bluuebunny/crossref_metadata_2025_split huggingface dataset:
3-Hydroxyisobutyryl-CoA Hydrolase (HIBCH) Deficiency Cases Diagnosed by Only HIBCH Gene Analysis and Novel Pathogenic Mutation
Authors: Çakar Nafiye Emel , Görükmez Orhan 
Published: January 2021

Objective:
3-Hydroxyisobutyryl-CoA hydrolase (HIBCH) deficiency is a rare metabolic disease of valine metabolism. Only 22 cases of HIBCH deficiency have been reported in the literature. Our algorithm could help in the diagnosis of this disease.

Methods:
HIBCH gene analysis was performed in all cases.

Results:
The common features of our five patients from the same family with a developmental delay, seizures, and neurological regression were the elevation of 3-hydroxy-isobutyryl-carnitine and Leigh-like abnormalities. Unlike other patients in the literature, our patients were diagnosed with HIBCH gene analysis, rather than whole exome sequencing (WES). In all our cases, a missense c.452C>T, p. Ser151Leu homozygous novel pathogenic mutation was detected in the HIBCH gene.

Conclusion:
In cases where HIBCH deficiency is considered in our differential diagnosis algorithm, HIBCH gene analysis, which is cost-effective, should be performed instead of WES, and the number of cases should be increased in the literature.
Diversité floristique des dépendances vertes périurbaines de Brazzaville (Congo) menacées de dégradation
Authors: Mikoungui Gomo Mat-Sheridan , N’zala Donatien , Ndzai Saint Fédriche 
Published: January 2021

A Brazzaville, les dépendances vertes jouent un rôle important sur le bien-être et le cadre de vie des citadins. Cependant elles sont menacées de dégradation à cause d’une urbanisation mal contrôlée. Cette étude a été menée pour identifier et caractériser la biodiversité végétale de ces milieux. A cet effet, des relevés floristiques ont été réalisés au niveau de deux types de phytocénoses « dégradée » et « semi intacte » dans deux sites des quartiers périphériques de Ngamakosso et Sadelmi suivant un dispositif de 17 parcelles (30 m x 20 m). Au total, 205 espèces végétales reparties en 160 genres et 65 familles ont été inventoriées. La présence/abondance de certaines espèces et familles (Asteraceae, Rubiaceae, Poaceae) associée avec l’appréciable proportion des nanophanérophytes, révèle le caractère arbustif et perturbé de cette végétation sur des sols sableux et pauvres, favorables aux érosions. La conservation et la reconstitution des phytocénoses étudiées sont assurées grâce à des espèces endémiques, guinéo-congolaises et pantropicales, à très large distribution. Les connaissances acquises de cette étude suggèrent d’intégrer la problématique de l’aménagement des espaces végétalisés urbains dans les politiques de développement durable de la ville.
Mots clés : Richesse floristique, biodiversité urbaine, phytocénose, conservation, reconstitution.
English Title: Floristic diversity of peri-urban green dependencies in Brazzaville (Congo) threatened by degradation
In Brazzaville, green dependencies have an important role in the well-being and living environment of city dwellers. However, they are threatened with degradation due to poorly controlled urbanization. This study was conducted to identify and characterize the plant biodiversity of these habitats. For this purpose, floristic surveys were carried out at the level of two kinds of "degraded" and "semi intact" phytocenosis at two sites in the outlying areas of Ngamakosso and Sadelmi following a scheme of 17 plots (30 m x 20 m). In total, 205 plant species distributed into 160 genera and 65 families were inventoried. The presence/abundance of certain species and families (Asteraceae, Rubiaceae, Poaceae) associated with the significant proportion of nanophanerophytes, revealed the shrubby and disturbed character of this vegetation on sandy, poor and erosive soils. The conservation and reconstitution of the studied phytocenosis are ensured by endemic, Guineo-Congolese and pantropical species, with very wide distribution. The knowledge acquired from this study suggests integrating the issue of vegetative landscaping into the city’s sustainable development policies.
Keywords: Floristic richness, urban biodiversity, phytocenosis, conservation, reconstitution
Caractérisation de la flore phytoplanctonique dans l’Aire Marine Protégée (AMP) de Bamboung et de deux sites environnants (Sénégal)
Authors: Abdou Salam Ali Mohamed , Ba Ngansoumana , Ndour Ismaïla , Sane Seyni , Thiaw Modou , Diouf Ndongo , Diouf Jules , Diop Djibril , Gueye Madiop , Brehmer Patrice , Mbaye Mame Samba , Kane Aboubacry , Noba Kandioura 
Published: January 2021

