bluuebunny/crossref_metadata_2025_split huggingface dataset:
0901 An odd presentation of a hidden curse
Authors: Buntak Vesna , Chang Anna , Loutoo Ariane , Bham Zubin , Kwon Jeff 
Published: May 2023

Abstract

Introduction
Congenital central hypoventilation syndrome (CCHS) is a rare disorder of respiratory deficiency due to a mutation in the PHOX2B gene. Clinical presentation may be mild in certain individuals due to different levels of ventilatory dysregulation.

Methods
We present the findings of exogenous ventilatory challenge testing and polysomnography (PSG) performed on a normocapnic adult female with late-onset (LO) CCHS patient.

Results
The patient was exposed to four separate ventilatory challenges. During exposure to hyperoxia using 100% oxygen (O2) administration, patient showed an inconsistent ventilatory response with a blunted and delayed increase in regional tissue oxygen saturation (rSO2). During hyperoxic hypercapnia with 5% carbon dioxide (CO2) and 95% O2, she demonstrated a reduced ventilatory response based on respiratory rate and tidal volume. During hypoxic hypercapnia, 7% CO2 and 14% O2, patient demonstrated a delayed and markedly attenuated ventilatory response. Hypoxia; 100% nitrogen inhalation did not result in increased ventilation. Ve slope, change in minute ventilation/change in end-tidal CO2 (EtCO2), with hypercapnia challenges was 4.88, which is lower than normal. Ventilatory EtCO2 threshold was 36 mmHg. PSG showed apnea-hypopnea index 3.3 with hypoxemia during rapid eye movement (REM), with an oxygen saturation nadir of 84%. EtCO2 baseline was around 36, peak was 50 and was associated with REM-related apnea events. Arterial blood gas was unremarkable.

Conclusion
CCHS is characterized by abnormal respiratory response to exogenous ventilatory challenge testing. Hyperoxia silences peripheral chemoreceptors (PC) responsible for stimulating ventilation in normal individuals. Hyperoxic hypercapnia silences PC but stimulates central chemoreceptors (CC). Hypoxic hypercapnia stimulates hyperventilation via both PC and CC. Our patient demonstrated inconsistent and reduced ventilatory responses to all four challenges. As expected, the classic chemosensory parameter, Ve slope, for hypercarbia challenge was markedly reduced. However, the ventilatory EtCO2 threshold fell well below threshold typically observed in CCHS. PSG noted greater alveolar hypoventilation during REM compared to non-REM, which is also atypical for CCHS. In conclusion, exogenous ventilatory challenge testing demonstrated derangements in ventilatory control typical in CCHS. Despite this, our patient had no evidence of daytime hypoventilation with normal CO2 threshold. This case illustrates the phenotypic variability present in LO-CCHS.

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0903 CPAP adherence rate in patients with ischemic stroke
Authors: Kim Jee Hyun , Song Tae-Jin , Park Moo-Seok , Kim Hyung Jun , Kim Dong Yeop 
Published: May 2023

Abstract

Introduction
Although treatment of OSA in patients with ischemic stroke is important for secondary prevention of stroke and improving neurologic outcomes, patients with stroke often are not adherent to nasal CPAP. The aim of the study is to examine the adherence rate and explore the predictive factors for CPAP compliance in patients with a recent history of ischemic stroke.

Methods
We reviewed the medical records of patients with ischemic stroke who were diagnosed with OSA after the onset of stroke and those who were recommended to use nasal CPAP at a single university Hospital from March 2020 to November 2022. Demographic information, neurologic functional status, and polysomnographic data were reviewed. For patients who agreed to use CPAP, CPAP compliance data were analyzed using cloud data run by various CPAP companies.

Results
76 patients with cerebral infarction or transient ischemic attack were diagnosed with OSA and were recommended to use nasal CPAP (mean age, 60.8±12.6, male 60). Night PSG was performed after the onset of the stroke as the evaluation process for risk factors for stroke based on clinical symptoms such as snoring or apnea. The acceptance rate for nasal CPAP was 88.1% (67/76) at the first visit after the PSG. The adherence rate was 55.2% (37/67). No difference between the adherent and non-adherent groups was found in polysomnographic variables, including AHI, RDI, and subjective sleep scales such as PSQI, ISI, and ESS. No difference in the neurologic functional deficit was found between the groups. The adherent group was younger but not statistically significant (58.4±13.10 vs. 63.6±11.65, p=0.10). Female patients with ischemic stroke were more intolerant to CPAP (24.1% vs. 11.7%).

