bluuebunny/crossref_metadata_2025_split huggingface dataset:
Uncombable hair improved by biotin
Authors: Drivenes Jakob L. , Grimalt Ramon , Betz Regina C. , Bygum Anette 
Published: February 2023

AbstractUncombable hair syndrome (UHS) is a rare disorder of hair shaft morphology. UHS presents in early childhood with blonde, dry and frizzy scalp hair. The rate of hair growth may be normal or slow. The UHS hair shaft is characterized by a triangular shape with longitudinal grooves along the hair shaft axis. At writing, no curative treatment is available for UHS. However, research suggests that oral biotin supplementation results in a clinical improvement in growth rate and texture in some cases. Here, we present the case of a girl with UHS who was successfully treated with oral biotin supplementation. Our case highlights the diagnostic pathway in UHS, and demonstrates that two cycles of treatment with biotin, each separated by a period of 3 years, led to a significant improvement in combability and growth rate in a patient with UHS. We propose that biotin is a safe treatment option for UHS when both the health care provider and the family are aware of the possible interference with laboratory test results.
Simultaneous Manipulation of Line‐Gap and Point‐Gap Topologies in Non‐Hermitian Lattices
Authors: Liu Dongjue , Wang Zihao , Cheng Zheyu , Hu Hao , Wang Qijie , Xue Haoran , Zhang Baile , Luo Yu 
Published: February 2023

AbstractNon‐Hermiticity may significantly influence the topology of a photonic/electronic lattice, giving rise to point gaps, which have attracted much attention in recent years. While the influences of the point‐gap topology on the bulk‐boundary correspondence for the line gap have been widely studied, the topological lattice that simultaneously processes the line‐gap and point‐gap topological transitions has not been reported. Here, a strategy to simultaneously manipulate the line‐gap and point‐gap topologies in non‐Hermitian lattices is proposed. By introducing the asymmetric intercell coupling, the line‐gap topological transition process is demonstrated. By further considering the nonreciprocal coupling between the nearest neighboring unit cells, the point‐gap and line‐gap topological transitions can be simultaneously realized. Finally, the influence of the next nearest coupling on non‐Hermitian line‐gap and point‐gap topologies is also discussed.
Pneumatically Tunable Droplet Microlaser
Authors: Yamagishi Hiroshi , Fujita Keitaro , Miyagawa Junnosuke , Mikami Yuya , Yoshioka Hiroaki , Oki Yuji , Takada Naoki , Baba Soumei , Saito Shimpei , Someya Satoshi , Lin Zhan‐Hong , Huang Jer‐Shing , Yamamoto Yohei 
Published: February 2023

AbstractAn ideally flexible laser may function in unison with minute fluctuations in nature. Lasers made solely from liquids are promising toward this end, but they are intrinsically unstable and have been inapplicable to steady operation under ambient conditions unless they are enclosed in a tailored container or a matrix to prevent the evaporation of the liquid. Here, a simple methodology is reported to form a self‐standing spherical microlaser that is composed fully of liquid and operates steadily even under atmosphere. The robustness and spherical morphology of the droplets are achieved by using ionic liquid as the liquid medium and gently casting the droplets on a substrate covered with hydrophobic nanoparticles to enhance the metastability of the contact angle. The resulting droplets are highly robust and work as efficient long‐lasting laser oscillators. The lasing wavelength is sensitively shifted when the droplets are subjected to a faint breeze or moisture, which is associated with the deformation of the droplet. The morphological and optical responses of the droplet under gas convection are consistently supported by aerodynamic and electromagnetic simulations. The droplets are readily scalable with an inkjet printer without the need for any further treatments.
A smooth ambiguity model of investment timing under asymmetric information
Authors: Pong Wong Kit 
Published: February 2023

AbstractThis paper incorporates ambiguity into a principal–agent framework wherein an owner delegates the investment decision of a project in continuous time to a manager. Information is asymmetric in that the owner faces ambiguity regarding the uncertain investment cost of the project (low or high) but the manager privately observes the true value. Ambiguity aversion is shown to induce the owner to act in a pessimistic manner with a perception that the probability of having the low investment cost is smaller than what the objective beliefs suggest, resulting in a distortion on the equilibrium tradeoff between investment efficiency and information rent.
A novel heterozygous ATP1A2 pathogenic variant in a Chinese child with MELAS‐like alternating hemiplegia
Authors: Zhang Xin , Qiu Shiyan , Yang Li , Li Yufen , Xu Liyun , Xu Na , Mi Changrui , Li Menglin 
Published: February 2023