Au Sénégal, peu d’études sur le plan planctonique ont été réalisées pour l’évaluation de l’efficacité des Aires Marines Protégées. Dans cette perspective, la caractérisation de la flore phytoplanctonique et sa distribution spatio-temporelle à l’intérieur et à l’extérieur de l’AMP de Bamboung a été réalisée en avril et en septembre 2016. Des prélèvements d’échantillons d’eau à l’aide d’un filet à plancton de 20 μm de mailles ainsi que des mesures de paramètres physico-chimiques ont été faits dans chaque station. L’analyse des données environnementales a montré l’existence d’un effet intersaison avec des valeurs plus élevées en avril excepté la température. Par ailleurs, un effet d’inter-sites a été observé uniquement en septembre pour la température, la salinité, la conductivité et l’oxygène dissout. En termes de diversité phytoplanctonique, en avril, 49 espèces réparties dans 5 classes ont été identifiées dans l’AMP contre 47 espèces et 5 classes identifiées à Diomboss et 42 espèces et 5 classes à Sangako. En septembre, 57 espèces réparties dans 5 classes ont été répertoriées dans l’AMP contre 54 espèces et 4 classes à Diomboss, et 51 espèces réparties dans 4 classes identifiées à Sangako. Les résultats seuls de la richesse spécifique du phytoplancton ne permettent pas d’apporter une contribution sur l’évaluation de l’efficacité de l’AMP de Bamboung.
Mots clés : Phytoplancton, dynamique, composition taxonomique, AMP, Bamboung.

English Title: Characterization of the phytoplankton flora in the Protected Marine Area (MPA) of Bamboung and two surrounding sites (Senegal)
In Senegal, few planktonic data exist in the Marine Protected Areas (AMP). In this perspective, the characterization of phytoplankton flora and its spatial and temporal distribution within and outside the Bamboung AMP was carried out during in April and in september. Water samples were taken using a 20 μm mesh plankton net and measurements of physico-chemical parameters were made at each station. The analysis of environmental data showed the existence of an inter-site effect during the two-month sampling period. In April, 49 species in 5 classes were identified in the AMP, compared to 47 species and 5 classes identified in Diomboss and 42 species and 5 classes in Sangako. In september, 57 species distributed in 5 classes were recorded in the AMP compared to 54 species distributed in 4 classes in Diomboss and 51 species and 4 classes identified in Sangako. The results of the specific richness of phytoplankton alone do not make it possible to make a contribution to the evaluation of the effectiveness of Bamboung's MPA.Keywords: Phytoplankton, dynamics, taxonomic composition, AMP; Bamboung.
Use of essential oils in combination with fibrolytic enzymes to decrease in vitro ruminal methane production
Authors: Nel T.C. , Hassen A. , Akanmu A.M. , Adejoro F.A. 
Published: January 2021

Exogenous enzymes and essential oils (EOs) have both improved fibre digestion in ruminants, but the effect on ruminal fermentation and methane emission of combining these additives requires further evaluation. Various EOs were used in combination with an enzyme in an in vitro gas production system to measure organic matter digestibility and methane after 48 hours of incubation of Eragrostis curvula. The treatments consisted of T1) unaugmented control; T2) enzyme (cellulose-xylanase mixture, 1:1 w/w, 1.5 mL/g DM); T3) enzyme combined with Next Enhance® (cinnamaldehyde with diallyl disulfide and diallyl trisulfide, 43:3.85 w/wt); T4) enzyme and cinnamon oil; T5) enzyme and garlic oil; and T6) enzyme with cinnamon and garlic oil, 1:1 w/w). At 2, 4, 8, 12, 24, 36, and 48 hours of incubation, gas production was measured, and gas samples were analysed for methane concentration. The inclusion of additives affected cumulative gas production (P <0.05), but only slightly affected methane production (P =0.05) after 48 hours of incubation. The addition of enzymes increased both gas and methane production. T5 and T6 both reduced gas production compared with T2. Combining EOs with an enzyme reduced methane production compared with T2 after 48 hours of incubation. The additives tended to increase in vitro organic matter digestibility (P =0.09), although they showed a tendency towards reduced methane production per unit of organic matter digested (P =0.07). Further studies on these enzyme and EO combinations under different doses and substrates are required to validate their efficacy for ruminant production.
Keywords: cinnamon oil, cinnamaldehyde, garlic oil, gas production, glucanase, in vitro digestibility, xylanase
La resolución de problemas matemáticos como estrategia de aprendizaje activo de los alumnos de 15 años: un estudio de los resultados de PISA en República Dominicana
Authors: Cruz-Pichardo Ivanovnna Milqueya 
Published: January 2021