Conclusion
The patients in our study performed a PSG due to the risk evaluation of stroke rather than pursuing treatment for sleep apnea symptoms. Half of the patients were not adherent to CPAP, although they agreed to try CPAP. The proper screening of sleep apnea and education on sleep apnea and its impact on the neurologic outcome would be mandated to increase CPAP adherence in patients with ischemic stroke.

Support (if any)
Basic Science Research Program through NRF funded by the Ministry of Education(2021R1I1A1A01059791)
0905 Factors Associated with Sleep Quality among Men with Multiple Sclerosis
Authors: Lorenz Rebecca , Alanazi Nouf 
Published: May 2023

Abstract

Introduction
Multiple sclerosis (MS), a neurodegenerative disease with typical onset in young to middle adulthood, results in disrupted sleep more commonly than the general population. There are known sex differences in MS onset and clinical course. MS, however, affects approximately three times more women than men, resulting in less attention to the male experience. Thus, there is a lack of information on disrupted sleep among men with MS. The purpose of this study was to examine the relationships between personal factors, behaviors, and sleep quality among men with MS.

Methods
Men with MS aged 20 to 90 years participated in an online anonymous survey recruited through the National MS Society website. Pearson’s correlations and linear regression were used to examine relationships between personal factors (age (years), type of MS, years since MS was diagnosed by a physician and race), sleep promoting behaviors (Sleep Behavior Self-Rating Scale), and sleep quality (Sleep Impact Short-Form total score).

Results
Four hundred ninety-six men with MS (mean age=53 years; SD=12.8; range 22-81 years) completed the survey. The majority were white (n=428; 86%) and married (n=358; 72%), with relapsing-remitting MS (n=323; 65%). Sleep quality ranged widely (mean=43.2; SD=3; range 27.9-59.9) with 90% reporting poor sleep. Pearson correlation results indicated a significant positive association between sleep quality and sleep promoting behaviors (r=.339) and negative associations with age (r=-234) and race (r=-.148). Multiple regression showed that sleep promoting behaviors and age statistically significantly predicted sleep quality (F(5,399) =15.51,p&lt;.001,R2=.152). MS type, years passed since MS diagnoses and race did not add significantly to the prediction.

Conclusion
These findings suggest that poor sleep quality was strongly associated with sleep promoting behaviors among in older men with MS. Improving sleep promoting behaviors may substantially improve sleep quality. Further qualitative research is required to address the lack of information on understanding factors that disturb sleep among men with MS.

Support (if any)
This study was supported by National Heart, Lung, and Blood Institute (NHLBI) UB Clinical Scholar Program in Implementation Science to Achieve Triple Aims -NIH K12 Faculty Scholar Program in Implementation Science (NHLBI) Award
0908 Multi-modal Recruitment of Individuals with REM Sleep Behavior Disorder into a Longitudinal Prodromal Parkinson’s Study
Authors: Dobkin Roseanne , Kuhl Maggie , Destro Christina , Brown Ethan , Chahine Lana , McMahon Bridget , Ricci Jillian , Videnovic Aleksandar , Kelliher Caitlin , Lynch Kimberly , Islam Natasha , Simuni Tanya , Siderowf Andrew , Tanner Caroline , Marek Ken 
Published: May 2023

Abstract

Introduction
In 2010, The Michael J. Fox Foundation launched the Parkinson’s Progression Markers Initiative (PPMI) to study how Parkinson’s disease (PD) starts and changes over time. Volunteers participate in clinic, online or both. PPMI is now prioritizing recruitment of individuals with possible or probable REM sleep behavior disorder (RBD) without a PD diagnosis (goal of 500 in clinic). As 30% of people with RBD and smell loss receive a PD diagnosis within four years, RBD may provide a model to understand the evolution of the prodromal phase of PD. This presentation will describe diverse recruitment strategies utilized since 2020 to enroll individuals with RBD, to inform best practices for engaging this population in research.