AbstractBackgroundPathogenic variants of ATP1A2 (OMIM ID: 182340) are usually associated with familial hemiplegic migraine type 2 (FHM‐2), alternating hemiplegia of childhood (AHC), early infantile epileptic encephalopathy (EIEE), transient cytotoxic edema, and so on. Here, we present a novel heterozygous ATP1A2 variant in a girl with alternating hemiplegia, febrile seizures, developmental delay (which subsequently subsided), and MELAS‐like syndrome (as indicated by brain MRI). The patient did not experience migraine with aura.MethodsThe patient was an 8‐year‐old girl with normal growth and development. Beginning from the age of 3 years and 8 months, the patient experienced several episodes of alternating limb paralysis. The episodes were accompanied by the appearance of MELAS‐like findings on brain MRI, which corresponded to the hemiplegia. There were abnormal linear signals in the cerebral cortex on the opposite side of the hemiplegic limb. Each time the patient recovered from hemiplegia, and each time MRI showed no lesions remained after recovery. No obvious abnormality was found in other examinations. Finally, the patient underwent whole‐exome sequencing (WES).ResultsWES revealed a novel and de novo heterozygous variant in the ATP1A2 (NM_000702.3) c.335C>A:p.Ala112Asp (not previously reported). We examined the variant position in the 3D protein structure and found that a missense mutation at this site is a nonconservative substitution. The variation is nonpolymorphic. It occurs at a very low frequency in the population, and its ACMG classification is likely pathogenic.ConclusionAt present, there are limited reports of mutations in the ATP1A2 gene causing AHC. This is the first case of brain MRI showing MELAS‐like imaging in an AHC patient, and more cases are needed for verification. Early genetic testing and family screening can aid in the diagnosis and treatment of genetic diseases. The relationship between ATP1A2 gene mutation genotype and clinical phenotype needs to be further studied.
Postoperative Radiation Therapy Refusal in Major Salivary Gland Cancers
Authors: Shah Keshav V. , Carey Ryan M. , Prasad Aman , Panara Kush , Rajasekaran Karthik , Cannady Steven B. , Brant Jason A. , Brody Robert M. 
Published: February 2023

AbstractObjectiveMajor salivary gland cancers (MSGCs) are often treated with primary surgery followed by adjuvant therapy for high‐risk pathology. Patients with these cancers may opt out of recommended postoperative radiation therapy (PORT) for many reasons and consequently may suffer worse outcomes.Study DesignRetrospective cohort study.SettingNational Cancer Database.MethodsPatients diagnosed with MSGC from 2004 to 2016 were identified, and overall survival and risk factors for refusal of recommended PORT were analyzed based on demographic, socioeconomic, and clinical factors. Multivariable logistic regression and a Cox model were used to conduct the analysis.Results211 out of 4704 qualifying patients (4.5%) refused recommended PORT. Multivariable analysis demonstrated increased PORT refusal for age >74 years (odds ratio OR 4.34, confidence interval [CI] [2.43‐7.85]), Asian race (OR 2.25, CI [1.10‐4.23]), and certain facility types (comprehensive cancer center, OR 2.39, CI [1.08‐6.34]; academic research program, OR 3.29, CI [1.49‐8.74]; and integrated network cancer program, OR 2.75, CI [1.14‐7.7]). N2 stage was associated with decreased PORT refusal (OR 0.67, CI [0.45‐0.98]). The 5‐year overall survival for patients who received and refused PORT were significantly different at 65.8% and 53.8%, respectively (p < .001). When controlling for several factors, PORT refusal was independently associated with significantly lower overall survival (HR 1.54, CI [1.21‐1.98]).ConclusionPatient refusal of recommended PORT in MSGC is rare, associated with various disease and socioeconomic factors, and may decrease overall survival. Our findings can assist clinicians in counseling patients and identifying those who may be more likely to opt out of recommended PORT.
Detecting spatial heterogeneity in the determinants of intercity migration in China
Authors: Li Sen , Gu Hengyu , Shen Jianfa 
Published: February 2023