La resolución de problemas es una de las habilidades más importantes para los estudiantes tanto para su formación escolar como en su diario vivir. Por dicha razón, resulta necesario que desde las aulas de matemática se desarrollen actividades y modelos que permitan el desarrollo de esta habilidad. En este artículo se hace un análisis de los resultados de la prueba PISA 2015 y 2018 con la finalidad de conocer las principales deficiencias de los estudiantes dominicanos, y a partir de estas, plantear iniciativas para la mejora del sistema, especialmente en el área de Matemática. Los resultados muestran las necesidades cognitivas y de habilidades de procesos que tienen los estudiantes que participaron en el estudio al momento de resolver problemas y la necesidad que tiene el sistema educativo dominicano de mejorar en el uso de estrategias que propicien esta competencia.
INFORMATION TECHNOLOGY CAPABILITIES AND COMPETITIVE ADVANTAGE: A REVIEW
Authors: Muazu UMAR Adeiza , ABDULMALIK SAMBO 
Published: January 2021

Purpose: The purpose of this review is to examine the relationship between information technology (IT) capabilities of enterprises and their ability to gain competitive advantage through IT resources, based on underpinning theories and empirical studies.
Methodology: The approach to the review was to conduct extensive literature searches in the fields of strategic management and information technology management, as a result of which four supporting models and perspectives, that is, technology acceptance model (TAM), unified theory of acceptance and use of technology (UTAUT), the IT capability theory, and RBV, and seven empirical studies that linked IT capability dimensions of IT infrastructure; IT human resources; and IT knowledge management and competitive advantage were eventually reviewed.
Findings: The review provides indications to better understand the relationship between IT capabilities and competitive advantage. The RBV was discovered to be a very popular and useful underpinning theory favored by many scholars in explaining the relationship. Evidence from empirical studies showed a positive and significant relationship between IT capabilities and competitive advantage, which led to conclusions and recommendations.
Contributions to theory, practice, and policy: The review was able to highlight the importance of investment and deployment of IT resources such as infrastructure, human resources, and knowledge management to gain a competitive advantage, and established the role of RBV in strategic management research. Recommendations were made on theory, practice, and policy
Fashion Remains
Authors: Pecorari Marco 
Published: January 2021

Fashion ephemera–from catalogues and invitations to press releases–have long been overlooked by the fashion industry and fashion academics. Fashion Remains redresses the balance, putting these objects centre stage and focusing on the wider creative practice of contemporary fashion designers, photographers, graphic designers, make-up artists and many more. Fashion ephemera are considered not as disposable promotional devices, but as windows into hidden networks of collaboration and value creation in the fashion system.
Taking an interdisciplinary approach, Fashion Remains explores the unseen and privately circulated fashion ephemera produced by today’s most prominent international fashion designers such as Margiela, Yamamoto and Raf Simons. Showcasing a unique archive of materials, it focuses on Antwerp’s avant-garde fashion scene and reveals the potential of these ephemeral objects to evoke and call into question material and immaterial knowledge about the fashion industry’s actors, practices and ideologies.
Fashion Promotion
Authors: Moore Gwyneth 
Published: January 2021