Methods
A key strategy for identifying individuals with possible RBD was the dissemination of educational content on the disorder and its connection to PD. Materials emphasized that not everyone with RBD develops PD but that, in some people, RBD is an early symptom of the disease. Educational materials and messages (with associated calls to actions) were shared via webinars, print and online publications, emails, animated videos, radio, TV, and paid social media ads. Materials included scientific, participant and influencer spokespeople and were targeted to both individuals with RBD and bed partners. PPMI study sites sought referrals from sleep physicians.

Results
More than 535 individuals with a self-reported diagnosis of RBD (without PD) have enrolled in the online PPMI platform (59.4% male, 68.1% aged ≥60). Nearly 185 have been screened for site enrollment; 111 are contributing data at a study site. Foundation-led emails (16.1%) and paid social media ads (9.3%) were highest drivers to online enrollments. The most common referral sources to a PPMI RBD information and screening phone line were email (38%), social media (19%), and family/friend referral (19%). Approximately one-fourth of clinic enrollments were from physician referrals.

Conclusion
Multi-modal recruitment strategies, linked to tailored educational content, are critical for enrollment of individuals with RBD in clinical research studies including, but not limited to, those investigating and aiming to prevent other neurologic disorders.

Support (if any)
PPMI – a public-private partnership – is funded by the Michael J. Fox Foundation for Parkinson’s Research.
0909 Pitolisant Pregnancy Registry: An Observational Study of the Safety of Pitolisant Exposure in Pregnant Women and Their Offspring
Authors: Adamkovic Anthony , Montgomery Padge , Roy Michele , Dayno Jeffrey , Veley Kristin 
Published: May 2023

Abstract

Introduction
WAKIX (pitolisant) is a potent and highly selective histamine 3 receptor antagonist/inverse agonist approved for the treatment of excessive daytime sleepiness or cataplexy in adult patients with narcolepsy. The safety of pitolisant in pregnant women has not been established. Available case reports from clinical trials and postmarketing experience have not demonstrated a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. The registry is a prospective, observational cohort study designed to evaluate the association between pitolisant exposure during pregnancy and subsequent maternal, fetal, and infant outcomes.

Methods
The primary study population consists of 3 cohorts: pregnant women with a diagnosis of narcolepsy who are exposed to pitolisant during pregnancy, pregnant women with narcolepsy who are unexposed to pitolisant but exposed to comparator products during pregnancy, and pregnant women with narcolepsy who are neither exposed to pitolisant nor comparator products. Comparator products include modafinil, armodafinil, sodium oxybate, oxybate mixed salts, solriamfetol, methylphenidate, and amphetamines. Outcomes of interest include major congenital malformation (primary outcome), minor congenital malformation, pre-eclampsia, eclampsia, spontaneous abortion, stillbirth, elective termination, small for gestational age, preterm birth, postnatal growth deficiency, and infant developmental deficiency. Data are collected from enrolled pregnant women and healthcare providers involved in their care or the care of their infants, if applicable. Only data that are routinely documented in patients' medical records during usual care are collected. Participation in the registry is voluntary and participants may withdraw their consent to participate at any time. The registry design follows current FDA guidance for designing and implementing pregnancy exposure registries.

Results
The registry was initiated in May 2021. Presently, data are insufficient to assess an association between pitolisant exposure and any endpoint of interest. Study completion is planned in 2030.

Conclusion
Healthcare providers are encouraged to discuss this voluntary pregnancy registry with their eligible patients and assist women who are interested in enrolling. The study is registered on Clinicaltrials.gov (ID# NCT05536011).

Support (if any)
This study is sponsored by Harmony Biosciences LLC.
0910 Potential Role of Physicians in Addressing the Needs of Those At-Risk of a Neurodegenerative Disorder – A Pilot Survey
Authors: Levendowski Daniel , Berka Chris , Neylan Thomas C , Mazeika Gandis , Walsh Christine , Boeve Bradley , St. Louis Erik 
Published: May 2023

Abstract

Introduction
In a recent study, most patients diagnosed with isolated REM sleep behavior disorder (iRBD) preferred their physician discussed their risk for developing a future neurodegenerative disorder (NDD) such as parkinsonism or dementia (PD). In the Mayo Clinic email survey, 75% would lose trust in their physician if PD-risk was not discussed following an iRBD diagnosis. For this survey, questions were designed to investigate preferences for how sleep physicians might communicate to community-based adults about risk of a NDD.

