bluuebunny/crossref_metadata_2025_split huggingface dataset:
831 Diagnostic and Treatment Challenges in a Child with Type 1 Narcolepsy
Authors: Nakka Sirish , Nandalike Kiran 
Published: May 2021

Abstract

Introduction
The diagnosis and management of Narcolepsy can be challenging in young children. We present a case of abrupt onset severe type 1 narcolepsy complicated by tactile hallucinations with stimulant medication therapy.

Report of case(s)
A previously healthy 7-year-old African American female presented to us at the beginning of the current pandemic with one week of excessive daytime sleepiness (EDS) with associated enuresis, hyperphagia and two episodes of cataplexy. She also presented with hypnagogic hallucinations and dream enactment behaviors. She was extremely sleepy with a score of 24/24 on the Epworth Sleepiness Scale. Her neurological exam was negative except for facial twitching appearing to be cataplectic facies. Extensive work up including urine toxicology, serology and cerebrospinal fluid (CSF) tests were undertaken to rule out infectious and demyelinating conditions; the results of these tests were negative. Brain magnetic resonance imaging as well as electroencephalogram testing were unremarkable. Overnight polysomnography demonstrated short sleep latency and rapid eye movement (REM) sleep without atonia and a multiple sleep latency test showed a mean sleep latency of less than 2 minutes and 4/4 sleep onset REM periods, highly suggestive of narcolepsy. Her HLA DQB1*0602 haplotype was positive but CSF Hypocretin testing was not done. She was given a diagnosis of Narcolepsy type I and started treatment on low dose Methylphenidate and Venlafaxine to address her EDS and cataplexy, respectively. Her EDS was gradually controlled with a higher dose of Methylphenidate but she developed severe tactile hallucinations. She complained of feeling bugs crawling under her skin and could not keep her clothes on due to constant discomfort. The tactile hallucinations improved with discontinuation of methylphenidate but reappeared with introduction of a different class of stimulants. Modafinil was ineffective in controlling her EDS symptoms. Her EDS, cataplexy and enuresis were better controlled after starting her on Sodium Oxybate. She continues to suffer from moderate to severe EDS with optimally dosed Sodium Oxybate with suboptimal response to various typical and atypical stimulants.

Conclusion
This case illustrates the difficulties in diagnosing and treating pediatric narcolepsy patients and a unique challenge of tactile hallucinations with stimulant medications.
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836 Issues with Sleep Can Be Skin Deep: Management of Obstructive Sleep Apnea in Cutaneous Sarcoidosis
Authors: Okorie Ugorji , Koothirezhi Rupa , Anne Pratibha , Monceaux Brittany , Liendo Cesar , Chernyshev Oleg 
Published: May 2021

Abstract

Introduction
Introduction/Background: PAP Intolerance is a common initial complaint among new PAP users, with mask issues and difficulty with pressure level leading the list. In clinical practice, feelings of claustrophobia and anxiety are usually cited as the reason for PAP intolerance. Sometimes craniofacial impairments such as congenital malformations or change in facial architecture after a major accident or surgery may play a role. Our case describes our experience with a patient afflicted with severe sinonasal cutaneous Sarcoidosis.

Report of case(s)
Case Description: A 43 year old AAF with a PMH of morbid obesity, depression and cutaneous sinonasal Sarcoidosis was referred to Sleep Medicine by ENT in late May 2020 with complaints of snoring, witnessed apneas, excessive daytime sleepiness, fatigue, and non-restorative sleep for &gt;20 years. The patient was first diagnosed with cutaneous Sarcoidosis limited to the internal sinonasal region around 2015 and managed by ENT until the lesions spread to her face. Due to this, the patient developed depression and became increasingly non-complaint with her medical regimens. As a result, her sarcoidosis and other co-morbidities worsened. Diagnostic Polysomnogram (PSG) in May 2020 showed presence of Hypopnea predominant Obstructive Sleep Apnea (OSA) with AHI 35.9 and O2 desaturation to 58%. Subsequently, the patient was placed on auto CPAP 6–20 cm H2O with a DreamWear FFM. At follow up in September 2020, her sarcoidosis had spread to her upper lip area, so she was prescribed a Fit Life mask to avoid this region. Follow up in December 2020 revealed improvement in Sarcoidosis and accompanying vast improvement in objective compliance though she reverted to using her DreamWear FFM due to “mask seal issues. An AirFit f20 mask was then given to help avoid the active lesions on her upper lip.

