bluuebunny/crossref_metadata_2025 huggingface dataset:
On the emergent system mass function: the contest between accretion and fragmentation
Authors: Clark Paul C , Whitworth Anthony P
Published: October 2020

We propose a new model for the evolution of a star cluster’s system mass function (SMF). The model involves both turbulent fragmentation and competitive accretion. Turbulent fragmentation creates low-mass seed proto-systems (i.e. single and multiple protostars). Some of these low-mass seed proto-systems then grow by competitive accretion to produce the high-mass power-law tail of the SMF. Turbulent fragmentation is relatively inefficient, in the sense that the creation of low-mass seed proto-systems only consumes a fraction, ${\sim }23{{\ \rm per\ cent}}$ (at most ${\sim }50{{\ \rm per\ cent}}$), of the mass available for star formation. The remaining mass is consumed by competitive accretion. Provided the accretion rate on to a proto-system is approximately proportional to its mass (dm/dt ∝ m), the SMF develops a power-law tail at high masses with the Salpeter slope (∼−2.3). If the rate of supply of mass accelerates, the rate of proto-system formation also accelerates, as appears to be observed in many clusters. However, even if the rate of supply of mass decreases, or ceases and then resumes, the SMF evolves homologously, retaining the same overall shape, and the high-mass power-law tail simply extends to ever higher masses until the supply of gas runs out completely. The Chabrier SMF can be reproduced very accurately if the seed proto-systems have an approximately lognormal mass distribution with median mass ${\sim } 0.11 \, {\rm M}_{\odot }$ and logarithmic standard deviation $\sigma _{\log _{10}({M/M}_\odot)}\sim 0.47$).
Innovation and product market concentration: Schumpeter, arrow, and the inverted U-shape curve
Authors: Delbono Flavio , Lambertini Luca
Published: October 2020

We investigate the relationship between market concentration and industry innovative effort within a familiar two-stage model of R&D race in which firms compete à la Cournot in the product market. With the help of numerical simulations, we show that such a setting is rich enough to generate Arrovian, Schumpeterian, and inverted U curves. We interpret these different patterns on the basis of the relative strength of the technological incentive and the strategic incentive. We then bridge our theoretical results and some recent empirical research.
A Pathway for Community-Acquired Pneumonia With Rapid Conversion to Oral Therapy Improves Health Care Value
Authors: Ciarkowski Claire E , Timbrook Tristan T , Kukhareva Polina V , Edholm Karli M , Hatton Nathan D , Hopkins Christy L , Thomas Frank , Sanford Matthew N , Igumnova Elena , Benefield Russell J , Kawamoto Kensaku , Spivak Emily S
Published: October 2020

Background

Evidence supports streamlined approaches for inpatients with community-acquired pneumonia (CAP) including early transition to oral antibiotics and shorter therapy. Uptake of these approaches is variable, and the best approaches to local implementation of infection-specific guidelines are unknown. Our objective was to evaluate the impact of a clinical decision support (CDS) tool linked with a clinical pathway on CAP care.

Methods

This is a retrospective, observational pre–post intervention study of inpatients with pneumonia admitted to a single academic medical center. Interventions were introduced in 3 sequential 6-month phases; Phase 1: education alone; Phase 2: education and a CDS-driven CAP pathway coupled with active antimicrobial stewardship and provider feedback; and Phase 3: education and a CDS-driven CAP pathway without active stewardship. The 12 months preceding the intervention were used as a baseline. Primary outcomes were length of intravenous antibiotic therapy and total length of antibiotic therapy. Clinical, process, and cost outcomes were also measured.

Results

The study included 1021 visits. Phase 2 was associated with significantly lower length of intravenous and total antibiotic therapy, higher procalcitonin lab utilization, and a 20% cost reduction compared with baseline. Phase 3 was associated with significantly lower length of intravenous antibiotic therapy and higher procalcitonin lab utilization compared with baseline.

Conclusions

A CDS-driven CAP pathway supplemented by active antimicrobial stewardship review led to the most robust improvements in antibiotic use and decreased costs with similar clinical outcomes.
The STAPH Score: A Predictor ofStaphylococcus aureusas the Causative Microorganism of Native Vertebral Osteomyelitis
Authors: Matsuo Takahiro , Hayashi Kuniyoshi , Uehara Yuki , Mori Nobuyoshi
Published: October 2020

Background

Staphylococcus aureus (SA) is the most common causative microorganism in native vertebral osteomyelitis (NVO). Few studies have compared the clinical features of NVO due to SA (SA-NVO) and NVO due to other organisms (NSA-NVO). This study was conducted to validate a predictive score for SA-NVO to facilitate NVO treatment without broad-spectrum antimicrobial agents.

Methods

This retrospective study compared the clinical features of patients with SA-NVO and NSA-NVO who were diagnosed from 2004 to 2019. Univariate associations were assessed using χ 2, Fisher’s exact, or Mann-Whitney U test. Multivariable analysis was conducted using logistic regression. The optimal age cutoff point was determined by classification and regression tree analysis.