Methods
A survey was administered to Rotary Club attendees prior to a sleep and neurodegenerative disease presentation, with options for responses being either strongly disagree, disagree, neutral, agree, or strongly agree. Thirty-four surveys were obtained from approximately equal numbers of men and women with estimated ages above 50 years. Personal information were not obtained (i.e., sex and age).

Results
The vast majority of respondents wanted their physician to ask their preference prior to sharing NDD risk (86% strongly agreed or agreed), while virtually all would lose trust in their physician if he/she did not discuss their NDD risk (97% strongly agreed/agreed). If identified with NDD risk, responders would seek out information about medications and adaptive therapies that could delay onset (97% strongly agreed/agreed), while only about half currently had a good understanding of lifestyle patterns that might help delay onset (47% strongly agreed/agreed and 47% disagreed/strongly disagreed). Not knowing when memory problems might begin would cause most responders to feel anxious (85% strongly agreed/agreed).

Conclusion
Those surveyed wanted their physician to ask their preference prior to sharing NDD risk, but virtually all would lose trust in their health care provider if the information was known but not shared. There appears to be a role for physicians in educating those who may be at NDD risk about interventions that could delay onset of NDD, given approximately half have a limited understanding of lifestyle patterns that could help delay onset. Further, routine follow-up for changes in prodromal NDD biomarker severity might help to reduce anxiety in the 85% of those concerned about when memory problems might begin.

Support (if any)
NIA-R44AG050326
0919 EMR-Integrated Clinical Pathway Improves Familiarity with Obesity Hypoventilation Syndrome Among Inpatient Clinicians
Authors: Camacho Jessica , Andani Sameer , Drake Lauren , Green Katherine 
Published: May 2023

Abstract

Introduction
Obesity hypoventilation syndrome (OHS) is an underrecognized chronic respiratory condition characterized by obesity (BMI &gt; 30kg/m2) and hypoventilation (PaCO2 &gt; 45mmHg) in the absence of other causes of hypoventilation. In OHS, obesity causes mass loading on the respiratory system, leading to CO2 retention, diminished ventilatory response, progressive hypoxia/hypercapnia, and eventual cardiopulmonary arrest. OHS is typically diagnosed while inpatient, wherein 18-month mortality approaches 23%. Non-invasive ventilation (NIV) improves OHS symptoms & prognosis which subsequently decreases healthcare utilization and mortality. With the proper tools and training, inpatient providers can facilitate accurate diagnosis and prompt treatment of OHS. Our team developed an EMR-integrated clinical pathway to increase recognition and facilitate treatment of OHS in hospitalized patients on the general medicine service.

Methods
The OHS Pathway was implemented among a large group of hospitalists at a tertiary academic medical center. A multi-disciplinary team of physicians, respiratory therapists, and care coordinators designed the pathway structure and content. Prior to integrating the OHS pathway into the EMR, hospitalists were surveyed to assess existing knowledge and experiences caring for patients with OHS. Hospitalists then participated in an educational series on OHS with orientation to the pathway. 12-months into the intervention, hospitalists were re-surveyed.

Results
Survey data analysis revealed that providers were significantly more familiar with the diagnostic criteria for OHS after (n=40) the introduction of the clinical pathway rather than before the introduction (n=24) of the pathway (t (62) = 2.88, p = .0027). Subjective success rates for prescribing NIV to patients upon discharge did not significantly improve

Conclusion
OHS diagnosed in the inpatient setting carries significant morbidity and mortality. Proper detection and prompt treatment of OHS are essential to improving the care and prognosis of this high-risk population. We demonstrate that our multi-disciplinary based, EMR-integrated clinical pathway increased provider familiarity with the diagnostic criteria for OHS. While treatment success did not significantly improve, numerous systems factors were at play, and further investigations are needed to address access to treatment.

