bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Spectrum of the Prader–Willi-like Pheno- and Genotype: A Review of the Literature
Authors: Juriaans Alicia F , Kerkhof Gerthe F , Hokken-Koelega Anita C S 
Published: August 2021

Abstract
Prader–Willi syndrome (PWS) is a rare genetic syndrome, caused by the loss of expression of the paternal chromosome 15q11-q13 region. Over the past years, many cases of patients with characteristics similar to PWS, but without a typical genetic aberration of the 15q11-q13 region, have been described. These patients are often labelled as Prader–Willi-like (PWL). PWL is an as-yet poorly defined syndrome, potentially affecting a significant number of children and adults. In the current clinical practice, patients labelled as PWL are mostly left without treatment options. Considering the similarities with PWS, children with PWL might benefit from the same care and treatment as children with PWS. This review gives more insight into the pheno- and genotype of PWL and includes 86 papers, containing 368 cases of patients with a PWL phenotype. We describe mutations and aberrations for consideration when suspicion of PWS remains after negative testing. The most common genetic diagnoses were Temple syndrome (formerly known as maternal uniparental disomy 14), Schaaf–Yang syndrome (truncating mutation in the MAGEL2 gene), 1p36 deletion, 2p deletion, 6q deletion, 6q duplication, 15q deletion, 15q duplication, 19p deletion, fragile X syndrome, and Xq duplication. We found that the most prevalent symptoms in the entire group were developmental delay/intellectual disability (76%), speech problems (64%), overweight/obesity (57%), hypotonia (56%), and psychobehavioral problems (53%). In addition, we propose a diagnostic approach to patients with a PWL phenotype for (pediatric) endocrinologists. PWL comprises a complex and diverse group of patients, which calls for multidisciplinary care with an individualized approach.
A visita da Comissão Interamericana de Direitos Humanos na Argentina durante a ditadura civil-militar (1976-1983) | The Inter-American Commission on Human Rights’ on-site visit in Argentina during the civil-military dictatorship (1976-1983)
Authors: Dauer Gabriel Roberto 
Published: August 2021

As violações de direitos humanos na ditadura civil-militar argentina (1976-1983) foram tema de foros multilaterais, sendo um deles a Comissão Interamericana de Direitos Humanos (CIDH) da Organização dos Estados Americanos. Nesse contexto, este trabalho analisa como a ditadura respondeu às críticas aos direitos humanos, particularmente quando da visita in loco da CIDH na Argentina em 1979 até a publicação de seu informe em 1980. Utilizamos da Análise de Política Externa para compreender as tomadas de decisão da ditadura para receber a CIDH, os atores envolvidos e as consequências nacionais e internacionais desse evento. A visita transformou o campo de oportunidades de denúncia e visibilidade de opositores ao expor as atrocidades da ditadura. Contudo, as decisões do regime não foram lineares: os militares não eram os únicos interessados em defender seus interesses; grupos de direitos humanos, exilados e organizações internacionais disputaram esse campo, somadas desavenças internas na Junta Militar que dificultaram uma congruência diplomática.Palavras-chave: Argentina; Ditadura; Comissão Interamericana de Direitos Humanos.ABSTRACT:Human rights violations during the argentine civil-military dictatorship (1976-1983) were a theme on multilateral forums, such as the Inter-American Commission on Human Rights (IACHR) of the Organization of American States (OAS). The article analyzes how the dictatorship reacted to criticisms regarding human rights violations in Argentina, especially during the IACHR's on-site visit in Argentina in 1979 until the publication of its report in 1980. Theoretically, Foreign Policy Analysis concepts were articulated to understand the decision-making of the dictatorship to receive the IACHR, the actors involved, and the national and international consequences of the event. IACHR's visit transformed the field of human rights actors’ opportunities of complaints and the visibility of opponents by exposing the atrocities of the dictatorship, whose decisions were not linear: the military was not the only interested actor in defending its interests; human rights groups, exiles, and international organizations also disputed this narrative, while internal disputes in the Military Junta made Argentina's diplomatic congruence difficult.Keywords: Argentina; Dictatorship; Inter-American Commission on Human Rights. Recebido em: 31 mar. 2021 | Aceito em: 23 jun. 2021.
On-road driving behaviour characteristics of patients with brain injury
Authors: Tanaka Hajime , Ito Emi , Yoshihara Ayami 
Published: August 2021

