bluuebunny/crossref_metadata_2025_split huggingface dataset:
NNT mutations: a cause of primary adrenal insufficiency, oxidative stress and extra-adrenal defects
Authors: Roucher-Boulez Florence , Mallet-Motak Delphine , Samara-Boustani Dinane , Jilani Houweyda , Ladjouze Asmahane , Souchon Pierre-François , Simon Dominique , Nivot Sylvie , Heinrichs Claudine , Ronze Maryline , Bertagna Xavier , Groisne Laure , Leheup Bruno , Naud-Saudreau Catherine , Blondin Gilles , Lefevre Christine , Lemarchand Laetitia , Morel Yves 
Published: April 2016

Objective
Nicotinamide nucleotide transhydrogenase (NNT), one of the several genes recently discovered in familial glucocorticoid deficiencies (FGD), is involved in reactive oxygen species detoxification, suggesting that extra-adrenal manifestations may occur, due to the sensitivity to oxidative stress of other organs rich in mitochondria. Here, we sought to identify NNT mutations in a large cohort of patients with primary congenital adrenal insufficiency without molecular etiology and evaluate the degree of adrenal insufficiency and onset of extra-adrenal damages.

Methods
Sanger or massive parallel sequencing of NNT and patient monitoring.

Results
Homozygous or compound heterozygous NNT mutations occurred frequently (26%, 13 unrelated families, 18 patients) in our cohort. Seven new mutations were identified: p.Met337Val, p.Ala863Glu, c.3G>A (p.Met1?), p.Arg129*, p.Arg379*, p.Val665Profs*29 and p.Ala704Serfs*19. The most frequent mutation, p.Arg129*, was found recurrently in patients from Algeria. Most patients were diagnosed belatedly (8–18 months) after presenting severe hypoglycemia; others experiencing stress conditions were diagnosed earlier. Five patients also had mineralocorticoid deficiency at onset. One patient had congenital hypothyroidism and two cryptorchidism. In follow-up, we noticed gonadotropic and genitalia impairments (precocious puberty, testicular inclusions, interstitial Leydig cell adenoma, azoospermia), hypothyroidism and hypertrophic cardiomyopathy. Intrafamilial phenotype heterogeneity was also observed.

Conclusions
NNT should be sequenced, not only in FGD, but also in all primary adrenal insufficiencies for which the most frequent etiologies have been ruled out. As NNT is involved in oxidative stress, careful follow-up is needed to evaluate mineralocorticoid biosynthesis extent, and gonadal, heart and thyroid function.
Derecho Constitucional Hispanoamericano frente a la realidad institucional, manifiesta divergencia
Authors: Humbarita Jose Ignacio 
Published: April 2016

<p>La clase política en el poder desde la independencia ha impedido la realización de principios y libertades esenciales a la democracia, muy a pesar de los esfuerzos de los verdaderos opositores (no de las derechas disfrazadas de opositoras cuando han perdido el poder) que han sido neutralizados por diferentes vías, haciendo inofensiva la consagración de algunos derechos fundamentales y la modernización de les estados nación de esta parte del mundo. De dónde surge que el verdadero reto jurídico, político y social se deba enfocar en tener en cuenta los intereses en disputa, en conciliar los mandatos constitucionales y legales con la realidad política de la región para que se convierta en generador del progreso. De suerte que a imagen de lo que ocurre en las democracias reales, las normas superiores sean el derecho viviente y se erijan en gestoras de progreso.</p><p>Luego de analizar la temática, se llegó a la conclusión de la existencia de una percepción negativa del poder político muy a pesar de la existencia y vigencia formal de democracias constitucionales debido a la incoherencia y desbordada ambición de los políticos, fenómeno que conduce inexorablemente al atraso en todos los campos y a las insalvables fisuras entre los diferentes sectores de la población en la que minoría se encuentra integrada por las élites que direccionan a su servicio las instituciones, manipulación fácilmente evidenciable, y la mayoría conformada por los estratos que ocupan los lugares de depresión en las grandes urbes y en el sector rural, gran masa que desempeña únicamente el rol de gregaria en la actividad productiva y política de estos estados.</p><p> </p>
Genres of Underemployment: A Dialogical Analysis of College Graduate Underemployment
Authors: Cunningham Joseph 
Published: April 2016