Fashion Promotion is an inspiring and practical guide to promoting a brand. It addresses the new ways in which brands engage with customers, through the latest digital channels as well as traditional methods. Topics covered include developing a brand from an original idea, the impact of influencers and street style sites, digital fashion, online and offline marketing techniques, creating the vision behind a brand and public relations.
From marketing, PR and collaboration to creating brand visuals, Fashion Promotion guides readers through the ways in which any brand - large or small - can embrace the opportunities brought about by developments in digital communication, in order to engage with consumers in new and exciting ways.
The new edition covers the impact of mobile technology on fashion retail transactions and marketing, the role of virtual and augmented reality and the increasing importance of sustainable production and distribution.
Ernesto Nathan Rogers
Authors: Sabini Maurizio 
Published: January 2021

Architect Ernesto Nathan Rogers (1909–1969) was a towering figure in twentieth-century Italian architecture. Through the work of his collaborative firm (Banfi Belgiojoso Peressutti Rogers, or BBPR), who were responsible for many of the most influential Italian projects of the time, and through the editorship of publications such as Domus and Casabella, Rogers ensured a lasting influence on the field. However his contributions have been largely neglected by scholarship, or more recently have had only superficial understandings attached to them.
Published as part of the Bloomsbury Studies in Modern Architecture series, which brings to light the work of significant yet overlooked modernist architects, this book will re-assess Ernesto Nathan Rogers’ cultural legacy. It will be the first comprehensive, critical work on Rogers in English, and will emphasize Rogers’ vision for the role of the architect as a public intellectual, as well as his commitment to pursue a renewed path of professional and cultural research within the “Modern Project.”
The book also discusses Rogers’ willingness to challenge academic classicized monumentality and the fascist administration to emerge as a leader of Italian design in the aftermath of World War II; his focus on urban design as well as planning; tradition in modernity; history and vernacular culture; and national identity, to bring a detailed account of the work and thought of Ernesto Nathan Rogers to an English-speaking audience for the first time.
With a foreword by Kenneth Frampton.
Boredom, Architecture, and Spatial Experience
Authors: Parreno Christian 
Published: January 2021

Boredom is a ubiquitous feature of modern life. Endured by everyone, it is both cause and effect of modernity, and of situations, spaces and surroundings. As such, this book argues, boredom shares an intimate relationship with architecture—one that has been seldom explored in architectural history and theory.
Boredom, Architecture, and Spatial Experience investigates that relationship, showing how an understanding of boredom affords us a new way of looking at and understanding the modern experience. It reconstructs a series of episodes in architectural history, from the 19th century to the present, to survey how boredom became a normalized component of the everyday, how it infiltrated into the production and reception of architecture, and how it serves to diagnose moments of crisis in the continuous transformations of the built environment.
Erudite and innovative, the work moves deftly from architectural theory and philosophy to literature and psychology to make its case. Combining archival material, scholarly sources, and illuminating excerpts from conversations with practitioners and thinkers—including Charles Jencks, Rem Koolhaas, Sylvia Lavin, and Jorge Silvetti—it reveals the complexity and importance of boredom in architecture.
And This Is My Friend Sandy
Authors: Philips Deborah 
Published: January 2021

This book situates the production of The Boy Friend and the Players’ Theatre in the context of a post-war London and reads The Boy Friend, and Wilson's later work, as exercises in contemporary camp. It argues for Wilson as a significant and transitional figure both for musical theatre and for modes of homosexuality in the context of the pre-Wolfenden 1950s.
Sandy Wilson's The Boy Friend is one of the most successful British musicals ever written. First produced at the Players' Theatre Club in London in 1953 it transferred to the West End and Broadway, making a star out of Julie Andrews and gave Twiggy a leading role in Ken Russell's 1971 film adaptation.
Despite this success, little is known about Wilson, a gay writer working in Britain in the 1950s at a time when homosexuality was illegal.
Drawing on original research assembled from the Wilson archives at the Harry Ransom Center, this is the first critical study of Wilson as a key figure of 1950s British theatre. Beginning with the often overlooked context of the Players' Theatre Club through to Wilson's relationship to industry figures such as Binkie Beaumont, Noël Coward and Ivor Novello, this study explores the work in the broader history of Soho gay culture. As well as a critical perspective on The Boy Friend, later works such as Divorce Me, Darling!, The Buccaneer and Valmouth are examined as well as uncompleted musical versions of Pygmalion and Goodbye to Berlin to give a comprehensive and original perspective on one of British theatre's most celebrated yet overlooked talents.
Performance in an Age of Precarity
Authors: Costa Maddy , Field Andy 
Published: January 2021