Background/aims To clarify the characteristics of automobile driving performance in patients with brain injury, 26 patients (patient group) and 26 driving instructors (control group) were evaluated via an on-road driving assessment. Methods On-road driving performance was evaluated using an event data recorder, driving analysis software, and a video-based driving assessment scale. The number of unsafe driving events, the score on the driving analysis software, and the score on the driving assessment scale were then compared between the two groups. Results The patient group had 12 unsafe events (7 braking, 4 acceleration, 1 curving), but the instructors only had two sudden braking events, both of which were to avoid a collision when another vehicle forcibly entered from outside the roadway. In the case of the driving analysis software, the patient group showed significantly lower scores for braking (P<0.05) and steering (P<0.05) than the control group. On the driving assessment scale, the patient group scored significantly lower for checking their blind spot during a lane change (P<0.01) and being aware of pedestrians at an intersection (P<0.01) than the control group. Conclusions These objective video and g-force-generated on-road driving assessments may provide important information regarding driving and advice for patients with brain injury.
Neurological complications in COVID-19: a single case study of rehabilitation treatment
Authors: Casertano Lorenzo , Nathanson Rae , Bassile Clare C , Quinn Lori 
Published: August 2021

Background/aims: COVID-19 is a global pandemic, which has seen over 198 million cases as of August 2021. This case study highlights the rehabilitation of a young patient with respiratory and neurologic sequalae of COVID-19 across the continuum of care, from the intensive care unit to the inpatient rehabilitation unit. Case description: A 45-year-old woman, with past medical history of fibromyalgia and morbid obesity, presented with complaints of shortness of breath. She tested positive for SARS-CoV-2, was transferred to the intensive care unit, and was intubated for 17 days. The day after extubation, she experienced worsened mental status; computed tomography and magnetic resonance imaging scans revealed bilateral strokes. On hospital day 21, she was transferred to the stroke step-down unit. On hospital day 24, she recovered some cognitive ability and movement of her lower extremities. On hospital day 30, she was admitted to the inpatient rehabilitation unit. Examination by occupational and physiotherapists found motor and sensory impairments of multiple peripheral nerves, including musculocutaneous, axillary and radial nerves. Interventions included passive range of motion, sitting balance, transfer training, rigid taping, upper extremity strengthening and functional training (gait, stair, activities of daily living). Her activities of daily living performance was limited by upper extremity weakness, sensory loss and pain. Conclusions This case highlights the medical, neurological and functional implications of COVID-19 on patients after prolonged hospitalisation. The plan of care was informed by collaboration between rehabilitation disciplines. Causes of her injuries are unclear but could include positioning, brachial plexus injuries, or post-critical illness syndrome. Further research on the evaluation and care of patients with COVID-19 that result in profound neurological impairments is warranted.
Optical properties of anatase and rutile TiO2 films deposited by using a pulsed laser
Authors: Cui Liqi , Wang Weitian 
Published: August 2021

Transparent titanium dioxide (

T
i
O

2

) films were deposited on quartz substrates by using a pulsed-laser deposition technique. The effects of deposition temperatures on the crystalline phases and optical properties of the films were investigated. Phase-pure anatase and rutile

T
i
O

2

films were obtained at temperatures of 600°C and 800°C, respectively. The deposited

T
i
O

2

films were transparent in the visible spectral region, and the linear refractive indices of the films were determined by using the optical transmission spectra. The optical bandgaps were obtained as 3.25 and 3.02 eV for the anatase and rutile

T
i
O

2

films, respectively. The third-order nonlinear optical properties of the films were investigated by using a single-beam

z

-scan method at a wavelength of 532 nm. The values of the nonlinear absorption coefficient and the nonlinear refractive index of the rutile