Conclusion: Discussion/Conclusion
In patients with compromised facial architecture, special attention must be paid to the type of mask interface used so it does not aggravate sensitive areas which may lead to poor compliance with PAP therapy. Instructions were given for her to alternate between her three masks—the Fit Life, the DreamWear FFM and the AirFit f20 FFM—in an effort to reduce irritation on her face over time.
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838 Not All Hypercapnia is COPD
Authors: Te Tue , Emanuel Hina , Velamuri Kanta , Singh Supriya 
Published: May 2021

Abstract

Introduction
Sleep breathing disorder related to isolated unilateral or bilateral diaphragmatic dysfunction (DD), in the absence of a generalized neuromuscular disorder, is not well understood and often under-recognized. There have been only a few cases reported of apneas and hypoponeas during REM sleep due to diaphragmatic dysfunction. We present here, a case of an 62 year-old man who developed acute hypercapnic respiratory failure with presumed COPD exacerbation requiring invasive ventilation.

Report of case(s)
A 62-year-old man was found on the sidewalk extremely short of breath and was intubated in the emergency department. Initial post-intubation arterial blood gas showed pH 7.1, pCO2 82, pO2 263, CO3 25.5. CXR showed no infiltrates. Echocardiography showed EFof 55%-65%. Long-term tobacco use supported the picture of COPD exacerbation. However, PFT was within normal limits. HSAT one year prior which showed severe OSA with AHI 52.6 event per hour. Patient had not pursued positive airway pressure (PAP) titration study afterward. In ICU, he was treated for presumed COPD exacerbation and successfully weaned off invasive ventilation. Inpatient PAP titration study recommended IPAP 12 and EPAP 8 cm H20. A fluoroscopy of the diaphragm was performed and showed that the right diaphragm had limited mobility. Electromyogram did not show generalized myopathy.

Conclusion
In conclusion, this case report describes the presentation of sleep disordered breathing seen in patients with unilateral diaphragmatic palsy. In these patients, the respiratory events seen are mainly hypopneas and desaturations, worse in REM sleep and supine position. This was an unusual presentation of a patient with untreated OSA and unilateral diaphragmatic palsy. A characteristic finding in these patients is worsening of the OSA in supine position. This has been reported in several studies and was seen in our case as well. This case underscores the need for critical thinking and diagnostic reasoning in the evaluation of a patient with hypercapnic respiratory failure and consider a wide differential and not only COPD exacerbation as the cause. Unilaterally diaphragmatic palsy is a rare cause of hypercapnic respiratory failure but must be considered when seen with obstructive sleep apnea with predominantly hypopneas and hypoxemia out of proportion of the respiratory events.
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847 Rapid Improvement of Pulmonary Hypertension with Adequate Ventilatory Support in Patient with Prader-Willi Syndrome
Authors: McHugh Erin , Gubichuk Megan , Malhotra Sonal 
Published: May 2021

Abstract

Introduction
Prader-Willi syndrome (PWS) is a genetic multi-system disorder characterized by developmental delay, hyperphagia, and often morbid obesity. Patients can have impairments in ventilatory control and are at risk for sleep disordered breathing due to craniofacial abnormalities, obesity, hypothalamic dysfunction, hypotonia and respiratory muscle weakness. This places them at higher risk for obstructive sleep apnea (OSA), hypoventilation and, if left untreated, may lead to cardiovascular complications. We present a patient with pulmonary hypertension (PH) in the setting of PWS and under supported OSA.

