Patient Perspectives of the Manifestations and Treatment of Anti-MDA5 Antibody-Positive Dermatomyositis: An Observational Survey.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune disorders classically characterized by proximal skeletal muscle inflammation leading to weakness, but they often possess additional systemic manifestations such as cutaneous, pulmonary, and articular disease.1 Although originally dichotomized as either dermatomyositis (DM) or polymyositis, the discovery of new myositis-specific antibodies (MSA) and myositis-associated antibodies has led to the delineation of more refined IIM patient subgroups.

In Vivo Verification of Electron Paramagnetic Resonance Biodosimetry Using Patients Undergoing Radiation Therapy Treatment.

PURPOSE: Electron paramagnetic resonance (EPR) biodosimetry, used to triage large numbers of individuals incidentally exposed to unknown doses of ionizing radiation, is based on detecting a stable physical response in the body that is subject to quantifiable variation after exposure. In vivo measurement is essential to fully characterize the radiation response relevant to a living tooth measured in situ. The purpose of this study was to verify EPR spectroscopy in vivo by estimating the radiation dose received in participants' teeth. METHODS AND MATERIALS: A continuous wave L-band spectrometer was used for EPR measurements. Participants included healthy volunteers and patients undergoing head and neck and total body irradiation treatments. Healthy volunteers completed 1 measurement each, and patients underwent measurement before starting treatment and between subsequent fractions. Optically stimulated luminescent dosimeters and diodes were used to determine the dose delivered to the teeth to validate EPR measurements. RESULTS: Seventy measurements were acquired from 4 total body irradiation and 6 head and neck patients over 15 months. Patient data showed a linear increase of EPR signal with delivered dose across the dose range tested. A linear least-squares weighted fit of the data gave a statistically significant correlation between EPR signal and absorbed dose (P < .0001). The standard error of inverse prediction (SEIP), used to assess the usefulness of fits, was 1.92 Gy for the dose range most relevant for immediate triage (≤7 Gy). Correcting for natural background radiation based on patient age reduced the SEIP to 1.51 Gy. CONCLUSIONS: This study demonstrated the feasibility of using spectroscopic measurements from radiation therapy patients to validate in vivo EPR biodosimetry. The data illustrated a statistically significant correlation between the magnitude of EPR signals and absorbed dose. The SEIP of 1.51 Gy, obtained under clinical conditions, indicates the potential value of this technique in response to large radiation events.

The personal financial burden associated with idiopathic inflammatory myopathies.

The economic burden of idiopathic inflammatory myopathies (IIMs) within the US is underexplored. We hypothesized that IIMs patients experience considerable personal financial burden due to risks of multi-specialist visits, chronic long-term care, costs associated with disability, medical treatment, and overall high spending costs within the US healthcare system. We surveyed members of Myositis Support and Understanding (MSU) (response rate 4.7 %), and of the 470 survey participants that self-reported with diagnoses of IIMs, we assessed financial burden using two validated measures: (1) Financial Worry Score, and (2) Financial Burden Composite Score (FBCS). We determined factors associated with increased FBCS using logistic and Poisson regression respectively. High financial worry was endorsed by 202 participants (43 %) and the average FBCS ± SD was 1.8 ± 1.9. The odds of financial worry among participants with Medicaid is 3.016 times the odds of financial worry among participants without Medicaid (p = 0.011), and the odds of financial worry among participants with private high-deductible insurance is 3.216 times the odds of financial worry among participants who do not have private high-deductible insurance (p =< 0.001). Given the link between personal financial burden and potential effects on patient outcomes, it is essential for physicians to consider patient financial health when determining management or treatment courses. Identifying specific risk factors that can further exacerbate personal financial burden can help physicians identify vulnerable patients to reduce financial hardship.

Tendon Transfers to Improve Grip and Pinch in Patients with Sporadic Inclusion Body Myositis.