Results

Among 155 NVO patients, 98 (63.2%) had a microbiologically confirmed diagnosis: 40 (25.8%) with SA-NVO and 58 (37.4%) with NSA-NVO. Six predictors, either independently associated with SA-NVO or clinically relevant, were used to develop the STAPH prediction score: atopic dermatitis (Skin) (3 points); recent Trauma (2 points); Age < 67 years (1 point); Abscess (1 point); central venous Port catheter (2 points); and History of puncture (2 points). In a receiver operating characteristic analysis, the area under the curve was 0.84 (95% confidence interval, 0.76–0.91). The best cutoff point was 3. A score ≥3 had a sensitivity, specificity, positive predictive value, and negative predictive value of 58%, 84%, 84%, and 73%, respectively.

Conclusions

The STAPH score has relatively high specificity for use by clinicians to predict SA as the causative microorganism in patients with NVO until results of a confirmatory culture are available.
Operative Technique and Lessons Learned From Surgical Implantation of the NeuroPace Responsive Neurostimulation® System in 57 Consecutive Patients
Authors: Krucoff Max O , Wozny Thomas A , Lee Anthony T , Rao Vikram R , Chang Edward F
Published: October 2020

BACKGROUND

The Responsive Neurostimulation (RNS)® System (NeuroPace, Inc) is an implantable device designed to improve seizure control in patients with medically refractory focal epilepsy. Because it is relatively new, surgical pearls and operative techniques optimized from experience beyond a small case series have yet to be described.

OBJECTIVE

To provide a detailed description of our operative technique and surgical pearls learned from implantation of the RNS System in 57 patients at our institution. We describe our method for frame-based placement of amygdalo-hippocampal depth leads, open implantation of cortical strip leads, and open installation of the neurostimulator.

METHODS

We outline considerations for patient selection, preoperative planning, surgical positioning, incision planning, stereotactic depth lead implantation, cortical strip lead implantation, craniotomy for neurostimulator implantation, device testing, closure, and intraoperative imaging.

RESULTS

The median reduction in clinical seizure frequency was 60% (standard deviation 63.1) with 27% of patients achieving seizure freedom at last follow up (median 23.1 mo). No infections, intracerebral hemorrhages, or lead migrations were encountered. Two patients experienced lead fractures, and four lead exchanges have been performed.

CONCLUSION

The techniques set forth here will help with the safe and efficient implantation of these new devices.
Social Development and Police Reform: Some Reflections on the Concept and Purpose of Policing and the Implications for Reform in the UK and USA
Authors: Williams Andrew , Paterson Craig
Published: October 2020

The increase in calls for police reform following the death of George Floyd has led to renewed debate about social inequality and the role of policing in society. Modern bureaucratic police systems emerged from locally administered structures and Anglo-American policing models continue to be aligned, to varying degrees, with the political, socio-cultural, legal, and ideological aspects of contemporary liberal democratic society with its emphasis on democratic localism and decentralised accountability. However, at a time when society is reimagining itself and technology, government, and nations are radically re-shaping themselves, a critical question is whether there is a sufficiently common philosophical and conceptual understanding of policing to support its development rather than just a common understanding of police functions. This is profoundly important when considering the current calls for reform of policing in the USA and other western democratic states. The article argues that there is an urgent need to reconsider how we conceptualize policing and its relationship with social development.
Gender and Genre in Fanny Van de Grift Stevenson's Periodical Writing
Authors: Wånggren Lena
Published: October 2020

Despite scholarly recovery of many nineteenth-century women writers, especially those publishing mainly in periodicals, the fiction, travel writing and journalism of Fanny Van de Grift Stevenson (1840–1914) are still neglected. Situating Van de Grift Stevenson in the context of late nineteenth-century US periodical culture, this article considers how many of the author’s periodical writings cross not only geographical but also generic boundaries. Van de Grift Stevenson’s periodical publishing appears in a variety of modes and genres, at times with journalistic material entering her fiction, domestic life including recipes inserted in her journalism, and autobiography glimpsed in both fiction and journalism. The article examines this author’s transatlantic periodical writings in the context of gender and genre, noting the ways in which these categories intersect throughout her authorship. Focusing especially on the author’s regional writing as seen in her non-fiction ‘cookbook articles’ Ramblings of a Housewife and the short story ‘The Warlock’s Shadow’, the article argues that Van de Grift Stevenson’s blending of regional and domestic modes of writing presents a specific interrogation of cultural and geographical identities.
COSMIC RADIATION EXPOSURE CALCULATIONS FOR INTERNATIONAL AND DOMESTIC FLIGHTS DEPARTS FROM ISTANBUL AND ANKARA
Authors: Susam L Amon , Alan H Yilmaz , Yilmaz A , Erol A , Inci C I , Akinci F C , Akkus B , Demir M , Emirhan M E , Faydasicok O , Gudekli E
Published: October 2020

In this study, cosmic radiation doses were calculated for domestic and international flights departing from Istanbul and furthermore for domestic flights departing from Ankara using a software program called CARI-7A and also a mathematical method approach. Main parameters for calculating cosmic radiation are vertical cut-off rigidity, flight duration, latitude–longitude and altitude of the flight. Our calculation results agree with the measurements for domestic and international flights departing from Istanbul and Ankara.
The metendosternite and penile flagellum: two unexplored character systems of pleasing fungus beetles (Coleoptera: Erotylidae)
Authors: Pecci-Maddalena Italo Salvatore De Castro , Lopes-Andrade Cristiano , Skelley Paul
Published: October 2020