With more individuals obtaining undergraduate and graduate degrees and the job market still recovering from the 2008 recession, the instances of college graduate underemployment (CGU) have increased throughout the United States. College graduate underemployment is an employment condition that is characterized by subjective and objective factors, most prominent of which is an incongruence between one’s education and one’s current job. The intriguing nature of CGU is how both employment and education merge together to influence the individual’s perception of their employment prospects, their educational experiences, and their identity. This study employs a dialogical qualitative analysis to examine CGU in order to ascertain how underemployed college graduates construct narratives of their experiences and define the value of their education. Twenty in-depth interviews of underemployed college graduates from different academic disciplines are analyzed with a dialogical genre analysis developed by Paul Sullivan. Two genre pairs—epic/romance and tragedy/black comedy—are employed to illustrate the correlating modes of experience for these participants, creating new narratives that problematize the dominant education-to-employment progression.
Jews, Muslims and the Ritual Male Circumcision Debate: Religious Diversity and Social Inclusion in Germany
Authors: Yurdakul Gökçe 
Published: April 2016

On 7 May 2012, the Cologne regional court ruled that circumcising young boys was a form of previous bodily harm (<em>körperverletzung</em>)<em>. </em>Although both Muslims and Jews circumcise infant boys as a religious practice, the Cologne court found that the child’s “fundamental right to bodily integrity” was more important than the parents’ rights, leaving Muslim and Jewish parents under suspicion of causing bodily harm to their children. After heated public discussions and an expedited legal process, legal authorities permitted the ritual circumcision of male children under a new law. However, the German debates on religious diversity are not yet over. On the third anniversary of the Court decision in 2015, thirty-five civil society organisations organised a rally in Cologne for “genital autonomy”, calling for a ban on ritual male circumcision. In this article, I will focus on religious diversity, which is undergoing changes through minority and immigrant claims for religious accommodation. Analysing the ongoing controversies of ritual male circumcision in Germany, I argue that this change is best observed with Muslim and Jewish claims for practicing their religion. By using political debates, news reports and information provided by lawyers and medical doctors who were involved in the public debate, I show that religious diversity debates are a litmus test for social inclusion: Muslims and Jews, in this context, are both passive subjects of social inclusion policies and active participants in creating a religiously diverse society in Germany.
Quantificação de depósitos em folhas de trapoeraba com diferentes tipos de pontas de pulverização
Authors: Pereira Rodolfo Gonçalves , Da Silva Thiago Barbosa Gonçalves , Anunciação Acácio Aparecido , Dos Santos Wellington Hipólito , Miranda Gustavo Rabelo Botrel , Alves Alberto Donizete , Sartori Raul Henrique 
Published: April 2016

Com o objetivo de verificar o melhor modelo de pontas de pulverização hidráulica para a quantificação de depósitos em folhas de Commelina benghalensis, foi desenvolvido um experimento, em março de 2012 no IFSULDEMINAS - Campus Muzambinho/MG, em lavoura de café da cultivar catuaí, com espaçamento de 3,7m x 1,0m. O delineamento utilizado foi em blocos ao acaso (DBC), com quatro tratamentos distribuídos em cinco blocos, onde foi avaliado o depósito de calda em folhas de trapoeraba. A solução marcadora era composta pelo corante azul brilhante (FD&C no 1) a 0,15% (p/v) e os tratamentos – tipos de pontas de pulverização – foram: leque duplo (modelo TJ-60 11002 VS); leque comum (modelo ADI 11002); indução de ar (modelo ADIA Magno 11002); e cônico vazio (modelo J-4) - todos acoplados a pulverizador costal pressurizado (CO2), utilizando barra com 2 bicos espaçados em 50 cm e altura de 40 cm do alvo biológico. As parcelas foram constituídas de 5 metros de comprimento por 1 metro de largura e, após a aplicação, foram coletadas 5 folhas de trapoeraba de cada parcela, que foram acondicionadas em sacos plásticos para serem lavadas e medidas posteriormente. A solução resultante foi analisada por espectrofotômetro a 630 nm, quando se determinou o volume depositado nas folhas em µL cm-². Os valores obtidos foram submetidos à análise de variância pelo teste F, e as médias, comparadas pelo teste de Tukey, ao nível de 5% de probabilidade, realizado pelo software SISVAR 4.3. Conclui-se que não há diferença significativa para deposição de calda em folhas de C. benghalensis para as pontas utilizadas.
Extração de informações da cena urbana combinando imagens RADARSAT-2 e GeoEye
Authors: Cavalheiro Anna Carolina 
Published: April 2016