AbstractSpatial variations exist in migration patterns and the processes causing the observed patterns. Yet, far less attention has been given to the latter. Even if a few studies have engaged in spatial heterogeneity in the process of migration, the scale multiplicity is not emphasised. This research aims to address these gaps with evidence from Chinese cities. To facilitate international comparisons, we use data on intercity migration over a 5‐year interval instead of the floating population. Following an elaboration of highly imbalanced migration patterns at the city scale, we detect spatial heterogeneity in the processes underlying these patterns based on 17 potential determinants in four domains (namely labour market conditions, site‐specific amenities, agglomerative effects, and institutional dividends) by multiscale geographically weighted regression. The results indicate that cities' attractiveness to migrants depends on a broad spectrum of factors whose influences are location‐dependent and exhibit specific patterns, including east–west, south–north, and southwest–northeast gradient patterns, as well as discrete clustered and concentric patterns. The diagnostic analysis has confirmed that the provenance of spatial‐varying effects is irrelevant to the nonlinearity. These effects are also scale‐sensitive, showing that the influencing scales of the labour market status and agglomeration factors tend to be larger (i.e., their effects are spatially stationary) than those relevant to amenities and policies, which vary considerably (i.e., the impacts of some factors change significantly over space). These findings regarding spatial heterogeneity deepen our understanding of migration in China and highlight the fact that there is no ‘one size fits all’ approach for government policies designed to attract migrants.
Plural Policing in the Pacific
Authors: Watson Danielle , Howes Loene , Dinnen Sinclair , Bull Melissa , Amin Sara N. 
Published: February 2023

AbstractPlural policing has become a key focus in critical policing and security scholarship, with growing acknowledgement that policing practices involve multiple actors and diverse institutional forms. While much of the recent interest in plural policing has been prompted by the global growth of private security, plural policing in the Pacific Islands has long been evident in the co-existence of state-based police organisations operating nationally, and traditional or customary policing forms operating at local levels in most of these countries and territories. This chapter examines the plural character of Pacific policing in the context of historical and more recent processes of pluralisation, including the expanding domains of private and transnational policing, highlighting the deepening entanglement and interdependency between these various policing forms. The geographic focus is on the independent Melanesian countries, the region’s most populous, socially diverse, and challenging policing environments.
The International Policing Agenda in the Pacific
Authors: Watson Danielle , Howes Loene , Dinnen Sinclair , Bull Melissa , Amin Sara N. 
Published: February 2023

AbstractInternational policing is a complex and non-neutral endeavour, which encompasses a diverse range of activities such as peace operations, capacity development, cross-border partnerships to combat transnational crime, and disaster assistance. In the Pacific region, while partnerships have long been part of the development and security landscapes, they entail various expectations and obligations, influenced by the historical relationships between countries and their relative status as aid donors or recipients. This chapter outlines the nature and development of international policing in the Pacific context. It then provides examples of international policing in the region, where many Pacific Islands countries have been involved both as host nations for—and contributors to—these efforts. The chapter highlights the complexities of partnerships in the Pacific region, and the challenges and benefits involved.
Supersymmetric solitons in gauged $$ \mathcal{N} $$ = 8 supergravity
Authors: Anabalón Andrés , Gallerati Antonio , Ross Simon , Trigiante Mario 
Published: February 2023

Abstract
We consider soliton solutions in AdS4 with a flat slicing and Wilson loops around one cycle. We study the phase structure and find the ground state and identify supersymmetric solutions as a function of the Wilson loops. We work in the context of a scalar field truncation of gauged $$ \mathcal{N} $$
N
= 8 supergravity, where all the dilatons are equal and all the axions vanish in the STU model. In this theory, we construct new soliton solutions parameterized by two Wilson lines. We find that there is a degeneracy of supersymmetric solutions. We also show that, for alternate boundary conditions, there exists a non-supersymmetric soliton solution with energy lower than the supersymmetric one.
Electric/magnetic Newton-Hooke and Carroll Jackiw-Teitelboim gravity
Authors: Avilés Luis , Gomis Joaquim , Hidalgo Diego , Zanelli Jorge 
Published: February 2023