Erotylidae (Cucujoidea) are currently divided into six subfamilies, which are regarded as monophyletic. However, there are doubts on the monophyly of lower ranked taxa within Erotylidae, including its most diverse tribe (Tritomini) and the highly diverse genus Mycotretus (Tritomini). The next steps in phylogenetic studies on these taxa rely on better sampling and on studies of unexplored character sets. Here, we conduct a comparative morphological study of the metendosternite and the penile flagellum within Erotylidae, with emphasis on Tritomini and Mycotretus, establishing a naming system for these structures. Representatives of 56 species belonging to all subfamilies of Erotylidae were analysed. A total of 17 genera and 45 species of Tritomini were selected, of which 21 species were Mycotretus. A total of 17 characters (eight of the metendosternite and nine of the penile flagellum) with potential phylogenetic value were recognized. Within Tritomini there is evidence of phylogenetic signal for the presence or absence of the metendosternal lamina at the generic level and above. On the other hand, the penile flagellum may be more informative for levels below the genus and morphological features of the flagellar ‘head’ seem to have phylogenetic signal for groups of potentially related species of Mycotretus.
Advances in research of HIV transmission networks
Authors: Dong Zhi-Long , Gao George Fu , Lyu Fan
Published: October 2020

Transmission network analysis is a crucial evaluation tool aiming to explore the characteristics of the human immunodeficiency virus epidemic, develop evidence-based prevention strategies, and contribute to various areas of human immunodeficiency virus/acquired immunodeficiency syndrome prevention and control. Over recent decades, transmission networks have made tremendous strides in terms of modes, methods, applications, and various other aspects. Transmission network methods, including social, sexual, and molecular transmission networks, have played a pivotal role. Each transmission network research method has its advantages, as well as its limitations. In this study, we established a systematic review of these aforementioned transmission networks with respect to their definitions, applications, limitations, recent progress, and synthetic applications.
Out-of-hospital cardiac arrest: comparing organised groups to individual first responders
Authors: Nabecker Sabine , Theodorou Matthias , Huwendiek Sören , Kasper Nina , Greif Robert
Published: October 2020

BACKGROUND

Fast delivery of high-quality cardiopulmonary resuscitation is crucial in improving patient outcome after out of hospital cardiac arrest. First responders (trained laypersons) are dispatched to shorten time to basic life support and can be organised in groups or individually.

OBJECTIVE

A comparison of factors enabling or impairing first responders’ engagement in groups and as individuals are unknown. Therefore, we investigated these factors.

DESIGN

Qualitative comparison.

SETTING

We set up six focus groups from March to June 2017 in the Canton of Bern, Switzerland. Thirteen group and 13 individual first responders participated.

INTERVENTION

Interviews were audio-recorded, transcribed, coded and analysed following a thematic analytic approach. Two researchers coded the transcripts separately, identified, discussed and adjusted categories, themes and subthemes.

RESULTS

Factors supporting first responders’ engagement are: additional training, support from peers and society, satisfaction of personal desires (all important for all first responders), interdisciplinary collaboration (important for group first responders).

Factors impairing first responders’ engagement are: individual first responders lack training opportunities and collaboration, individual first responders lack support from peers and society, all first responders report lack of medical knowledge/skills and technical problems, confidentiality issues, legal insecurity and ethical concerns bother all first responders, intimidation by ‘professional first responders’ and professional burden. First responders organised in groups benefit from more training, enhanced peer support and collaboration with other groups. Individual first responders lack training opportunities and collaboration with emergency medical services.

CONCLUSION

Team spirit and peer-support engages group first responders, whereas individual first responders are impaired by lack of social support. Involvement with society triggers both first responder types to become and stay first responders. As first responders in groups have substantial additional benefits, enhancing such groups might strengthen current first responder systems.
Comparison of intravenous fentanyl versus lidocaine for attenuation of hemodynamic response to laryngoscopy and endotracheal intubation in patients undergoing elective surgery in Dilla University Referral Hospital, Ethiopia, a randomized controlled trial
Authors: Neme Doba Derartu , Ferede Zemedu Aweke , Mola Kasa Simeneh , Eyob Gebre Engida , Ayalew Nugusu , Abiy Workneh Sileshi , Jemal Abafita Bedru , Hailu Asgedom Sileshi , Mekonin Abate Semang , Regasa Degife Teshome
Published: October 2020

Background:

Laryngoscopy and endotracheal intubation is one of the most stressing moments in technique of general anesthesia. It can cause pressure response and sympato adrenal response due to stimulation of epipharynx and laryngopharynx. The main objective of this study is to compare the effect of intravenous lidocaine and fentanyl for attenuation of hemodynamic response during laryngoscopy and intubation in elective patients who undergo general anesthesia with endotracheal intubation.

Methods:

A randomized controlled trial was conducted in 52 eligible adults allocated in to 2 group; group F (n=26) patients received fentanyl 2 mcg/kg and group L (n=26) patients received lidocaine (2%) 1.5 mg/kg. The hemodynamic variables were recorded at 1st, 3rd, 5th, and 10th minutes after laryngoscopy and intubation. Kolmogorov test were used to test for distributions of data while homogeneity of variance were assessed using Levene test for equality of variance. Between groups comparison of vital sign were made using independent t test symmetric data and Mann-Whitney U test was used for asymmetric data. A post hoc effect size calculated using G-power. Those variables with P-value <0.05 were considered as statistically significant.