T
i
O

2

film were determined to be

−

7.44

×

10

−
10

m

/

W

and

−

1.36

×

10

−
16

m

2

/

W

, respectively, which are greater than those of the anatase

T
i
O

2

film. The metal–oxygen bond lengths based on the bond-orbital theory could be used to explain the results.
Tuning the luminous properties and optical thermometry of Cs2SnCl6 phosphor microcrystals via Bi and Sb codoping
Authors: Gong Shunfa , Wu Ruirui , Yang Sen , Wu Lifang , Zhang Minmin , Han Qiuju , Wu Wenzhi 
Published: August 2021

The toxicity and instability of lead halide perovskite seriously limit its commercial application in lighting, although it has high photoluminescence (PL) efficiency and adjustable emission. Here, lead-free bismuth (Bi) and antimony (Sb) codoped

Cs

2

SnCl

6

(BSCSC) microcrystals (MCs) are prepared successfully by a solvothermal method. The PL spectrum is composed of dual emission bands with the peak at 485 and 650 nm, of which relative intensity can be tunable through the change of Bi and Sb feeding contents, respectively. Because of the phonon–electron interaction, the PL intensity is enhanced as the temperature rises within the range of 80–260 K. Then, the nonradiative transition is intensified until 380 K, which results in decrease in PL intensity. Simultaneously, combining with time-resolved PL, it is concluded that the emission peak at 485 nm is attributed to the [

Bi

Sn

+

V

Cl

] as the luminescent centers with the lifetime of hundreds of nanoseconds, and the emission peak at 650 nm is attributed to microsecond-timescale self-trapped excitons. The maximum values of relative sensitivity (

S
R

) and absolute sensitivity (

S
A

) values obtained are

3.82
%

K

−
1

and

5.11

ns

·

K

−
1

, which for the first time to our knowledge demonstrate that BSCSC MCs can be novel luminescent materials for developing better optical thermometry. White-light-emitting diodes (WLEDs) are constructed using BSCSC MCs only combined with an LED chip, the Commission Internationale de L’Eclairage color coordinates of which are (0.30, 0.37). It provides a novel scheme for the lighting field to realize WLEDs without adding additional commercial phosphors.
The spread of cat-transmitted sporotrichosis due to Sporothrix brasiliensis in Brazil towards the Northeast region
Authors: de Oliveira Bento Aurélio , de Sena Costa Alexandre Soares , Lima Soraia Lopes , do Monte Alves Manoella , de Azevedo Melo Analy Salles , Rodrigues Anderson Messias , da Silva-Rocha Walicyranison Plinio , Milan Eveline Pipolo , Chaves Guilherme Maranhão 
Published: August 2021

Background
Sporotrichosis is a worldwide subcutaneous mycosis caused by Sporothrix spp. In the past, this infection was associated with armadillo hunting, horticulturists, miners, and gardeners, being considered an implantation mycosis acquired by plant debris injury. Nevertheless, since the late nineties, it has been considered a zoonotic disease in Brazil. Here we report a case series of 121 patients with cat-transmitted sporotrichosis seen in Northeast Brazil.

Methodology/Principal findings
Patient’s demographic, clinical data, and length of treatment were recorded. In addition, a mycological examination and further PCR confirmation of species identification were performed. One hundred and twenty two patients were diagnosed with subcutaneous sporotrichosis from October 2016 to December 2019, while PCR revealed that 71 of them were due to S. brasiliensis. The majority of the individuals were female (n = 86; 70.5%). Patient’s age ranged from 5 to 87 years old. The clinical forms found were lymphocutaneous (58.2%) and fixed cutaneous (39.4%). Interestingly, 115 patients reported previous contact with cats diagnosed with sporotrichosis. Patients were successfully treated with itraconazole and potassium iodide.

Conclusions/Significance
Our study adds important contributions for the investigation of the spread of cat-transmitted subcutaneous sporotrichosis in Brazil, specifically towards the Northeast region of a continental-size country. It will also help clinicians to be aware of the existence and importance to accurately diagnose sporotrichosis and treat patients with this infectious disease in the lowest income region of Brazil.
“Assessing Today for a Better Tomorrow”: An observational cohort study about quality of care, mortality and morbidity among newborn infants admitted to neonatal intensive care in Guinea
Authors: Merscher Alves Maria Bea , Conté N’Fanly , Diallo Boubacar , Mamadou Moustapha , Delamou Albert , John Oliver , von Felten Stefanie , Diallo Ibrahima Sory , Roth-Kleiner Matthias 
Published: August 2021

Background
Neonatal mortality in Guinea accounts for about 30% of all fatalities in children younger than five years. Countrywide, specialized neonatal intensive care is provided in one single clinic with markedly limited resources. To implement targeted measures, prospective data on patient characteristics and factors of neonatal death are needed.