Background: Sporadic inclusion body myositis (sIBM) is a rare and slowly progressive skeletal muscle disease that can cause hand dysfunction, which is a major source of disability. Tendon transfers have been reliably used to improve function in other neuromuscular settings. Given that sIBM patients often present with flexion impairments and mostly functioning extensors, we investigated the potential opportunity for tendon transfer surgery to improve hand dysfunction in sIBM patients. Methods: We conducted a scoping review for studies of sIBM and tendon transfers, extracted descriptions of hand function and surgical technique, and recorded results in terms of hand function. We also conducted an institutional review board-approved survey with 470 participants to determine baseline patient-reported function and to determine participant perceptions and expectations for tendon transfer surgery to improve hand function in sIBM. Results: We identified three published case reports on tendon transfers in sIBM patients with subjectively improved grip and pinch strength, but standardized measures of hand function or quality-of-life were not reported. Within the surveyed cohort, half of participants reported that they would consider surgery, yet only 8% had been referred to a hand surgeon. Fifty four percent of participants reported that they would consider surgery if there would be 1-2 years of benefit after surgery. All participants who would consider surgery also had significant upper extremity disability. Discussion: Tendon transfer surgery has the potential to improve quality-of-life for sIBM patients, and there is significant patient interest in this approach. To objectively assess its efficacy, we propose conducting a surgical trial.

Insomnia diagnosis and treatment across the lifespan.

Insomnia impairs quality of life and is associated with an increased risk for physical and mental health problems and substance misuse. Here's how you can help.

Does physical exercise reduce dementia-associated agitation?

Not consistently. Physical exercise demonstrates inconsistent benefit for neuropsychiatric symptoms, including agitation, in patients with dementia (strength of recommendation: B, inconsistent meta-analyses, 2 small randomized controlled trials [RCTs]). The care setting and the modality, frequency, and duration of exercise varied across trials; the impact of these factors is not known.

Factors Associated With In-Hospital Mortality in Mycosis Fungoides Patients: A Multivariable Analysis.

Background Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma (CTCL). Although it often has an indolent course, it can progress to more aggressive CTCL forms. There is sparse data in current literature describing specific clinical factors associated with in-hospital mortality in mycosis fungoides patients. An understanding of patients at greatest risk for in-hospital mortality can aid in developing recommendations for prophylaxis and empirical management. Aim We aim to characterize factors associated with in-hospital mortality in MF patients. Materials and methods The Nationwide Emergency Department Sample (NEDS) was queried for MF cases from 2006 to 2015. Baseline demographic and hospital characteristics were stratified based on survival outcomes. Multivariable logistic regression was used to identify factors associated with in-hospital mortality. Results A total of 57,665 patients with MF presenting to the ED between 2006 and 2015 were identified. Sézary syndrome, sepsis, and advanced age were associated with MF in-hospital mortality, while female sex was inversely associated. There was a downtrend in in-hospital mortality among MF patients presenting to the ED from 2006 to 2015. Conclusions Our study highlights factors crucial for risk-stratification for hospitalized MF patients.

Pain profile and opioid medication use in patients with idiopathic inflammatory myopathies.

OBJECTIVES: Pain is commonly reported in people living with myositis. This study assesses the presence of pain in the subtypes of myositis as well as the frequency of opioid and non-opioid pain medication use. METHODS: A survey was developed and distributed by Myositis Support and Understanding, a patient-led advocacy organization, to members of its group. Multivariate logistic regression analysis and chi-squared tests were performed. RESULTS: A total of 468 participants completed the survey. A total of 423 participants (DM n = 183, PM n = 109 and IBM n = 131) were included, based on reported diagnosis, for final analysis. Some 91.5% of myositis participants reported current or past pain, with 99% attributing their pain to myositis. There was a lower likelihood of pain in participants aged >60 years [odds ratio (OR) 0.2, 95% confidence interval (CI) 0.1, 0.6, P = 0.003]. The percentage of participants reporting pain was statistically different based on myositis type (DM 97.2%, IBM 80.9% and PM 94.5%, P < 0.001), with a higher likelihood of pain in DM compared with IBM (OR 3.7, 95% CI 1.3, 10.2, P = 0.011). There was a lower likelihood of pain in participants aged >60 years (OR 0.2, 95% CI 0.1, 0.6, P = 0.003). Of the 387 participants reporting pain, 335 reported using pain medications (69% prescribed opioids). Male sex, age >60 years and myositis subtype were not associated with likelihood of non-opioid use. CONCLUSION: Pain is a commonly reported symptom in myositis with variable treatment strategies, including opioid medications. This study highlights the importance of addressing pain as part of myositis treatment as well as the need for future studies understanding treatment effectiveness.

Benefit of Deep Inspiratory Breath Hold for Right Breast Cancer When Regional Lymph Nodes Are Irradiated.