Result:

The mean rise in heart rate from base line was higher in lidocaine group 29.26±15.02 bpm compared with fentanyl group 17.56±10.28 bpm at first minute. The mean rise in systolic blood pressure from base line at first minute is lower in fentanyl group 17.53±13.04 compared with lidocaine group 31.53±17.24. The mean rise in hemodynamic variables (heart rate, systolic blood pressure, means arterial pressure, and diastolic blood pressure) from base line at 3rd, 5th, and 10th were comparable between groups with (P-value >0.05).

Conclusion and Recommendation:

Fentanyl at 2 µg/kg is superior in attenuation of hemodynamic response to laryngoscopy and intubation at first minute. There is comparable hemodynamic attenuation effect between groups at 3rd, 5th, and 10th minutes after intubation. Based on this we recommended that use of fentanyl 2 µg/kg better blunt hemodynamic response at first minute where both are equal at 3rd, 5th, and 10th minutes.
Noninvasive Ventilation Accelerates Oxygen Uptake Recovery Kinetics in Patients With Combined Heart Failure and Chronic Obstructive Pulmonary Disease
Authors: Mazzuco Adriana , Souza Aline Soares , Goulart Cássia da Luz , Medeiros Wladimir Musetti , Sperandio Priscila Abreu , Alencar Maria Clara Noman , Arbex Flávio Ferlin , Neder José Alberto , Arena Ross , Borghi-Silva Audrey
Published: October 2020

Purpose:

Oxygen uptake (V˙o2) recovery kinetics appears to have considerable value in the assessment of functional capacity in both heart failure (HF) and chronic obstructive pulmonary disease (COPD). Noninvasive positive pressure ventilation (NIPPV) may benefit cardiopulmonary interactions during exercise. However, assessment during the exercise recovery phase is unclear. The purpose of this investigation was to explore the effects of NIPPV on V˙o2, heart rate, and cardiac output recovery kinetics from high-intensity constant-load exercise (CLE) in patients with coexisting HF and COPD.

Methods:

Nineteen males (10 HF/9 age- and left ventricular ejection fraction-matched HF-COPD) underwent 2 high-intensity CLE tests at 80% of peak work rate to the limit of tolerance (Tlim), receiving either sham ventilation or NIPPV.

Results:

Despite greater V˙o2 recovery kinetics on sham, HF-COPD patients presented with a faster exponential time constant τ (76.4 ± 14.0 sec vs 62.8 ± 15.2 sec, P < .05) and mean response time (MRT) (86.1 ± 19.1 sec vs 68.8 ± 12.0 sec, P < .05) with NIPPV and greater ΔNIPPV-sham (τ: 5.6 ± 19.5 vs −25.2 ± 22.4, P < .05; MRT: 4.1 ± 32.2 vs −26.0 ± 19.2, P < .05) compared with HF. There was no difference regarding Tlim between sham and NIPPV in both groups (P < .05).

Conclusion:

Our results suggest that NIPPV accelerated the V˙o2 recovery kinetics following high-intensity CLE to a greater extent in patients with coexisting HF and COPD compared with HF alone. NIPPV should be considered when the objective is to apply high-intensity interval exercise training as an adjunct intervention during a cardiopulmonary rehabilitation program.
Markers of Right Ventricular Dysfunction Predict Maximal Exercise Capacity After Left Ventricular Assist Device Implantation
Authors: Bouzas-cruz Noelia , Koshy Aaron , Gonzalez-Fernandez Oscar , Ferrera Carlos , Green Thomas , Okwose Nduka C. , Woods Andrew , Tovey Sian , Robinson-smith Nicola , Mcdiarmid Adam K. , Parry Gareth , Gonzalez-juanatey Jose R. , Schueler Stephan , Jakovljevic Djordje G. , Macgowan Guy
Published: October 2020

Although left ventricular assist device (LVAD) improves functional capacity, on average LVAD patients are unable to achieve the aerobic capacity of normal healthy subjects or mild heart failure patients. The aim of this study was to examine if markers of right ventricular (RV) function influence maximal exercise capacity. This was a single-center prospective study that enrolled 20 consecutive HeartWare ventricular assist device patients who were admitted at the Freeman Hospital (Newcastle upon Tyne, United Kingdom) for a heart transplant assessment from August 2017 to October 2018. Mean peak oxygen consumption (Peak VO2) was 14.0 ± 5.0 ml/kg/min, and mean peak age and gender-adjusted percent predicted oxygen consumption (%VO2) was 40.0% ± 11.5%. Patients were subdivided into two groups based on the median peak VO2, so each group consisted of 10 patients (50%). Right-sided and pulmonary pressures were consistently higher in the group with poorer exercise tolerance. Patients with poor exercise tolerance (peak VO2 below the median) had higher right atrial pressures at rest (10.6 ± 6.4 vs. 4.3 mmHg ± 3.2; p = 0.02) and the increase with passive leg raising was significantly greater than those with preserved exercise tolerance (peak VO2 above the median). Patients with poor functional capacity also had greater RV dimensions (4.4 cm ± 0.5 vs. 3.7 cm ± 0.5; p = 0.02) and a higher incidence of significant tricuspid regurgitation (moderate or severe tricuspid regurgitation in five patients in the poor exercise capacity group vs. none in the preserved exercise capacity group; p = 0.03). In conclusion, echocardiographic and hemodynamic markers of RV dysfunction discriminate between preserved and nonpreserved exercise capacity in HeartWare ventricular assist device patients.
Comparison of Circulatory Unloading Techniques for Venoarterial Extracorporeal Membrane Oxygenation
Authors: Stephens Andrew F. , Wanigasekara Devindi , Pellegrino Vincent A. , Burrell Aidan J.C. , Marasco Silvana F. , Kaye David M. , Steinseifer Ulrich , Gregory Shaun D.
Published: October 2020

