Citations

Abstract Context The impact of active hypercortisolism on sleep is incompletely characterized. Studies report impaired sleep in patients with Cushing syndrome (CS). Patients with mild autonomous cortisol secretion (MACS) demonstrate mild nocturnal hypercortisolism that could impact sleep. Objectives To characterize sleep abnormalities in patients with CS and MACS using the Pittsburgh Sleep Quality Index (PSQI), identify factors associated with poor sleep, and compare sleep abnormalities in patients with MACS vs referent subjects. Methods We conducted a single-center cross-sectional study of adults with active CS and MACS. Clinical and biochemical severity scores for hypercortisolism were calculated. Parallelly, we enrolled referent subjects. Quality of life was assessed using (1) the Short Form-36 (SF-36) in all participants and (2) the CushingQoL in patients with active hypercortisolism. Sleep quality was assessed using PSQI. Results PSQI was assessed in 154 patients with CS (mean 12, SD ±4.5), 194 patients with MACS (mean 11, SD 4.6), and 89 referents (mean 5, SD ±3.4). Patients with MACS exhibited shorter sleep duration, longer sleep latency, more severe daytime dysfunction, lower sleep efficiency, and a higher sleep medication use compared to referent subjects (P = <.001 for all). Age-, sex, and body mass index-adjusted analysis demonstrated no differences in PSQI or its subcomponents between patients with CS and MACS (P > .05 for all). In a multivariable analysis of patients with MACS, younger age, female sex, and higher clinical hypercortisolism severity score were associated with impaired sleep. In patients with CS, only younger age was associated with poor sleep. Conclusion Patients with MACS demonstrate sleep impairment that is similar to patients with CS. Younger women with higher clinical severity of MACS are more likely to have impaired sleep.

ABSTRACT Background The Geriatric Emergency Department (ED) guidelines recommend screening older adults for delirium using evidence‐based screening tools, but these tools are often underutilized. We aimed to identify barriers and facilitators to implementing an electronic health record (EHR)‐based delirium screening tool as intended. Methods This mixed methods and focused ethnographic study combined a review of structured delirium screening data with observations of real‐time delirium screenings and semi‐structured in‐person interviews at an academic ED that previously implemented the Delirium Triage Screen (DTS) and Brief Confusion Assessment Method (bCAM) tool. Content analysis of field notes from observations and interview transcripts was guided by the Exploration, Preparation, Implementation, and Sustainment implementation framework. Results From a total of 40,818 ED visits, 6196 (15.2%) had incomplete screens (i.e., without fidelity). We observed 62 encounters, of which 25 (40.3%) screenings were implemented without fidelity due to (1) not asking the required screening questions, (2) assuming patient responses, or (3) not completing the screen due to uncertainty regarding a patient's baseline mentation. We conducted 32 interviews. Identified barriers included staff prioritization of other tasks (e.g., stroke code, cleaning patients), limited knowledge of the screen's importance and utility, and language discordance. Facilitators included care partners providing baseline mental status information and nurses prefacing the screening to increase patient engagement. Conclusion The fidelity of ED delirium screening is influenced by an interplay of environmental, patient, provider, and caregiver factors. Fidelity may be improved by focusing education on the importance of screening, training screeners on how to determine whether a patient's mentation is acutely changed, providing clear action steps when a patient screens positive, and addressing how to administer the delirium screen to patients who do not speak English fluently.

