. Phase 3-Look at health care providers who used the approaches, tools, and technology in the Pandemic Action Plan and document the results. This document represents Phase 3. This document is Phase 3. In this report we look back at health care providers who used the approaches in the Phase 1 as published in Phase 2. In this document we report on the challenges and results of implementing parts of the Pandemic Action Plan. It records the findings, conclusions, and recommendations resulting from the experience of health care providers and the professional experiences of the team and their organizations in implementing parts or all of the plan. The same multidisciplinary team that constructed Phase 1 and Phase 2 were engaged to develop this Phase 3 report. The members of the team represent leadership expertise and key stakeholders in health care delivery during a pandemic (administration, infection control, physicians, nurses, public health, contingency planning, disaster response, and information technology) as well as a facilitator. For Phase 3, the group used structured brainstorming to define the findings, issues, and results of their own organizations' digital health response to the pandemic. In addition, eight health care providers (hospitals) identified by the Telemedicine Resource Centers' (TRCs) organizations, who used the Pandemic response Plan (created in Phases 1 and 2), were interviewed. All interviews were conducted by the same facilitator with leaders (CEO, and leaders of the telemedicine programs) in each of the eight programs, using a standard questionnaire created by the team. Current literature references are included in this report to illustrate when findings are known to have broader applicability. The impact of the COVID-19 Pandemic was severe and identified multiple critical challenges and weaknesses. Applying the approaches, tools, and technology outlined in the proved to be effective in addressing critical provider challenges. However, implementing these tools during a crisis was difficult unless the organization had experience with the tools and necessary workflows in advance. Implementing these tools as part of standard workflows and everyday operations increased the capabilities and resilience of these organizations in the provision of care during this and for future pandemics.
PubMed: 38963756
DOI: 10.1089/tmj.2023.0248 -
International Journal of Surgery... Jul 2024This review explores the application of induced pluripotent stem cells (iPSCs) in regenerative medicine. The therapeutic significance of iPSC-derived cell therapy within...
This review explores the application of induced pluripotent stem cells (iPSCs) in regenerative medicine. The therapeutic significance of iPSC-derived cell therapy within regenerative medicine, emphasizes their reprogramming process and crucial role in cellular differentiation while setting the purpose and scope for the comprehensive exploration of iPSC-derived cell therapy. The subsequent sections intricately examine iPSC-derived cell therapy, unravelling the diverse derivatives of iPSCs and striking a delicate balance between advantages and limitations in therapeutic applications. Mechanisms of action, revealing how iPSC-derived cells seamlessly integrate into tissues, induce regeneration, and contribute to disease modeling and drug screening advancements is discussed. The analysis extends to clinical trials, shedding light on outcomes, safety considerations, and ethical dimensions. Challenges and concerns, including the risk of tumorigenesis and scalability issues, are explored. The focus extends to disease-specific applications, showcasing iPSC-derived cell therapy as a promising avenue for various medical conditions, supported by illustrative case studies. Future directions and research needs are outlined, identifying areas for further exploration, safety considerations and potential enhancements that will shape the future landscape of iPSC-derived therapies. In conclusion, this review provides significant understanding of iPSC-derived cell therapy's status, that contemplates the implications for regenerative medicine and personalized treatment using iPSCs, offering a comprehensive perspective on the evolving field within the confines of a dynamic and promising scientific frontier.
PubMed: 38963728
DOI: 10.1097/JS9.0000000000001892 -
JMIR MHealth and UHealth Jul 2024Climate change increasingly impacts health, particularly of rural populations in sub-Saharan Africa due to their limited resources for adaptation. Understanding these... (Observational Study)
Observational Study
BACKGROUND
Climate change increasingly impacts health, particularly of rural populations in sub-Saharan Africa due to their limited resources for adaptation. Understanding these impacts remains a challenge, as continuous monitoring of vital signs in such populations is limited. Wearable devices (wearables) present a viable approach to studying these impacts on human health in real time.