Left ventricular (LV) distention and pulmonary congestion are major complications inherent to venoarterial extracorporeal membrane oxygenation (ECMO). This study aimed to quantitatively compare the hemodynamic differences between common circulatory unloading methods for ECMO. Ten circulatory unloading techniques were evaluated on a mock circulatory loop simulating acute LV failure supported by ECMO. Simulated unloading techniques included: surgical and percutaneous pulmonary artery (PA) venting, surgical left atrial venting, surgical and percutaneous LV venting, atrial septal defect, partial support ventricular assist device, intraaortic balloon pump, and temporary VAD with inline oxygenator (tVAD). The most LV unloading occurred with the surgically placed LV vent and tVAD, which reduced LV end-diastolic volume from 295 to 167 ml and 82 ml, respectively. Meanwhile, the PA surgical vent was the most effective at reducing mean PA pressure from 21.0 to 10.6 mm Hg, and the tVAD was most effective at reducing left atrial pressure from 13.3 to 4.4 mm Hg. The major limitation of this study was the use of a mock circulatory loop, which simulated lower left atrial pressure than is typically seen clinically. This study identified clinically significant hemodynamic variability between the different circulatory unloading techniques evaluated. However, the applicability of these techniques will vary with different patient disease etiology. Further studies on ECMO unloading will help to quantify hemodynamic benefits and establish treatment guidelines.
Prevalence and Characteristics of Chronic Hepatitis C Among Asian Americans Are Distinct From Other Ethnic Groups
Authors: Tien Andy , Sahota Amandeep , Yang Su-Jau , Balbuena Ronald , Chang Mimi , Lim Carolina , Fong Tse-Ling
Published: October 2020

Goal:

The goal of this study was to determine the prevalence and characteristics of chronic hepatitis C (CHC) among Asian Americans compared with other ethnicities.

Background:

Chronic hepatitis C virus (HCV) affects an estimated 2.7 million in the United States, but there are limited data on HCV among Asian Americans.

Study:

A total of 3,369,881 adults over the age of 18 who were patients of the integrated health care system in Southern California and 4903 Asian participants at community hepatitis screenings were included in a cross-sectional study. Variables included HCV serology, HCV genotype, comorbidities, and coinfections.

Results:

The prevalence of CHC was 1.3% in the general population (8271 adults) and 0.6% among Asians. The prevalence of CHC was significantly higher in the 1945-1965 birth cohort with 2.7% (5876) in the general population and 1.0% (313) among Asians (P<0.001). Asians had the highest rates of hepatitis B coinfection (2.9% vs. 0.2%, P<0.001). The distribution of genotypes among Asians differed from the general population with the most common genotype being 1b (27.5%) and a higher presence of genotype 6 (9.5%) (P<0.001). The presence of cirrhosis was 17.6% in Asians. Disaggregated Asian data showed that CHC was highest among Vietnamese and Cambodian and that genotype 6 was predominant among these 2 subgroups.

Conclusions:

The prevalence of chronic HCV was significantly lower in Asians compared with other ethnicities. However, disaggregated data among Asians showed the highest prevalence rates among adults from Vietnam and Cambodia.
Cholestasis Due to USP53 Deficiency
Authors: Bull Laura N. , Ellmers Rebecca , Foskett Pierre , Strautnieks Sandra , Sambrotta Melissa , Czubkowski Piotr , Jankowska Irena , Wagner Bart , Deheragoda Maesha , Thompson Richard J.
Published: October 2020

Objectives:

Although a number of genetic forms of cholestasis have been identified, the genetic etiology of disease remains unidentified in a subset of cholestasis patients.

Methods:

Whole exome sequencing (WES) was performed in DNA from patients diagnosed with cholestasis, at different points on the continuum from progressive familial intrahepatic cholestasis to benign recurrent intrahepatic cholestasis, in whom no disease mutations in known cholestasis genes had been identified. Candidate genes were then assessed in a larger patient sample, by targeted next-generation sequencing (NGS). Disease features at presentation and follow-up were collected from available medical records.

Results:

By WES, we identified 3 patients with homozygous mutations in USP53. Screening of USP53 in a larger set of patients identified 4 additional patients with homozygous mutations in USP53. Six of the 7 patients had deletion mutations, and 1 had a missense mutation; 3 of the patients were siblings, all bearing a deletion that also disrupted neighboring MYOZ2. Age of onset ranged from early infancy to adolescence. Cholestasis tended to be biochemically mild and intermittent, and responsive to medication. Liver fibrosis was, however, present in all 4 patients who were biopsied, and splenomegaly was apparent in 5 of 7 at last ultrasound.

Conclusions:

Two groups recently identified patients with liver disease and mutation in USP53. We have now identified biallelic mutation in USP53 in 7 further patients with cholestasis, from 5 families. Most individuals had evidence of chronic liver disease, and long-term follow-up is recommended.
Feasibility, Acceptability, and Preliminary Effects of “Mindful Moms”
Authors: Kinser Patricia A. , Thacker Leroy R. , Rider Amy , Moyer Sara , Amstadter Ananda B. , Mazzeo Suzanne E. , Bodnar-Deren Susan , Starkweather Angela
Published: October 2020

Background

Nonpharmacological and accessible therapies that engage individuals in self-management are needed to address depressive symptoms in pregnancy. The 12-week “Mindful Moms” intervention was designed to empower pregnant women with depressive symptomatology to create personal goals and engage in mindful physical activity using prenatal yoga.