Objective
To determine the rates of morbidity and mortality, to describe clinical characteristics of admitted newborns requiring intensive care, to assess the quality of disease management, and to identify factors contributing to neonatal mortality.

Methods
Prospective observational cohort study of newborns admitted to the hospital between mid-February and mid-March 2019 after birth in other institutions. Data were collected on maternal/prenatal history, delivery, and in-hospital care via convenience sampling. Associations of patient characteristics with in-hospital death were assessed using cause-specific Cox proportional-hazards models.

Results
Half of the 168 admitted newborns underwent postnatal cardiopulmonary resuscitation. Reasons for admission included respiratory distress (49.4%), poor postnatal adaptation (45.8%), prematurity (46.2%), and infections (37.1%). 101 newborns (61.2%) arrived in serious/critical general condition; 90 children (53.9%) showed clinical signs of neurological damage. Quality of care was poor: Only 59.4% of the 64 newborns admitted with hypothermia were externally heated; likewise, 57.1% of 45 jaundiced infants did not receive phototherapy. Death occurred in 56 children (33.3%) due to birth asphyxia (42.9%), prematurity (33.9%), and sepsis (12.5%). Newborns in serious/critical general condition at admission had about a fivefold higher hazard to die than those admitted in good condition (HR 5.21 95%-CI 2.42–11.25, p = <0.0001). Hypothermia at admission was also associated with a higher hazard of death (HR 2.00, 95%-CI 1.10–3.65, p = 0.023).

Conclusion
Neonatal mortality was strikingly high. Birth asphyxia, prematurity, and infection accounted for 89.3% of death, aggravated by poor quality of in-hospital care. Children with serious general condition at admission had poor chances of survival. The whole concept of perinatal care in Guinea requires reconsideration.
Prevalence of burnout among university students in low- and middle-income countries: A systematic review and meta-analysis
Authors: Kaggwa Mark Mohan , Kajjimu Jonathan , Sserunkuma Jonathan , Najjuka Sarah Maria , Atim Letizia Maria , Olum Ronald , Tagg Andrew , Bongomin Felix 
Published: August 2021

BackgroundBurnout is common among university students and may adversely affect academic performance. Little is known about the true burden of this preventable malady among university students in low-and-middle-income countries (LMICs).ObjectivesThis study aimed to systematically estimate the prevalence of burnout among university students in LMICs.MethodsWe searched PubMed, Google Scholar, CINAHL, Web of Science, African Journals Online, and Embase from the inception of each database until February 2021. Original studies were included. No study design or language restrictions were applied. A random-effects meta-analysis was performed using STATA version 16.0. Heterogeneity and publication bias were assessed using Q-statistics and funnel plots, respectively.ResultsFifty-five unique articles, including a total of 27,940 (Female: 16,215, 58.0%) university students from 24 LMICs were included. The Maslach Burnout Inventory (MBI) was used in 43 studies (78.2%). The pooled prevalence of burnout was 12.1% (95% Confidence Interval (CI) 11.9–12.3; I2= 99.7%, Q = 21,464.1,p= < 0.001). The pooled prevalence of emotional exhaustion (feelings of energy depletion), cynicism (negativism), and reduced professional efficacy were, 27.8% (95% CI 27.4–28.3; I2= 98.17%.p= <0.001), 32.6 (95% CI: 32.0–33.1; I2: 99.5%;p= < 0.001), and 29.9% (95% CI: 28.8–30.9; I2: 98.1%;p= < 0.001), respectively.ConclusionNearly one-third of university students in LMICs experience burnout. More studies are needed to understand the causes of burnout in this key population. There is a need to validate freely available tools for use in these countries.
Documents of Doubt: The Photographic Conditions of Conceptual Art, Heather Diack (2020)
Authors: Watson Thomas 
Published: August 2021