Report of case(s)
A 17-year-old female with PWS, obesity, type II diabetes mellitus, developmental delay and severe OSA, non-adherent to positive airway pressure (PAP) therapy, presented with 6 months of pedal edema, weight gain with acute shortness of breath, fatigue, and decreased appetite. Upon arrival she developed hypoxemic, hypercapnic respiratory failure requiring intubation. Echocardiogram two months prior to admission showed normal right ventricular size and function with normal septal configuration. Echocardiogram after intubation showed signs of PH with dilated and hypokinetic ventricles compared to prior exam, worsening tricuspid valve regurgitation, and septal bowing. Sleep history was notable for severe OSA in 2017 with obstructive apnea-hypopnea index (oAHI) of 22.6, oxygen nadir of 74%, peak transcutaneous pCO2 of 51 mmHg. Patient was lost to follow-up and as per our history, was non-adherent to PAP therapy. On admission she was started on aggressive diuresis and tadalafil. After successful extubation to BPAP and wean to room air while awake, a BPAP titration PSG was performed. It demonstrated low baseline oxygen saturations (93%), an oxygen nadir of 70%, transcutaneous CO2 peak of 57mmHg with obstructive events eliminated with BPAP of 24/14 cmH20 and hypoxemia treated with 4L supplemental oxygen. With adequate respiratory support with sleep combined with PH monotherapy, repeat echocardiogram prior to discharge showed improved systolic function and only mild septal flattening.

Conclusion
It is unclear the prevalence of PH in patients with PWS independent of OSA, however it is likely increased overall secondary to the increased prevalence of OSA. As a result, patients with PWS may benefit from routine interval echocardiogram to monitor for signs of PH.
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850 REM Sleep and ST Deviation in Acute Myocardial Infarction
Authors: Ramaniuk Aliaksandr , Carbajal-Mamani Semiramis , Hadigal Susheela , Ricaurte Basma 
Published: May 2021

Abstract

Introduction
Sleep stage architecture and amount of REM sleep have been associated with mortality and clinical recovery, without clear etiology. Patients recovering from critical illness frequently experience sleep disturbances, episodic arrhythmogenesis, EKG changes. This case aims to add to current field of study and describes an unusual pattern of sleep stage dependent, hypoxia independent, ST segment variation, which may benefit from further exploration and utilization of polysmongoraphy (PSG) in the immediate post acute MI period.

Report of case(s)
46 year old female with history of smoking, obesity, and diabetes presented for a sleep medicine evaluation, four days following a hospitalization for non ST elevation myocardial infarction (NSTEMI) and percutaneous coronary intervention. Her split night PSG data revealed severe obstructive sleep apnea (OSA) with apnea hypopnia index (AHI) of 131. Patient did not report acute cardiac symptoms during overnight sleep evaluation. On close observation of PSG data, the patient had grossly evident baseline ST segment depression during wake period. The ST depression persisted through stages 1 and 2 with unchanged morphology. During Stage 3, the ST segment showed progressive elevation to near the isoelectric line. During REM sleep without positive airway pressure (PAP), ST segment was noted at or near isoelectric line, even in the setting of hypoxia with saturation (Sao2) of 75%. During REM Sleep with PAP, the ST segment remained at the isoelectric line, and returning to baseline depression during wake phase while on BiPAP.

Conclusion
Residual ST segment deviation, and its resolution, are strong predictors of prognosis in patients with MI. Prior studies focused on hypoxic tolerance and sleep disordered breathing, with limited attention on specific sleep stage evaluation. REM sleep has been described as potentially having restorative effect on ischemic myocardium. Additionally, the transition period from non REM to REM sleep was reported to provide potential for myocardial restoration. PSG with cardiac monitoring remains a unique tool in further assessment of a possible association. This case aims to bring attention to the potential association of EKG ST segment variation with sleep stages, especially REM and S3, independent of hypoxia.
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858 Transitions to Virtual Administration of CBT-I: Strengths & Barriers
Authors: Nguyen Anna Marie , Campbell Rebecca , Mobley Alita , Vargas Ivan 
Published: May 2021

Abstract

Introduction
In March 2020, the University of Arkansas Psychological Clinic began social distancing resulting in a CBT-I group (four patients, 3 trainee co-leaders) transitioning from in-person meetings to telehealth via Webex. Two co-leaders led the group while the third disseminated electronic materials and scored the patients’ online sleep diaries. Virtual administration of CBT-I had benefits and challenges.