Objectives

This longitudinal pilot study evaluated the feasibility, acceptability, and preliminary effects of the “Mindful Moms” intervention in pregnant women with depressive symptoms.

Methods

We evaluated enrollment and retention data (feasibility) and conducted semistructured interviews (acceptability). We evaluated the intervention’s effects over time on participants’ depressive symptoms, anxiety, perceived stress, self-efficacy, and maternal–child attachment, and we compared findings to an archival comparison group, also assessed longitudinally.

Results

Enrollment and retention rates and positive feedback from participants support the intervention’s acceptability and feasibility. “Mindful Moms” participants experienced decreases in depressive symptoms, perceived stress, anxiety, ruminations, and maternal–child attachment and no change in physical activity self-efficacy from baseline to postintervention. Comparisons of the “Mindful Moms” intervention to the comparison groups over time indicated differences in depressive symptoms between all groups and a trend in differences in perceived stress.

Discussion

Results support the feasibility and acceptability of “Mindful Moms” for pregnant women with depressive symptoms and suggest that further research is warranted to evaluate this intervention for reducing depressive and related symptoms. Lack of a concurrent control group, with equivalent attention from study staff, and no randomization limit the generalizability of this study; yet, these preliminary findings support future large-scale randomized controlled trials to further evaluate this promising intervention.
Diagnosis and Management of Vulvovaginal Lichen Planus
Authors: Jacques Laura , Kornik Rachel , Bennett Daniel D. , Eschenbach David A.
Published: October 2020

Importance

Genital lichen planus is a debilitating disorder that lacks definitive recommendations regarding diagnosis and treatment.

Objective

The aim of this study was to present best practices from available evidence for the diagnosis and treatment of genital lichen planus.

Evidence Acquisition

We conducted a narrative review of the literature on genital lichen planus by searching PubMed using the following search terms: “vulvar lichen planus” OR (vulvar diseases[mesh] OR vulva[mesh]) AND lichen planus[mesh] OR vulvar[ti] AND “lichen planus”[ti]. We included all languages and years in the search.

Results

The search resulted in 273 citations that we reviewed for relevancy and selected 60 as the foundation for this review that focuses on genital sites. Diagnosis can be made without biopsy, and when a biopsy is taken, the pathologic findings may be nonspecific. Topical ultrapotent corticosteroids are most commonly used as first-line treatment of genital lichen planus.

Conclusions and Relevance

When patients present with genital lichen planus, a complete review of systems and a thorough physical examination should be performed because of the prevalence of extragenital sites. Treatment of genital disease should start with a topical, ultrapotent steroid, and follow-up visits should occur to ensure improvement and to monitor for adverse drug reactions and malignancy.

Target Audience

Obstetricians, gynecologists, and family physicians.

Learning Objectives

After reading this article, readers should be able to differentiate between lichen planus and other common vulvar dermatoses on physical examination; evaluate a patient for nongenital manifestations of lichen planus; assess if a vulvar biopsy is necessary for diagnosis; and construct a management plan for patients affected by genital lichen planus.
YAP1-FAM118B Fusion Defines a Rare Subset of Childhood and Young Adulthood Meningiomas
Authors: Schieffer Kathleen M. , Agarwal Vibhuti , LaHaye Stephanie , Miller Katherine E. , Koboldt Daniel C. , Lichtenberg Tara , Leraas Kristen , Brennan Patrick , Kelly Benjamin J. , Crist Erin , Rusin Jerome , Finlay Jonathan L. , Osorio Diana S. , Sribnick Eric A. , Leonard Jeffrey R. , Feldman Alexander , Orr Brent A. , Serrano Jonathan , Vasudevaraja Varshini , Snuderl Matija , White Peter , Magrini Vincent , Wilson Richard K. , Mardis Elaine R. , Boué Daniel R. , Cottrell Catherine E.
Published: October 2020

Meningiomas are a central nervous system tumor primarily afflicting adults, with <1% of cases diagnosed during childhood or adolescence. Somatic variation in NF2 may be found in ∼50% of meningiomas, with other genetic drivers (eg, SMO, AKT1, TRAF7) contributing to NF2 wild-type tumors. NF2 is an upstream negative regulator of YAP signaling and loss of the NF2 protein product, Merlin, results in YAP overexpression and target gene transcription. This mechanism of dysregulation is described in NF2-driven meningiomas, but further work is necessary to understand the NF2-independent mechanism of tumorigenesis. Amid our institutional patient-centric comprehensive molecular profiling study, we identified an individual with meningioma harboring a YAP1-FAM118B fusion, previously reported only in supratentorial ependymoma. The tumor histopathology was remarkable, characterized by prominent islands of calcifying fibrous nodules within an overall collagen-rich matrix. To gain insight into this finding, we subsequently evaluated the genetic landscape of 11 additional pediatric and adolescent/young adulthood meningioma patients within the Children’s Brain Tumor Tissue Consortium. A second individual harboring a YAP1-FAM118B gene fusion was identified within this database. Transcriptomic profiling suggested that YAP1-fusion meningiomas are biologically distinct from NF2-driven meningiomas. Similar to other meningiomas, however, YAP1-fusion meningiomas demonstrated overexpression of EGFR and MET. DNA methylation profiling further distinguished YAP1-fusion meningiomas from those observed in ependymomas. In summary, we expand the genetic spectrum of somatic alteration associated with NF2 wild-type meningioma to include the YAP1-FAM118B fusion and provide support for aberrant signaling pathways potentially targetable by therapeutic intervention.
Follicle Cysts of the Ovary: A Report of 30 Cases of a Common Benign Lesion Emphasizing its Unusual Clinical and Pathologic Aspects
Authors: Watkins Jaclyn C. , Young Robert H.
Published: October 2020