BACKGROUND: Although deep inspiratory breath-hold (DIBH) is routinely used for left-sided breast cancers, its benefits for right-sided breast cancer (rBC) have yet to be established. We compared free-breathing (FB) and DIBH treatment plans for a cohort of rBC undergoing regional nodal irradiation (RNI) to determine its potential benefits. METHODS AND MATERIALS: rBC patients considered for RNI (internal mammary nodal chains, supraclavicular field, with or without axilla) from October 2017 to May 2020 were included in this analysis. For each patient, FB versus DIBH plans were generated and dose volume histograms evaluated the following parameters: mean lung dose, ipsilateral lung V20/V5 (volumes of lung receiving 20 Gy and 5 Gy, respectively); mean heart dose and heart V5 (volumes of heart receiving 5 Gy); liver V20 absolute /V30 absolute (absolute volume of liver receiving 20 Gy and 30 Gy, respectively), liver Dmax, and total liver volume irradiated (TVIliver). The dosimetric parameters were compared using Wilcoxon signed-rank testing. RESULTS: Fifty-four patients were eligible for analysis, comparing 108 FB and DIBH plans. DIBH significantly decreased all lung and liver parameters: mean lung dose (19.7 Gy-16.2 Gy, P < .001), lung V20 (40.7%-31.7%, P < .001), lung V5 (61.2%-54.5%, P < .001), TVIliver (1446 cc vs 1264 cc; P = .006) liver Dmax (50.2 Gy vs 48.9 Gy; P = .023), liver V20 (78.8-23.9 cc, P < .001), and liver V30 (58.1-14.6 cc, P < .001) compared with FB. DIBH use did not significantly improve heart parameters, although the V5Heart trended on significance (1.25-0.6, P = .067). CONCLUSIONS: This is the largest cohort to date analyzing DIBH for RNI-rBC. Our findings demonstrate significant improvement in all lung and liver parameters with DIBH, supporting its routine consideration for rBC patients undergoing comprehensive RNI.

Trends and Outcomes of Cardiac Transplantation in the Lowest Urgency Candidates.

Background Because of discrepancies between donor supply and recipient demand, the cardiac transplantation process aims to prioritize the most medically urgent patients. It remains unknown how recipients with the lowest medical urgency compare to others in the allocation process. We aimed to examine differences in clinical characteristics, organ allocation patterns, and outcomes between cardiac transplantation candidates with the lowest and highest medical urgency. Methods and Results We performed a retrospective analysis of the United Network for Organ Sharing database. Patients listed for cardiac transplantation between January 2011 and May 2020 were stratified according to status at time of transplantation. Baseline recipient and donor characteristics, waitlist survival, and posttransplantation outcomes were compared in the years before and after the 2018 allocation system change. Lower urgency patients in the old system were older (58.5 versus 56 years) and more likely female (54.4% versus 23.8%) compared with the highest urgency patients, and these trends persisted in the new system (P<0.001, all). Donors for the lowest urgency patients were more likely older, female, or have a history of cytomegalovirus, hepatitis C, or diabetes (P<0.01, all). The lowest urgency patients had longer waitlist times and under the new allocation system received organs from shorter distances with decreased ischemic times (178 miles versus 269 miles, 3.1 versus 3.5 hours; P<0.001, all). There was no difference in posttransplantation survival (P<0.01, all). Conclusions Patients transplanted as lower urgency receive hearts from donors with additional comorbidities compared with higher urgency patients, but outcomes are similar at 1 year.

A 4-pronged approach to foster healthy aging in older adults.

Monitoring patients' health, mobility, mentation, and ability to maintain social connections can help you promote healthy aging for your older patients.

Evaluation of Case Volumes of a Heart Transplant Program and Short-term Outcomes After Changes in the United Network for Organ Sharing Donor Heart Allocation System.