Review of: Documents of Doubt: The Photographic Conditions of Conceptual Art, Heather Diack (2020)
Minneapolis, MN: University of Minnesota Press, 296 pp.,
ISBN 978-1-51790-757-0, p/bk, $30.00
Numerical modeling of electromagnetic scattering from complex shape object with coating
Authors: Legenkiy Maxim , Khrychov Vlad 
Published: August 2021

Abstract
Designing and creating invisible samples of military equipment is important modern science and technical problem. Physically, this is equivalent to reducing the object RCS. It requires fast and accurate tools (experimental or theoretical ones) to determine the RCS of different objects to which various means of reducing visibility are applied. The experimental studies require a lot of time and money, also it provides incomplete information about the object. So, it is important to create numerical effective and physically justified method for the theoretical determining of the complex shape objects RCS. There are well known numerical methods such as SBR. However, in this method there is no suitable approach for effective coating calculating, in particular such coating as a tent. The main goal of this paper is proposing a new method for taking into account some coating that is used for the object masking. To improve the accuracy of such modeling, it is necessary to use a large number of facets. Thus, recalculation of the set of parameters for the coating becomes also a difficult and time-consuming task. Therefore, it is important to develop a method for quickly assessing the effect of the certain coating parameters on the object RCS.
Quantum Entanglement Undermines Structural Realism
Authors: Park Seungbae 
Published: August 2021

Abstract
Quantum entanglement poses a challenge to the traditional metaphysical view that an extrinsic property of an object is determined by its intrinsic properties. So structural realists might be tempted to cite quantum entanglement as evidence for structural realism. I argue, however, that quantum entanglement undermines structural realism. If we classify two entangled electrons as a single system, we can say that their spin properties are intrinsic properties of the system, and that we can have knowledge about these intrinsic properties. Specifically, we can know that the parts of the system are entangled and spatially separated from each other. In addition, the concept of supervenience neither illuminates quantum entanglement nor helps structural realism.
The crystal structure of (E)-N′-(butan-2-ylidene)isonicotinohydrazide 0.5 hydrate C10H13N3O·0.5H2O
Authors: Setshedi Itumeleng B. , Lemmerer Andreas , Smith Mark G. 
Published: August 2021

Abstract
C10H13N3O·0.5H2O, orthorhombic, Aba2 (no. 41), a = 19.9676(19) Å, b = 8.4674(7) Å, c = 12.4082(10) Å, V = 2097.9(3) Å3, Z = 8, R

gt
(F) = 0.0299, wR

ref
(F
2) = 0.0849, T = 173 K.

CCDC no.: 2086756
Neuroplasticity mediated by motor rehabilitation in Parkinson’s disease: a systematic review on structural and functional MRI markers
Authors: Baglio Francesca , Pirastru Alice , Bergsland Niels , Cazzoli Marta , Tavazzi Eleonora 
Published: August 2021

Abstract
Parkinson’s disease (PD) is the second most common neurological disease affecting the elderly population. Pharmacological and surgical interventions usually employed for PD treatment show transient effectiveness and are associated with the insurgence of side effects. Therefore, motor rehabilitation has been proposed as a promising supplement in the treatment of PD, reducing the global burden of the disease and improving patients quality of life. The present systematic review aimed to critically analyse the literature concerning MRI markers of brain functional and structural response to motor rehabilitation in PD. Fourteen out of 1313 studies were selected according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria. Despite the limited number of retrieved studies coupled with their heterogeneity prevent ultimate conclusions from being drawn, motor rehabilitation seems to have beneficial effects on PD as measured both with clinical outcomes and MRI derived indices. Interestingly, consistent results seem to indicate that motor rehabilitation acts via a dual mechanism of strengthening cortico-subcortical pathways, restoring movements automaticity, or activating compensatory networks such as the fronto-parietal one. The employment of more advanced and quantitative MRI methods is warranted to establish and validate standardized metrics capable of reliably determining the changes induced by rehabilitative intervention.