The common ovarian follicle cyst is typically straightforward from both clinical and pathologic perspectives, but may have a variety of unusual features from both aspects at various stages of life. Lack of familiarity with these may lead to diagnostic quandaries, the most common of which is distinguishing between a follicle cyst and cystic granulosa cell tumor of either adult or juvenile type. We reviewed 30 cases of follicle cysts, all sent in consultation, to highlight unusual aspects of a common lesion. Patients ranged from 3 d to 47 yr old. Clinical presentations included precocious puberty, pelvic pain, or an incidentally discovered pelvic mass, including those occurring in neonates and in 2 adults with pituitary adenomas, one of which was diagnosed 3 yr after presentation with the ovarian cyst. Size ranged from 0.5 cm (deflated) to 18.5 cm, with 7 exceeding 8 cm in greatest dimension. Twelve cases demonstrated small satellite cystic follicles in the wall of the dominant cyst. The granulosa cell layer varied in thickness and mitotic activity (which ranged from 1 to 36 per 10 HPF), but uniformly displayed round nuclei that lacked nuclear grooves. Luteinization of the granulosa cell layer, theca layer, or both was seen across all clinical scenarios, with unluteinized cysts being most common in precocious puberty patients. This series documents that although typically smaller, a subset of follicle cysts are the same size as cystic granulosa cell tumors and the 2 entities may be grossly indistinguishable. Helpful clues to the diagnosis of follicle cyst are the lack of nuclear grooves (vs. adult granulosa cell tumor) and lack of invagination of granulosa cells into the cyst wall (vs. both forms of granulosa cell tumor). Mitoses in the granulosa cells are of no aid in the differential with either form of granulosa cell tumor as follicle cysts may exhibit brisk mitotic activity. Our series highlights some of the unusual clinical aspects, one relatively well known—an association with isosexual precocity, but 2 not as widely known, those occurring in neonates and those due to a pituitary adenoma, the latter sometimes not being discovered until a few years after presentation with a follicle cyst.
Extraskeletal Myxoid Chondrosarcoma of the Vulva Confirmed by EWSR1 FISH: A Case Report and Review of the Literature
Authors: Salah Haneen T. , Stivers Nicole S. , Jarboe Elke A. , Dodson Mark K. , Ward Michael B.
Published: October 2020

Extraskeletal myxoid chondrosarcoma of the vulva is a very rare tumor, with less than 10 cases reported in the literature. We report a case of a 45-yr-old woman with extraskeletal myxoid chondrosarcoma of the vulva confirmed by EWSR1 fluorescence in situ hybridization. Given the unusual site and prominent myxoid morphology, a broad differential diagnosis and a variety of ancillary testing was required. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva, the differential diagnosis of a myxoid spindle cell neoplasm of the vulva, and the diagnostic importance of immunohistochemistry and EWSR1 fluorescence in situ hybridization.
Three-Dimensional Planning of the Mandibular Margin in Hemifacial Microsomia Using a Printed Patient-Specific Implant
Authors: Igelbrink Sebastian , Zanettini Leonardo Matos Santolim , Bohner Lauren , Kleinheinz Johannes , Jung Susanne
Published: October 2020

Hemifacial microsomia (HFM) is also known as malformation of the first and second branchial arches (BAs), oculoauriculovertebral dysplasia syndrome, and Goldenhar syndrome. Possibilities resulting from CAD/CAM may be a valuable tool to existing procedures to treat these abnormalities. The aim of this study was to report a case where the planning and implementation of an intraorally inserted 2-part patient-specific mandible implant was used for the treatment of HFM esthetic remained problem after orthognathic surgery. A 20-year-old female patient who continued to suffer from the esthetic defect of the unilateral hypoplastic mandible after completion of the orthognathic surgery attended for consultation. Using CT scan and the software Geomagic Freeform (3D Systems, USA), a 2-piece titanium implant was designed and printed to restore the osseous frame of the basal border of the mandible. The base was made of solid polished titanium to minimize soft tissue abrasion. Due to its split design, the implant could be placed anatomically exactly at the mandibular margin via an intraoral access and to avoid damage of the mental nerve. There were no postoperative complications such as infections, soft tissue reactions to the implant, sensitivity disorders, or dehiscence. The occlusion was regular. A measurement of the postoperative x-rays showed a clear increase in bony symmetry. No postoperative pain or trismus was seen. Functionally, the therapy ended with the completion of wound healing and the jaw was fully resilient again. A combined treatment using PSI and additional autogenous fat grafting may represent a valid treatment option for the treatment of facial asymmetry in patients with HFM.
Liver Resection Versus Local Ablation Therapies for Hepatocellular Carcinoma Within the Milan Criteria
Authors: Shin Seong Wook , Ahn Keun Soo , Kim Sang Woo , Kim Tae-Seok , Kim Yong Hoon , Kang Koo Jeong
Published: October 2020

Objective:

To compare the oncologic outcomes of liver resection (LR) and local ablation therapies for HCC.