Importance: Institution-level strategic changes may be associated with heart transplant volume and outcomes. Objective: To describe changes in practice that markedly increased heart transplant volume at a single center, as well as associated patient characteristics and outcomes. Design, Setting, and Participants: A pre-post cohort study was conducted of 107 patients who underwent heart transplant between September 1, 2014, and August 31, 2019, at Yale New Haven Hospital before (September 1, 2014, to August 31, 2018; prechange era) and after (September 1, 2018, to August 31, 2019; postchange era) a strategic change in patient selection by the heart transplant program. Exposure: Strategic change in donor and recipient selection at Yale New Haven Hospital that occurred in August 2018. Main Outcomes and Measures: Outcome measures were transplant case volume, donor and recipient characteristics, and 180-day survival. Results: A total of 49 patients (12.3 per year; 20 women [40.8%]; median age, 57 years [interquartile range {IQR}, 50-63 years]) received heart transplants in the 4 years of the prechange era and 58 patients (58 per year; 19 women [32.8%]; median age, 57 years [IQR, 52-64 years]) received heart transplants in the 1 year of the postchange era. Organ offers were more readily accepted in the postchange era, with an offer acceptance rate of 20.5% (58 of 283) compared with 6.4% (49 of 768) in the prechange era (P < .001). In the postchange era, donor hearts were accepted with a higher median number of prior refusals by other centers than in the prechange era (16.5 [IQR, 6-38] vs 3 [IQR, 1-6]; P < .001). Hearts accepted in the postchange era were from older donors than in the prechange era (median age, 40 years [IQR, 29-48 years] vs 30 years [IQR, 24-42 years]; P < .001). Recipients had a significantly shorter time on the waiting list in the postchange era compared with prechange era (median, 41 days [IQR, 12-289 days] vs 242 days [IQR, 135-428 days]; P < .001). More patients were supported on temporary circulatory assist devices preoperatively in the postchange era than the prechange era (14 [24.1%] vs 0; P < .001). Survival rates at 180 days were not significantly different (43 [87.8%] in the prechange era vs 52 [89.7%] in the postchange era). Mortality while on the waiting list was similar (2.8 deaths per year in the prechange era vs 3 deaths per year in the postchange era). During the comparable time period, 4 other regional centers had volume change ranging from -10% to 68%, while this center's volume increased by 374%. Conclusions and Relevance: This study suggests that strategic changes in donor heart and recipient selection may significantly increase the number of heart transplants while maintaining short-term outcomes comparable with more conservative patient selection. Such an approach may augment the allocation of currently unused donor hearts.

A Blinded, Prospective Study of Error Detection During Physician Chart Rounds in Radiation Oncology.

PURPOSE: Peer review during physician chart rounds is a major quality assurance and patient safety step in radiation oncology. However, the effectiveness of chart rounds in detecting problematic treatment plans is unknown. We performed a prospective blinded study of error detection at chart rounds to clarify the effectiveness of this quality assurance step. METHODS AND MATERIALS: Radiation Oncology Incident Learning System publications were queried for problematic plans approved for treatment that would be detectable at chart rounds. A resident physician, physicist, and dosimetrist collaboratively generated 20 treatment plans with simulated errors identical in nature to those reported to the Radiation Oncology Incident Learning System. These were inserted randomly into weekly chart rounds over 9 weeks, with a median of 2 problematic plans presented per chart rounds (range, 1-4). Data were collected on detection, attendance, length, and number of cases presented at chart rounds. Data were analyzed using descriptive statistics and univariable logistic regression with odds ratios. RESULTS: The median length of chart rounds over the study period was 60 minutes (range, 42-79); median number of cases presented per chart rounds was 45 (range, 38-50). The overall detection rate was 55% (11 of 20). Detection rates were higher for cases presented earlier in chart rounds: 75% versus 25% of problematic plans were detected within 30 minutes of start of chart rounds versus after 30 minutes (odds ratio, 0.11; 95% confidence interval, 0.01-0.88; P = .037). Detection rates showed a trend toward increase during the study period but this was not significant: 33% in weeks 1 to 5 and 73% during weeks 6 to 9 (5.3; 95% confidence interval, 0.78-36; P = .08). CONCLUSIONS: The detection of clinically significant problematic plans during chart rounds could be significantly improved. Problematic plans are more frequently detected earlier in chart rounds and inserting such plans into chart rounds may enhance detection; however, larger studies are needed to confirm these findings. A multi-institutional study is planned.

Outcomes for allogeneic stem cell transplantation in refractory mycosis fungoides and primary cutaneous gamma Delta T cell lymphomas.

We report results on 23 patients with cutaneous T cell lymphoma (7 primary cutaneous γδ T cell lymphoma [PCGDT], 16 mycosis fungoides/Sézary syndrome [MF/SS]) who underwent allogeneic stem cell transplantation. All pts had skin involvement, 14 had total skin electron beam before conditioning. Donors were 10/10 HLA matched related (13), 5/10 haploidentical (4), and matched unrelated (5) or mismatched unrelated (1). Thirteen were in a clinical complete remission at the time of transplant. The 100-day transplant related mortality for the MF/SS and PCGDT patients was 12% and 29%, respectively. At a median follow up of 5.5 years, the overall survival and disease-free survival for MF/SS patients was 75% and 64%, respectively, and 6 of 10 MF/SS pts in long term complete response had a partial response at the time of transplant. Of seven PCGDT patients, four of seven are alive at a median follow up of 5 years and three are disease-free.