OBJECTIVE
The aim of this study was to assess the feasibility and effectiveness of consumer-grade wearables in measuring the health impacts of weather exposure on physiological responses (including activity, heart rate, body shell temperature, and sleep) of rural populations in western Kenya and to identify the health impacts associated with the weather exposures.
METHODS
We conducted an observational case study in western Kenya by utilizing wearables over a 3-week period to continuously monitor various health metrics such as step count, sleep patterns, heart rate, and body shell temperature. Additionally, a local weather station provided detailed data on environmental conditions such as rainfall and heat, with measurements taken every 15 minutes.
RESULTS
Our cohort comprised 83 participants (42 women and 41 men), with an average age of 33 years. We observed a positive correlation between step count and maximum wet bulb globe temperature (estimate 0.06, SE 0.02; P=.008). Although there was a negative correlation between minimum nighttime temperatures and heat index with sleep duration, these were not statistically significant. No significant correlations were found in other applied models. A cautionary heat index level was recorded on 194 (95.1%) of 204 days. Heavy rainfall (>20 mm/day) occurred on 16 (7.8%) out of 204 days. Despite 10 (21%) out of 47 devices failing, data completeness was high for sleep and step count (mean 82.6%, SD 21.3% and mean 86.1%, SD 18.9%, respectively), but low for heart rate (mean 7%, SD 14%), with adult women showing significantly higher data completeness for heart rate than men (2-sided t test: P=.003; Mann-Whitney U test: P=.001). Body shell temperature data achieved 36.2% (SD 24.5%) completeness.
CONCLUSIONS
Our study provides a nuanced understanding of the health impacts of weather exposures in rural Kenya. Our study's application of wearables reveals a significant correlation between physical activity levels and high temperature stress, contrasting with other studies suggesting decreased activity in hotter conditions. This discrepancy invites further investigation into the unique socioenvironmental dynamics at play, particularly in sub-Saharan African contexts. Moreover, the nonsignificant trends observed in sleep disruption due to heat expose the need for localized climate change mitigation strategies, considering the vital role of sleep in health. These findings emphasize the need for context-specific research to inform policy and practice in regions susceptible to the adverse health effects of climate change.
Topics: Humans; Kenya; Wearable Electronic Devices; Female; Male; Adult; Rural Population; Hot Temperature; Middle Aged; Heart Rate; Cohort Studies; Outcome Assessment, Health Care
PubMed: 38963698
DOI: 10.2196/54669 -
JMIR Research Protocols Jul 2024Evidence suggests that having a chronic physical illness (CPI; eg, asthma, diabetes, and epilepsy) is an independent risk factor for suicidality (ie, suicidal ideation...
BACKGROUND
Evidence suggests that having a chronic physical illness (CPI; eg, asthma, diabetes, and epilepsy) is an independent risk factor for suicidality (ie, suicidal ideation or attempts) among youth. Less is known about the mechanisms linking CPI and suicidality. Some evidence suggests that mental illness (eg, depression and anxiety) or neurodevelopmental disorder (eg, attention-deficit/hyperactivity disorder) mediates or moderates the CPI-suicidality association. Missing from the knowledge base is information on the association between having co-occurring CPI and mental illness or neurodevelopmental disorder (MIND) on youth suicidality.
OBJECTIVE
This study uses epidemiological data from the 2019 Canadian Health Survey of Children and Youth (CHSCY) to study the intersection of CPI, MIND, and suicidality in youth. We will estimate prevalence, identify predictors, and investigate psychosocial and service use outcomes for youth with CPI-MIND comorbidity versus other morbidity groups (ie, healthy, CPI only, and MIND only).
METHODS
Conducted by Statistics Canada, the CHSCY collected data from 47,850 children (aged 1-17 years) and their primary caregiving parent. Measures of youth CPI, MIND, family environment, and sociodemographics are available using youth and parent informants. Information on psychiatric services use is available via parent report and linkage to national administrative health data found in the National Ambulatory Care Reporting System and the Discharge Abstract Database, which allow the investigation of hospital-based mental health services (eg, emergency department visits, hospitalizations, and length of stay in hospital). Questions about suicidality were restricted to youths aged 15-17 years (n=6950), which form our analytic sample. Weighted regression-based analyses will account for the complex survey design.