Summary of Background Data:

Although several studies have compared LR and local ablation therapies, the optimal treatment of choice for HCC within the Milan criteria remains controversial.

Methods:

We systemically searched the MEDLINE, Embase, and Cochrane Library databases for randomized control trials (RCTs) and matched nonrandomized trials (NRTs) that compared LR and local ablation therapies for HCC within the Milan criteria. The primary outcome was overall survival (OS). Secondary outcomes were recurrence free survival (RFS) and recurrence pattern.

Results:

A total of 7 RCTs and 18 matched NRTs, involving 2865 patients in the LR group and 2764 patients in the local ablation therapy group [RFA, MWA, RFA plus trans-arterial chemoembolization (TACE)], were included. Although there was no significant difference in OS between LR and RFA, LR showed a significantly better 5-year RFS than RFA in the analysis of RCTs (hazards ratio: 0.75; 95% confidence interval: 0.62–0.92; P = 0.006). The RFA group showed a significantly higher local recurrence than the LR group in both analyses of RCTs and NRTs. Additionally, the LR group showed better OS and RFS than the MWA or RFA plus TACE groups.

Conclusion:

Our meta-analysis showed that LR was superior to RFA in terms of RFS and incidence of local recurrence. Moreover, LR showed better oncologic outcomes than MWA or RFA plus TACE.
Stereotactic Body Radiotherapy Versus Delayed Surgery for Early-stage Non-small-cell Lung Cancer
Authors: Mayne Nicholas R. , Lin Belle K. , Darling Alice J. , Raman Vignesh , Patel Deven C. , Liou Douglas Z. , D’Amico Thomas A. , Yang Chi-Fu Jeffrey
Published: October 2020

Objective:

To evaluate the overall survival of patients with operable stage IA non-small-cell lung cancer (NSCLC) who undergo “early” SBRT (within 0–30 days after diagnosis) versus “delayed” surgery (90–120 days after diagnosis).

Summary of Background Data:

During the COVID-19 pandemic, national guidelines have recommended patients with operable stage IA NSCLC to consider delaying surgery by at least 3 months or, alternatively, to undergo SBRT without delay. It is unknown which strategy is associated with better short- and long-term outcomes.

Methods:

Multivariable Cox proportional hazards modeling and propensity score-matched analysis was used to compare the overall survival of patients with stage IA NSCLC in the National Cancer Data Base from 2004 to 2015 who underwent “early” SBRT (0–30 days after diagnosis) versus that of patients who underwent “delayed” wedge resection (90–120 days after diagnosis).

Results:

During the study period, 570 (55%) patients underwent early SBRT and 475 (45%) underwent delayed wedge resection. In multivariable analysis, delayed resection was associated with improved survival [adjusted hazard ratio 0.61; (95% confidence interval (CI): 0.50–0.76)]. Propensity-score matching was used to create 2 groups of 279 patients each who received early SBRT or delayed resection that were well-matched with regard to baseline characteristics. The 5-year survival associated with delayed resection was 53% (95% CI: 45%–61%) which was better than the 5-year survival associated with early SBRT (31% [95% CI: 24%–37%]).

Conclusion:

In this national analysis, for patients with stage IA NSCLC, extended delay of surgery was associated with improved survival when compared to early treatment with SBRT.
Barriers to the Use of Active Surveillance for Thyroid Cancer Results of a Physician Survey
Authors: Hughes David T. , Reyes-Gastelum David , Ward Kevin C. , Hamilton Ann S. , Haymart Megan R.
Published: October 2020

Objective:

The aim of this study was to determine physician-reported use of and barriers to active surveillance for thyroid cancer.

Summary Background Data:

It is not clear whether active surveillance for thyroid cancer is widely used.

Methods:

Surgeons and endocrinologists identified by thyroid cancer patients from the Surveillance, Epidemiology, and End Results (SEER) registries of Georgia and Los Angeles County were surveyed between 2018 and 2019. Multivariable weighted logistic regression analyses were conducted to determine physician acceptance and use of active surveillance. Results: Of the 654 eligible physicians identified, 448 responded to the survey (69% response rate). The majority (76%) believed that active surveillance was an appropriate management option, but only 44% used it in their practice. Characteristics of physicians who stated that active surveillance was appropriate management, but did not report using it included more years in practice (reference group <10 years in practice): 10 to 19 years [odds ratio, OR 0.50 [95% confidence interval, CI 0.28–0.92]; 20 to 29 years [OR 0.31 (95% CI 0.15–0.62)]; >30 years [OR 0.30 (95% CI 0.15–0.61)] and higher patient volume 11 to 30 patients per year [OR 0.39 (95% CI 0.21 –0.70)] and >50 patients per year [OR 0.33 (95% CI 0.16–0.71)] compared to < 10, with no significant difference in those seeing 31 to 50 patients. Physicians reported multiple barriers to implementing active surveillance including patient does not want (80.3%), loss to follow-up concern (78.4%), more patient worry (57.6%), and malpractice lawsuit concern (50.9%).

Conclusion and Relevance:

Despite most physicians considering active surveillance to be appropriate management, more than half are not using it. Addressing existing barriers is key to improving uptake.