“This magical book is a love letter to the artists whose imagination and cleverness transport us and unite us, and to the beauty and fragility of their performance. When I read it I feel like I am constantly on the joyful edge of falling in love, trying so hard to keep hold of the feelings evoked. A very precious book in our precarious times.” Vicky Featherstone
An anthology of critical essays that draw on a decade of the authors thinking, writing about and working within contemporary performance as critics, producers, dramaturgs, makers, archivists and more.
Together, the 40 essays sketch a map of the contemporary performance landscape from avant-garde dance to live art to independent theatre, tracing the contours of its themes, aims, desires and relationship to the wider worlds of mainstream theatre, art and politics.
Each essay focuses on a particular artist and these include Bryony Kimmings, Dickie Beau, Forced Entertainment, rashdash, Scottee, Selina Thompson, Tania El Khoury and Uninvited Guests.
Reflecting the radical nature of the work considered, the authors attempt to find a new vocabulary and a non-conventional way of considering live performance in these essays.
As both a fresh survey of contemporary performance and an exploration of how to think and write about upstream and avant-garde work, this book should be an essential resource for students, artists and audiences, as well as an accessible entry point for anyone curious to know about the beautiful and strange things happening beyond the UK’s theatrical mainstream.
1997 The Future that Never Happened
Authors: Sayeed Richard Power 
Published: January 2021

Beautifully written, brilliantly insightful’
Owen Jones
Tony Blair and Noel Gallagher shaking hands at No. 10. Saatchi’s YBAs setting the international art world aflame. Geri Halliwell in a Union Jack dress. A time of vibrancy and optimism: when the country was united by the hope of a better and brighter future. So why, twenty years on, did that future never happen?
Richard Power Sayeed takes a provocative look at this epochal year, arguing that the dark undercurrents of that time had a much more enduring legacy than the marketing gimmick of ‘Cool Britannia’. He reveals how the handling of the Stephen Lawrence inquiry ushered in a new type of racism. How the feminism-lite of ‘Girl Power’ made sexism stronger. And how the promises of New Labour left the country more fractured than ever.
This lively, rich and evocative book explores why 1997 was a turning point for British culture and society – away from a fairer, brighter future and on the path to our current malaise.
America’s Backyard
Authors: Livingstone Grace 
Published: January 2021

The United States has shaped Latin American history, condemning it to poverty and inequality by intervening to protect the rich and powerful. America’s Backyard tells the story of that intervention.
Using newly declassified documents, Grace Livingstone reveals the US role in the darkest periods of Latin American history, including Pinochet’s coup in Chile, the Contra War in Nicaragua and the death squads in El Salvador. She shows how George W Bush’s administration used the War on Terror as a new pretext for intervention; how it tried to destabilise leftwing governments and push back the ‘pink tide’ washing across the Americas. America’s Backyard also includes chapters on drugs, economy and culture. It explains why US drug policy has caused widespread environmental damage yet failed to reduce the supply of cocaine, and it looks at the US economic stake in Latin America and the strategies of the big corporations.
Today Latin Americans are demanding respect and an end to the Washington Consensus. Will the White House listen?
Bipolar Orders: The Two Koreas Since 1989
Authors: Lynn Hyung Gu 
Published: January 2021

For over 1500 years before the Empire Windrush docked on British shores, people of African descent have played a significant and far-ranging role in the country’s history, from the African soldiers on Hadrian’s Wall to the Black British intellectuals who made London a hub of radical, Pan-African ideas. But while there has been a growing interest in this history, there has been little recognition of the sheer breadth and diversity of the Black British experience, until now.
This collection combines the latest work from both established and emerging scholars of Black British history. It spans the centuries from the first Black Britons to the latest African migrants, covering everything from Africans in Tudor England to the movement for reparations, and the never ending struggles against racism in between.
An invaluable resource for both future scholarship and those looking for a useful introduction to Black British history, Black British History: New Perspectives has the potential to transform our understanding of Britain, and of its place in the world.