Abstract
We construct the electric and magnetic Newton-Hooke and Carroll Jackiw-Teitelboim gravity theories using the isomorphism of Newton-Hooke± and (A-)dS Carroll algebras in (1+1)-spacetime dimensions. The starting point is the non-relativistic and Carroll version of Jackiw-Teitelboim gravity without restrictions on the geometry studied in [1].
Environmental justice in South Africa: the dilemma of informal settlement residents
Authors: Kekana H. N. , Ruhiiga T. M. , Ndou N. N. , Palamuleni L. G. 
Published: February 2023

AbstractThe concept of environmental justice is well established in the literature; however, scholars still battle to agree on what it really means. This concept has become more relevant to the studies of informal settlements amongst others. The location and environmental variables in informal settlements suggests a variety of injustices that comes with location, limited access to water, poor or lack of sanitation, challenges with transport availability, accessibility, affordability, and lack of other social amenities. These and many other socio-economic needs forms part of the value chain of environmental justice debates across the world. This paper deals with environmental justice in the informal settlements of Kosmos, in the Madibeng Local Municipality, Bojanala Region in the North-West Province of South Africa. The paper highlights some of the environmental challenges faced by the informal settlement residents such as pollution, waste management (landfill sites, waste collection) sanitation and water provision. The paper explores how the Kosmos informal settlement community has been excluded from decision making processes regarding their own environment and considers the levels of environmental injustices commonly associated with this kind of practice.
Advances in the Mechanistic Study of the Control of Oxidative Stress Injury by Modulating HDAC6 Activity
Authors: Xue Yuanye , Gan Bing , Zhou Yanxing , Wang Tingyu , Zhu Tong , Peng Xinsheng , Zhang Xiangning , Zhou Yanfang 
Published: February 2023

AbstractOxidative stress is defined as an injury resulting from a disturbance in the dynamic equilibrium of the redox environment due to the overproduction of active/radical oxygen exceeding the antioxidative ability of the body. This is a key step in the development of various diseases. Oxidative stress is modulated by different factors and events, including the modification of histones, which are the cores of nucleosomes. Histone modification includes acetylation and deacetylation of certain amino acid residues; this process is catalyzed by different enzymes. Histone deacetylase 6 (HDAC6) is a unique deacetylating protease that also catalyzes the deacetylation of different nonhistone substrates to regulate various physiologic processes. The intimate relationship between HDAC6 and oxidative stress has been demonstrated by different studies. The present paper aims to summarize the data obtained from a mechanistic study of HDAC6 and oxidative stress to guide further investigations on mechanistic characterization and drug development.
North–South discrepancy and gender role attitudes: evidence from Vietnam
Authors: Do Trang Thu , Nguyen-Trung Kien , Le Chau Hai 
Published: February 2023

AbstractIn Vietnam, it is commonly believed that gender norms, sex labour segregation, and structural organization of social institutions often favour male dominance while restricting women’s roles in domestic spheres. However, there is a scant literature of Vietnamese scholarship on the determinants of gender role attitudes, especially geographical disparities. This paper aims to fill this void by using a nationally representative survey with 8288 respondents. Our findings suggested that age, marital status, religion, education, living area, region, ethnicity, and personal monthly income are the factors that predicted gender attitudes. In terms of regional disparities, we found that Northerners were more permissive in their gender attitudes than Southerners, which may be explained by distinct historical and political trajectories in Northern and Southern Vietnam during the last century. There were, however, inconsistent patterns among different age cohorts whereby region significantly impacted the attitudes of women born before the end of the French War in 1954, men born after the Reunification in 1975, as well as both men and women born between 1954 and 1975.
Boundedness of Complements for Log Calabi–Yau Threefolds
Authors: Chen Guodu , Han Jingjun , Xue Qingyuan 
Published: February 2023

AbstractIn this paper, we study the theory of complements, introduced by Shokurov, for Calabi–Yau type varieties with the coefficient set [0, 1]. We show that there exists a finite set of positive integers$$\mathcal {N}$$N, such that if a threefold pair$$(X/Z\ni z,B)$$(X/Z∋z,B)has an$$\mathbb {R}$$R-complement which is klt over a neighborhood ofz, then it has ann-complement for some$$n\in \mathcal {N}$$n∈N. We also show the boundedness of complements for$$\mathbb {R}$$R-complementary surface pairs.
ON LARGE ORBITS OF FINITE SOLVABLE GROUPS ON CHARACTERS
Authors: YANG YONG , LI JINBAO 
Published: February 2023