ABSTRACT Light chain multiple myeloma (LCMM) is a subtype of multiple myeloma (MM) characterized by the exclusive production of immunoglobulin light chains and accounts for 15%–20% of newly diagnosed MM. A comprehensive comparison of LCMM with MM producing intact immunoglobulin (non‐LCMM) remains limited. In this retrospective study, we described distinct clinical and cytogenetic features and assessed long‐term outcomes in 852 LCMM patients diagnosed between 01/01/2004 and 12/31/2022, compared with non‐LCMM controls matched for age, sex, and year of diagnosis. On univariable analysis, LCMM patients were more likely to present with elevated creatinine (> 2 mg/dL), beta‐2‐microglobulin ≥ 5.5 mg/L, elevated LDH, hypercalcemia, t(11;14), del(13q), and t(6;14). Conversely, IMS‐IMWG high‐risk disease, hyperdiploidy, t(4;14), and serum albumin < 3.5 g/dL were less common. On multivariable analysis, elevated creatinine (OR: 5.1, 95% CI: 1.2–27, p = 0.04), t(11;14) (OR: 2.6, 95% CI: 1.3–5.2, p = 0.006), and del(13q) (OR: 4.1, 95% CI: 2.1–8.2, p < 0.001) were independently associated with LCMM, while IMS‐IMWG high‐risk disease (OR: 0.3, 95% CI: 0.1–0.8, p = 0.02) and hyperdiploidy (OR: 0.3, 95% CI: 0.1–0.7, p = 0.002) were less frequent. Despite these differences, progression‐free survival with frontline treatment (HR: 0.97, 95% CI: 0.84–1.11, p = 0.63) and overall survival (HR: 0.99, 95% CI: 0.87–1.13, p = 0.94) were similar between LCMM and non‐LCMM patients. This study highlights the distinct clinical and cytogenetic features of LCMM, marked by higher rates of renal failure, t(11;14), and del(13q), lower prevalence of IMS‐IMWG high‐risk disease, and comparable survival outcomes.

Abstract Objective This study aimed to understand the factors limiting access to medications for the preventive treatment of migraine and to improve access to evidence‐based preventive care. Background For decades, the effective use of medication for the preventive treatment of migraine was limited by slow onset, slow and complex dose titration schedules, modest benefits, drug interactions, frequent side effects, and very low long‐term adherence. The calcitonin gene‐related peptide (CGRP) targeted preventive medications mitigate some of these limitations and demonstrated substantial therapeutic benefits in a significant proportion of adults with migraine. The American Headache Society considers these medications among the first‐line options for migraine prevention, although access to them remains limited. The American Migraine Foundation hosted a single‐day, multidisciplinary expert panel discussion to identify barriers to optimal preventive care and developed recommendations to address them. Methods Participants identified and prioritized barriers and used a modified nominal group technique to achieve consensus on them. A series of moderated discussions in plenary and breakout sessions was used to create possible solutions. Modified nominal group technique was also employed to achieve consensus on the priorities among these barriers and to achieve whole‐group consensus on the recommendations. Ethical issues that inform access were discussed. Results Participants included eight neurologists and board‐certified headache specialists, six representatives of reimbursement decision‐makers, six employees of life sciences companies, four patient advocates with lived experience with migraine, and a medical ethicist. Among those who have consulted healthcare professionals and received a diagnosis of migraine, we identified four main barriers to accessing preventive treatment: restrictive prior authorization requirements, the perceived lack of real‐world evidence and treatment guidelines, the need for clinician education, and the need for patient education. Consensus recommendations for eliminating barriers centered on using new evidence to evaluate policies that restrict the selection of first‐line therapies, initiating/improving collaboration among stakeholders, sharing of data and best practices, and increased training. Participants agreed to explore novel definitions of the value of preventive treatment and to establish the Migraine Prevention Network to facilitate ongoing cooperation and collective action. However, due to financial limitations, staffing changes, and time constraints, post‐meeting discussions led to a shift from establishing a broad Migraine Prevention Network to forming smaller task forces focused on the top‐priority barriers (real‐world evidence and The Patient Playbook ) identified through collaborative voting among American Headache Society, American Migraine Foundation, and industry stakeholders. Conclusions Adults with migraine face multiple barriers in accessing novel migraine‐specific, CGRP‐targeted preventive treatment. Stakeholders in the delivery of care, including clinicians, reimbursement decision‐makers, life sciences companies, and patient and clinician advocates, may be able to overcome many of these barriers and improve access by working with and on behalf of patients.