Report of case(s)
Firstly, many of the patients were familiar with teleconferencing as their jobs also transitioned to virtual meetings and could identify environments with limited interruptions. Telehealth group etiquette such as muting audio, timely diary submission, and peer support were easily mastered. However, one patient had poor internet connection, which necessitated switching between their laptop and phone to connect and seemed to negatively impact engagement with the group. Furthermore, the clinicians were confronted with the difficult decision to continue helping troubleshoot technical issues or continue with the session. During in-person visits, co-leaders could address difficulties with sleep prescription or questions about the material; however, balancing technical issues, answering questions, and facilitating support amongst the patients via telehealth was often distracting and inefficient. From a clinician perspective, it was difficult to co-lead the group due to patients respectfully muting their mics, resulting in less contributions to discussion. Additionally, many of the administrative tasks typically completed with clinic staff after an in-person session were completed during session, making it challenging to maintain confidentiality. Presentation of visual aids was difficult as patient using cellphones to connect could not view and download files while connected to session. Finally, basic clinical techniques were impacted using the virtual platform. For instance, “sitting in silence” was less effective while maintaining eye contact with the entire group was facilitated by looking directly at the camera. Unfortunately, reading facial expressions and non-verbals was often more difficult and clinicians relied on more direct questions rather than open-ended questions.

Conclusion
These challenges provide opportunities to learn how to make CBT-I via telehealth more effective. Despite the challenges of transitioning to telepsychology, the CBT-I group was effective as evidenced by high satisfaction ratings from patients, reductions in PHQ-9 and ISI scores, SOL, WASO, and increases in sleep efficiency.
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A Gender Gap in Publishing? Understanding the Glass Ceiling in Pediatric Orthopaedic Surgery
Authors: Johnson Mitchell A. , Mulvey Hillary , Parambath Andrew , Anari Jason B. , Wall Lindley B. , Shah Apurva S. 
Published: May 2021

Background:
Women are underrepresented across most surgical specialties and especially in the field of orthopaedic surgery. Despite pediatric orthopaedic surgery being one of the most gender diverse subspecialties in orthopaedics, women may still face barriers to academic advancement. Research presentations at national meetings and publication record are important drivers of advancement in the field of academic orthopaedic surgery. The aim of this study is to assess whether research abstracts authored by women are less likely to be published than abstracts authored by men.

Methods:
Abstracts from research podium presentations given at the Pediatric Orthopaedic Society of North America annual meetings from 2006 to 2020 were reviewed to assess research quality and determine basic study characteristics. For each abstract, author gender was determined through a search of institutional websites and professional networking websites for gender-specific pronouns. Resulting publications corresponding to the 2006 to 2018 were identified using a systematic search of PubMed and Google Scholar databases. Kaplan-Meier inverse survival analysis with log rank test were used to determine differences in publication rates based on whether the last (senior) author was female versus male. Multivariate, binary logistic regression was performed to assess factors predictive of eventual publication.

Results:
One thousand five hundred and eighty-one of 1626 (97.2%) of abstracts from 2006 to 2020 had an identifiable last author gender, with 17.8% (281/1581) female. No differences in study quality were identified across genders including sample size, level of evidence, or impact factor of journal if leading to publication. Women were more likely to author abstracts in foot, ankle, or lower extremity surgery [17.8% (50/281) vs. 12.9% (168/1300), P=0.032] and less likely to author abstracts focusing on the hip [11.0% (31/281) vs. 17.1% (222/1300), P=0.012]. Abstracts with women as the last author were significantly less likely to be published compared with abstracts with men as the last author [59.6% (143/240) vs. 67.9% (783/1154), P=0.013]. Multivariate analysis demonstrated that last author female gender was predictive of a lower likelihood of publication (odds ratio: 0.684, 95% confidence interval: 0.513-0.912, P=0.010). Women were less likely to be the last author of abstracts presented by study groups [2.1% (6/281) vs. 5.5% (71/1300), P=0.019].

Conclusion:
In pediatric orthopaedic surgery, abstracts authored by women are less likely to reach publication, despite no identifiable differences in study quality. Reasons for this discrepancy must be explored including insufficient mentorship, exclusion from study group participation, or potential bias against female researchers in the field of orthopaedic surgery.

Level of Evidence:
Level IV.
A Trend-Based Early Warning Score Can Be Implemented in a Hospital Electronic Medical Record to Effectively Predict Inpatient Deterioration
Authors: Bell David , Baker John , Williams Chris , Bassin Levi 
Published: May 2021

OBJECTIVES:
To determine whether a statistically derived, trend-based, deterioration index is superior to other early warning scores at predicting adverse events and whether it can be integrated into an electronic medical record to enable real-time alerts.