Lower-Hybrid Drift Waves Driving Electron Nongyrotropic Heating and Vortical Flows in a Magnetic Reconnection Layer.

We report measurements of lower-hybrid drift waves driving electron heating and vortical flows in an electron-scale reconnection layer under a guide field. Electrons accelerated by the electrostatic potential of the waves exhibit perpendicular and nongyrotropic heating. The vortical flows generate magnetic field perturbations comparable to the guide field magnitude. The measurements reveal a new regime of electron-wave interaction and how this interaction modifies the electron dynamics in the reconnection layer.

Management of primary skin cancer during a pandemic: Multidisciplinary recommendations.

During the coronavirus disease 2019 (COVID-19) pandemic, providers and patients must engage in shared decision making regarding the pros and cons of early versus delayed interventions for localized skin cancer. Patients at highest risk of COVID-19 complications are older; are immunosuppressed; and have diabetes, cancer, or cardiopulmonary disease, with multiple comorbidities associated with worse outcomes. Physicians must weigh the patient's risk of COVID-19 complications in the event of exposure against the risk of worse oncologic outcomes from delaying cancer therapy. Herein, the authors have summarized current data regarding the risk of COVID-19 complications and mortality based on age and comorbidities and have reviewed the literature assessing how treatment delays affect oncologic outcomes. They also have provided multidisciplinary recommendations regarding the timing of local therapy for early-stage skin cancers during this pandemic with input from experts at 11 different institutions. For patients with Merkel cell carcinoma, the authors recommend prioritizing treatment, but a short delay can be considered for patients with favorable T1 disease who are at higher risk of COVID-19 complications. For patients with melanoma, the authors recommend delaying the treatment of patients with T0 to T1 disease for 3 months if there is no macroscopic residual disease at the time of biopsy. Treatment of tumors ≥T2 can be delayed for 3 months if the biopsy margins are negative. For patients with cutaneous squamous cell carcinoma, those with Brigham and Women's Hospital T1 to T2a disease can have their treatment delayed for 2 to 3 months unless there is rapid growth, symptomatic lesions, or the patient is immunocompromised. The treatment of tumors ≥T2b should be prioritized, but a 1-month to 2-month delay is unlikely to worsen disease-specific mortality. For patients with squamous cell carcinoma in situ and basal cell carcinoma, treatment can be deferred for 3 months unless the individual is highly symptomatic.

The Usefulness of Procedure-Based Assessments in Postgraduate Surgical Training Within the Intercollegiate Surgical Curriculum Programme; A Scoping Review.

BACKGROUND: Workplace-based assessments are a mandatory component of postgraduate surgical training within the United Kingdom and Ireland. Procedure-based assessments (PBAs) and direct observation of practical skills (DOPS) are integrated within the Intercollegiate Surgical Curriculum Programme online platform and aim to assess trainees' performance in practical surgical skills; no reviews have previously investigated their educational usefulness in postgraduate surgical training. Usefulness was defined by the 5 criteria detailed by Van der Vleuten for determining the usefulness of educational assessment tools: validity; reliability; acceptability to learners and faculty; impact on future learning and practice; and costs (to the individual trainee, the institution, and society at large). METHODS: Scoping review methodology was used to examine the educational usefulness of PBA and DOPS assessments in postgraduate surgical training. A literature search of Ovid MEDLINE, EMBASE, and Web of Science databases was undertaken. The preferred reporting items for systematic reviews and meta-analyses standards for systematic reviews were followed. RESULTS/DISCUSSION: Ten studies met the inclusion criteria, with 1368 trainees and trainers included. A variety of study methodologies were identified. Although there is some evidence for the validity and reliability of both PBA and DOPS assessments, further work is required in both these domains including on the number of assessments required to ensure satisfactory reliability. This is a research priority, especially if these assessments become a component of summative assessment of trainee competency. The literature indicates that these assessments are generally acceptable to learners and faculty but their acceptability is negatively impacted upon by uncertainty over whether these assessments constitute a formative or summative assessment of trainees. With regards to costs, correct use of PBAs does require allocated time and resources to ensure their correct use and this must be factored into trainer and trainee job plans. CONCLUSIONS: PBA and DOPS assessments are educationally useful tools in postgraduate surgical training. Further research is required to determine the number of assessments required to ensure adequate reliability. To ensure the educational benefits of these assessments are not diminished, clarification from postgraduate training schemes is required regarding whether these assessments an assessment for learning or an assessment of learning.