RESULTS
Our study began in November 2023, funded by the American Foundation for Suicide Prevention (SRG-0-008-22). Access to the linked CHSCY microdata file was granted in May 2024. Initial examination of CHSCY data shows that approximately 20% (1390/6950) of youth have CPI, 7% (490/6950) have MIND, 7% (490/6950) seriously considered suicide in the past year, and 3% (210/6950) had attempted suicide anytime during their life.
CONCLUSIONS
Findings will provide estimates of suicidality among youth with CPI-MIND comorbidity, which will inform intervention planning to prevent loss of life in this vulnerable population. Modeling correlates of suicidality will advance understanding of the relative and joint effects of factors at multiple levels-information needed to target prevention efforts and services. Understanding patterns of psychiatric service use is vital to understanding access and barriers to services. This will inform whether use matches need, identifying opportunities to advise policy makers about upstream resources to prevent suicidality. Importantly, findings will provide robust baseline of information on the link between CPI-MIND comorbidity and suicidality in youth, which can be used by future studies to address questions related to the impact of the COVID-19 pandemic and associated countermeasures in this vulnerable population of youth.
INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID)
DERR1-10.2196/57103.
Topics: Humans; Adolescent; Child; Canada; Suicidal Ideation; Suicide, Attempted; Female; Male; Comorbidity; Child, Preschool; Mental Disorders; Infant; Chronic Disease; Prevalence; Risk Factors; Health Surveys
PubMed: 38963692
DOI: 10.2196/57103 -
JMIR Aging Jul 2024Persons living with dementia experience autonomy loss and require caregiver support on a daily basis. Dementia involves a gradual decline in communication skills,...
Efficacy of COMPAs, an App Designed to Support Communication Between Persons Living With Dementia in Long-Term Care Settings and Their Caregivers: Mixed Methods Implementation Study.
BACKGROUND
Persons living with dementia experience autonomy loss and require caregiver support on a daily basis. Dementia involves a gradual decline in communication skills, leading to fewer interactions and isolation for both people living with dementia and their caregivers, negatively impacting the quality of life for both members of the dyad. The resulting stress and burden on caregivers make them particularly susceptible to burnout.
OBJECTIVE
This study aims to examine the efficacy of Communication Proches Aidants (COMPAs), an app designed following the principles of person-centered and emotional communication, which is intended to improve well-being in persons living with dementia and caregivers and reduce caregiver burden.
METHODS
In this implementation study, volunteer caregivers in 2 long-term care facilities (n=17) were trained in using COMPAs and strategies to improve communication with persons living with dementia. Qualitative and quantitative analyses, semistructured interviews, and questionnaires were completed before and after 8 weeks of intervention with COMPAs.
RESULTS
Semistructured interviews revealed that all caregivers perceived a positive impact following COMPAs interventions, namely, improved quality of communication and quality of life among persons living with dementia and caregivers. Improved quality of life was also supported by a statistically significant reduction in the General Health Questionnaire-12 scores (caregivers who improved: 9/17, 53%; z=2.537; P=.01). COMPAs interventions were also associated with a statistically significant increased feeling of personal accomplishment (caregivers improved: 11/17, 65%; t=2.430; P=.03; d=0.61 [medium effect size]).
CONCLUSIONS
COMPAs intervention improved well-being in persons living with dementia and their caregivers by developing person-centered communication within the dyad, increasing empathy, and reducing burden in caregivers although most caregivers were unfamiliar with technology. The results hold promise for COMPAs interventions in long-term care settings. Larger group-controlled studies with different populations, in different contexts, and at different stages of dementia will provide a clearer picture of the benefits of COMPAs interventions.
Topics: Humans; Dementia; Caregivers; Long-Term Care; Female; Male; Quality of Life; Mobile Applications; Aged; Communication; Middle Aged; Surveys and Questionnaires; Aged, 80 and over
PubMed: 38963691
DOI: 10.2196/47565