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0923 Poor sleep hygiene is widespread among first-year medical students
Authors: Marquardt Matthew , Fisher Fielding , Grandner Michael , Khan Meena 
Published: May 2023

Abstract

Introduction
Poor sleep health is widespread within healthcare and is notoriously neglected during residency; however, little is known about sleep quality during the pre-clinical years of medical school. Sleep hygiene is broadly defined as the sleep-adjacent behaviors and beliefs that significantly contribute to sleep quality. This study sought to determine the prevalence of various sleep hygiene related behaviors among first-year medical students.

Methods
A one-time, 130-item survey was distributed to the first-year medical school class three months into the first semester. Quantitative and open-text questions sought to understand student demographics, mental health, sleep quality, and sleep-related behaviors, beliefs, and knowledge. Mean bedtimes were grouped based on when students drank their last caffeinated beverage and compared using one-way analysis of variance (ANOVA) with the Turkey HSD test. Statistical significance was considered a p-value ≤0.05.

Results
138 of the 209 person (66%) first-year medical school class completed the survey. Demographics mirrored those of the whole class: 46% male, 54% female, and average age 23.5. While only 15% of students reported pulling an all-nighter (obtaining less than 2 hours of sleep over 24 hours) during the current academic year, many other behaviors associated with poor sleep hygiene were prevalent. Large proportions of students reported using social media (78%), texting (68%), watching television (41%), and thinking (53%) in bed more than three times per week. Caffeine use was also high. Only 12 respondents (8.7%) reported never consuming caffeinated products, while just over 50% consumed caffeine at least once per day. Furthermore, of the 126 students who reported caffeine use at least once per month, 74 (59%) drank their last caffeinated beverage after 2:00 p.m. Intriguingly, average weekday bedtimes did not differ based on when students consumed their last caffeinated beverage (p=0.059), suggesting that caffeine consumption is driven by factors other than predicted bedtime.

Conclusion
Poor sleep hygiene is widespread among first-year medical students. Future interventions should seek to increase awareness among medical students about sleep hygiene and its importance while providing them with feasible tips for how to incorporate them into their lives.

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0926 Teaching Medical Students About Insomnia: Early Results of a Brief Lecture for Third Year Medical Students
Authors: Tubbs Andrew , Karp Jordan , Parthasarathy Sairam , Perlis Michael , Grandner Michael 
Published: May 2023

Abstract

Introduction
Insomnia affects 10 to 20 percent of Americans annually. Unfortunately, evidence-based training on the diagnosis and management of insomnia during U.S. undergraduate medical education is limited. This educational gap may promote use of off-label medications or supplements (e.g., melatonin) and contribute to the “medicalization of sleeplessness”. By contrast, cognitive behavioral therapy for insomnia (CBT-I) is the first-line recommendation of the American Academy of Sleep Medicine and the American College of Physicians, and CBT-I is increasingly available in a digital format that can even be prescribed by primary care providers. Most physicians are not trained in how CBT-I works, however, and thus are ill-equipped to prescribe or refer this service. Therefore, this pilot study evaluated a brief educational intervention to improve medical student knowledge about insomnia.

Methods
The first author (AST) developed a 20-minute in-person lecture on insomnia which was delivered to 122 undergraduate medical students during their third-year medical curriculum. Topics included epidemiology, Spielman’s 3-P model of pathophysiology, and the treatment recommendations from the AASM with an emphasis on CBT-I. A total of 43 students (36%) provided informed consent and provided pre- and post-presentation ratings (0–100-point scale) of 1) their comfort in discussing insomnia with their patients, 2) the importance of insomnia for human health, 3) their familiarity with the pathophysiology of insomnia, and 4) their familiarity with treatments for insomnia. Participants were additionally asked to categorize a list of treatments as evidence-based or not.

Results
Following the presentation, students reported increased comfort in discussing insomnia with their patients, knowledge of the pathophysiology of insomnia, and knowledge of evidence-based treatments. In selecting treatments, more students chose CBT-I as an evidence-based treatment, while fewer students identified sleep hygiene, PAP therapy, diphenhydramine, hydroxyzine, melatonin, quetiapine, and trazodone.

Conclusion
A brief lecture on insomnia improved medical students’ knowledge of the disease process and evidence-based treatments for insomnia. Forthcoming analyses will determine whether students utilized this knowledge during subsequent clerkships. Future work will examine whether alternative formats (e.g., flipped-class, case-based instruction, interactive modules) are more effective for conveying this information.

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