OBJECTIVE Resection of common peroneal nerve (CPN) schwannomas is generally believed to be associated with a high risk of postoperative deficit, especially the chance for development of a foot drop. The goal of this study was to investigate the surgical results for resection of schwannomas from the CPN around the knee and specifically the chance of developing a postoperative motor deficit. METHODS Data from 36 patients with sporadic schwannomas and 8 patients with schwannomatosis (12 schwannomas total) treated at two centers were retrospectively analyzed. For sporadic cases, different locations around the knee were compared (i.e., proximal to the fibular head [FH], at the FH, and distal to the FH), taking into account the preoperative duration of symptoms, size at presentation, and surgical results of resection. The literature was systematically reviewed for reported cases by searching the PubMed and Embase databases. RESULTS A total of 24 schwannomas proximal to the FH were surgically treated: 11 at the FH, and 13 distal to the FH. For the entire cohort, the mean size of CPN schwannomas distal to the FH at presentation was smaller (1.4 × 1.8 cm) compared with those proximal to (2.0 × 2.2 cm) and at (2.2 × 2.3 cm) the FH, although these differences were not statistically significant. The mean preoperative duration of symptoms was slightly longer for schwannomas distal to the FH (35 months) than for those proximal to the FH (21 months) and at the FH (27 months); however, this difference was not significant. Postoperative deficits occurred in 3 sporadic cases: 2 patients with temporary weakness (Medical Research Council grade 4) that completely resolved within several months and 1 patient who had previously undergone surgery elsewhere and presented with deficits and in whom weakness increased after resection. Improvement in preoperative deficits was observed in 1 patient with extensor hallucis longus muscle paralysis that completely recovered. One patient with schwannomatosis developed muscle weakness after resection of a plexiform schwannoma. A systematic review of 21 previously reported cases in the literature showed that larger CPN schwannomas (> 5 cm) were more likely to result in permanent motor deficits. CONCLUSIONS This retrospective study of 44 patients shows that peroneal nerve schwannomas around the knee can be safely removed with a low risk of deficits. The systematic review of the literature suggests that larger schwannomas are more likely to result in permanent deficit. In the authors’ opinion, CPN schwannomas can best be resected, preferably when the lesion is relatively small.

Objective Prior work has identified numerous barriers to standardized outcome measurement in cleft care. The objective of this study was to evaluate a multifaceted implementation strategy to collect standardized outcomes for children with cleft lip and/or palate (CL/P). Design A one-armed, single-site pilot study of a multifaceted implementation strategy. Setting A metropolitan children's hospital. Patients Five-year-old children with CL/P. Interventions A multifaceted implementation strategy for standardized outcome measurement. Main Outcomes Measures The primary outcome measure was penetration of the intervention, defined as the proportion of children for whom the standardized measures of aesthetic, dental, and speech outcomes were collected. Penetration pre-implementation and 12 months post-implementation were compared. The secondary outcome was acceptability of standardized outcome measurement among providers and staff, evaluated with the Acceptability of Intervention Measure. Results The implementation strategy resulted in high penetration of standardized outcome measurement: measurement of aesthetic outcomes increased from 7% to 82% ( p < .0001); measurement of dental outcomes increased from 0% to 56% ( p < .0001); measurement of speech outcomes increased from 29% to 77% ( p = .0001). Acceptability of standardized outcome measurement was high, with 93% of cleft team providers and staff (n = 14/15) reporting that they somewhat or completely agreed that standardized outcome measurement met their approval, was appealing, was welcomed, and was liked. Penetration continued to improve after the implementation period, with high rates of outcome measure collection sustained for four years following implementation. Conclusions Deployment of a multifaceted implementation strategy led to substantial increases in the collection of aesthetic, dental, and speech outcomes among children with CL/P.

Respiratory muscle fatigue could occur following involuntary breathing movements during maximal breath-holds. We measured respiratory muscle strength pre and post three preparatory and maximal breath-holds. We found significant reductions in diaphragm strength and total inspiratory and expiratory strength in some, but not all, divers. Divers that had respiratory muscle fatigue had a lower pseudo O 2 delivery (HR × [Formula: see text]) during the struggle phase. This suggests that reduced oxygen delivery may contribute to respiratory muscle fatigue during a breath-hold.