DESIGN:
Forty-three variables and their trends from cases and controls were used to develop a logistic model and deterioration index to predict patient deterioration greater than or equal to 1 hour prior to an adverse event.

SETTING:
Two large Australian teaching hospitals.

PATIENTS:
Cases were considered as patients who suffered adverse events (unexpected death, unplanned ICU transfer, urgent surgery, and rapid-response alert) between August 1, 2016, and April 1, 2019.

INTERVENTIONS:
The logistic model and deterioration index were tested on historical data and then integrated into an electronic medical record for a 6-month prospective “silent” validation.

MEASUREMENTS AND MAIN RESULTS:
Data were acquired from 258,732 admissions. There were 8,002 adverse events. The addition of vital sign and laboratory trend values to the logistic model increased the area under the curve from 0.84 to 0.89 and the sensitivity to predict an adverse event 1–48 hours prior from 0.35 to 0.41. A 48-hour simulation showed that the logistic model had a higher area under the curve than the Modified Early Warning Score and National Early Warning Score (0.87 vs 0.74 vs 0.71). During the silently run prospective trial, the sensitivity of the deterioration index to detect adverse event any time prior to the adverse event was 0.474, 0.369 1 hour prior, and 0.327 4 hours prior, with a specificity of 0.972.

CONCLUSIONS:
A deterioration prediction model was developed using patient demographics, ward-based observations, laboratory values, and their trends. The model’s outputs were converted to a deterioration index that was successfully integrated into a live hospital electronic medical record. The sensitivity and specificity of the tool to detect inpatient deterioration were superior to traditional early warning scores.
In-hospital Complications Are More Likely to Occur After Reverse Shoulder Arthroplasty Than After Locked Plating for Proximal Humeral Fractures
Authors: Köppe Jeanette , Stolberg-Stolberg Josef , Rischen Robert , Faldum Andreas , Raschke Michael J. , Katthagen J. Christoph 
Published: May 2021

AbstractBackgroundCurrently, there seems to be a paradigm change in the surgical treatment of proximal humeral fractures in patients aged 65 years and older, with a considerable increase in the use of reverse total shoulder arthroplasty (RTSA) compared with angular stable internal fixation (locking plate fixation). However, even among shoulder specialists there is controversy regarding the best treatment strategy.Questions/purposesTo evaluate for (1) a greater risk of in-hospital major adverse events, (2) a greater risk for in-hospital surgical complications, and (3) a greater risk of 30-day mortality, locking plate fixation and RTSA were compared for the treatment of proximal humerus fractures of patients aged 65 years and older after controlling for potentially confounding variables in a large-database analysis.MethodsHealth claims data of the largest German insurance company including approximately one-third of the population (26.5 million policyholders) between 2010 and 2018 were analyzed. This database was chosen because of its size, nationwide distribution, and high quality/completeness. In total, 55,070 patients (≥ 65 years of age) treated with locking plate fixation (75% [41,216]) or RTSA (25% [13,854]) for proximal humeral fracture were compared. As primary endpoints, major adverse events (including acute myocardial infarction, stroke, organ failure, resuscitation, and death) and surgical complications (infection, hematoma, loss of reduction, dislocation, and revision surgery) were analyzed. The risk of all endpoints was analyzed with multivariable logistic regression models in the context of comorbidities to address existing group differences.ResultsAfter controlling for potentially confounding variables such as age, sex, and risk profile, RTSA was associated with a higher risk for major adverse events (OR 1.40 [95% CI 1.29 to 1.53]; p < 0.001) and surgical complications (OR 1.13 [95% CI 1.05 to 1.21]; p < 0.01) compared with locking plate fixation. There was no evidence for an increase in mortality (OR 0.98 [95% CI 0.86 to 1.12]; p = 0.81).ConclusionThe increased in-hospital risk for major adverse events and surgical complications may moderate the enthusiasm associated with RTSA for proximal humeral fractures in patients 65 years and older. Treatment decisions should be based on individual risk estimation to avoid potential harmful events. Future studies must include long-term outcomes and quality of life to enlighten these findings in a broader context.Level of EvidenceLevel III, therapeutic study.