AbstractWe prove that if a solvable group A acts coprimely on a solvable group G, then A has a relatively ‘large’ orbit in its corresponding action on the set of ordinary complex irreducible characters of G. This improves an earlier result of Keller and Yang [‘Orbits of finite solvable groups on characters’, Israel J. Math.199 (2014), 933–940].
Explicit Gender Stereotyping in Canadian Politics
Authors: Chen Philip , Thomas Melanee , Harell Allison , Gosselin Tania 
Published: February 2023

AbstractIn this research note, we document the extent to which negative beliefs about women's capacity to hold public office are widespread in Canada. Using a list experiment, our results demonstrate that many Canadians believe that men are “naturally better” leaders than are women and that women are “too emotional” and “too nice” for politics. While some groups are willing to explicitly own these views when asked directly about them (for example, older people, men, those who are more conservative and religious), others are unwilling to do so unless social desirability is mitigated (for example, younger people, left-leaning). By overcoming concerns with social desirability, we show that women still face explicit, often sexist, barriers in political work.
To Claim or Not to Claim: Investigating Non-Take-Up of Welfare Schemes Targeting Hong Kong Older Adults and the Stigma Attached to Them
Authors: Kühner Stefan , Chou Kee-Lee 
Published: February 2023

AbstractThis article investigates the unique contribution of specific programme characteristics together with personal stigma, stigmatisation by the public, and claims stigma, to the non-take-up of targeted income support among Hong Kong older adults. Drawing on data from a sample of 3,299 Hong Kong older adults aged 65 or above, we find that between 11-14 per cent of eligible participants did not receive cash transfers from Normal and Higher Old Age Living Allowance (OALA) and old-age Comprehensive Social Security Assistance (old-age CSSA). By combining mainstream economic analysis with attempts to quantify welfare stigma (Baumberg, 2016) we find that transaction costs were most consistently and strongly related to non-take-up of targeted income support; non-take-up of old-age CSSA and Higher OALA but not Normal OALA varied with welfare stigma after controlling for personal and household characteristics of study participants. This article further adds to the literature by examining the effect of recent reforms to asset- and means-tested benefits for the same target population of older adults on take-up in the East Asian context. The article suggests that automatic switching of beneficiaries from Normal OALA to Higher OALA effectively facilitated higher take-up of the latter. The policy implications of these various findings are discussed.
Novel approach to decision making for orphan drugs
Authors: Decker Barbora , Mlcoch Tomas , Pustovalova Anastasie , Dolezal Tomas 
Published: February 2023

Abstract

Background
Out of 185 orphan medicinal products (OMPs) registered in 2015–2021, a mere 110 (59 percent) were available to Czech patients, and only 54 (29 percent) were officially reimbursed. Moreover, this proportion has steadily decreased over time. After years of public debate induced by this unsatisfactory OMP patient access, the national viewpoint shifted toward creating a special pathway for the reimbursement of OMP. Thus, a rigorous pricing and reimbursement procedure with strict timelines and elaborated methodology has been recently adopted in Czechia.

Methodology
The innovative legislation follows the recommendations for value assessment and funding processes for rare diseases and incorporates additional elements of value, such as the societal perspective. First, the application with clinical evidence, cost-effectiveness, and budget impact analyses is submitted to the governmental health technology assessment (HTA) agency by the Marketing Authorization Holder or a Health Insurance Fund. Moreover, professional associations and patients’ organizations are rightful participants in the proceeding, providing evidence and comments. Then, the HTA agency performs the assessment/appraisal of the evidence. It subsequently publishes the assessment report summarizing available information. The report is then forwarded to the Ministry of Health and its advisory body consisting of patients, clinical experts, health insurance funds, and the State. They critically evaluate the documents and issue a binding opinion following prespecified decision-making criteria. Based on this binding opinion, the decision is issued by the HTA agency. Thus, the role of the advisory body in this process is crucial.

Conclusion
We believe that this novel approach may offer satisfactory patient access to orphan drugs. Moreover, it serves as a real-world example of “value-based” decision making.