Neste trabalho, foi analisada a contribuição da imagem RADARSAT-2 na discriminação de elementos de uma cena urbana, quando fundida com uma imagem GeoEye. A vantagem da imagem-radar é que sua tomada independe das condições atmosféricas, e a resolução espacial nominal no modo ultrafino é de 3 metros. Já as imagens multiespectrais, mesmo sendo de alta resolução, não permitem discriminar todos os elementos que compõem uma cena urbana. O objetivo deste trabalho foi discriminar elementos da cena, utilizando a combinação de informações obtidas de imagem óptica e de radar, e também verificar se o modelo de transformação projetiva é adequado para a correção geométrica da imagem radar. Pretende-se, ainda, analisar o efeito dos filtros para redução do speckle na imagem e constatar a contribuição da imagem radar para a identificação de elementos presentes na cena. A transformação projetiva apresentou um resultado satisfatório na correção da imagem, apesar de o ângulo de visada ser lateral; os valores de resíduos para os pontos de controle e verificação ficaram menores que 1 metro. Para tratar o speckle, verificou-se que o filtro estatístico de média com janela 5x5 foi o que permitiu sua eliminação com maior eficiência. Os resultados dos experimentos de classificação, utilizando árvore de decisão, apresentaram-se coerentes com a realidade da área de estudo. O melhor resultado foi verificado no experimento utilizando a imagem GeoEye segmentada, combinada com a imagem radar, uma vez que essa contribui para a caracterização de estruturas metálicas que apresentam alto fator de reflexão. Foi possível comprovar a confiabilidade da técnica na visita de campo.
TWISTING THE KNIFE- DISCRIMINATION IN THE LAW
Authors: GURNEY KAREN 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>[</span><span>Of the many different variations that can occur in human sexual formation, trans- sexualism no doubt remains the least understood by the wider Australian commu- nity. As a consequence, the process of attaining human rights to legal status, privacy, dignity and freedom from discrimination for those who experience this unusual condition has been a slow and sometimes frustrating one. The article seeks to introduce the reader to some of the more recent developments in the interna- tional jurisprudence of transsexualism and the underlying medical evidence that has supported them. It also offers criticism of the belated attempt by the State of Victoria, with the </span><span>Births, Deaths &amp; Marriages Registration (Amendment) Act 2004, </span><span>to establish certain statutory rights in this regard. While the legislation was en- acted with the stated and very laudable purpose of providing for the correction of birth records on the Register of Births of those people with transsexualism who have altered their phenotypic sex by hormonal medication and surgery, the article argues it has also served to remove other equally important rights already won and proposes that a final remedy will only be found, as on previous occasions, in the courts.</span><span>] </span></p></div></div></div>
ETHICS - THE ADVERSARIAL SYSTEM AND BUSINESS PRACTICE*
Authors: NETTLE GEOFFREY 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>[</span><span>This article is an edited version of a lecture delivered in November 2004 to Melbourne University Juris Doctor students. It takes as its starting point the argument that the ethics of the adversarial legal system ill accord with the norms of society and that we should discard it in favour of the inquisi- torial system. That view is contrasted with the school of thought which takes the ethical superiority of the adversarial system as given and with the idea that increasing commercialisation of the legal profession may be leading us away from the nobler traditions of the adversarial system.</span><span>] </span></p></div></div></div>
Crime and Justice: A Review of Research (Vol. 30) EDITED BY MICHAEL TONRY (CHICAGO, UNIVERSITY OF CHICAGO PRESS, 2003) 521 PAGES. PRICE: US$54.00. ISBN: 0 226 80862 9
Authors: RENAUD GILLES 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>In what might well be the most robust and valuable contribution to date, Volume 30 of </span><span>Crime and Justice A Review of Research </span><span>provides a signal understanding of the state of inquiry and knowledge surrounding some of the most significant crimino- logical debates yet undertaken, with ground-breaking attention to the emerging areas of international trafficking in stolen vehicles and the question of police profil- ing. </span></p></div></div></div>
NOT QUITE A BRAVE NEW WORLD: VICTORIA’S OCCUPATIONAL HEALTH AND SAFETY ACT 2004
Authors: ADAMS K LEE 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>[</span><span>This article provides a concise overview of the Victoria’s new </span><span>Occupa- tional Health and Safety Act 2004 </span><span>(“OHSA 2004”). After outlining the Maxwell Report on which much of the OHSA 2004 is based, the article ex- amines the principal legislative provisions of the Act, especially those that differ from the </span><span>Occupational Health and Safety Act 1985 </span><span>(Vic) (“1985 Act”). Analysis of the legislation evaluates some positive developments, as well as suggests amendments. Although the OHSA 2004 contains numer- ous alterations in its scheme as compared to the 1985 Act, these changes are unlikely to usher in a brave new world of occupational health and safety regulation in Victoria.</span><span>] </span></p></div></div></div>
MULTIPLE PRINCIPLES AND THE OBLIGATION TO OBEY THE LAW
Authors: AHIAUZU NKIRUKA 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>[</span><span>George Klosko’s multiple principle theory of political obligation is a re- cent formulation for the existence of a general obligation to obey the law. In the paper, I argue that the generality requirement of the obligation to obey the law gives rise to normative and factual problems of, respectively, motivation and comprehensiveness. I aim to show that whereas the multi- ple principle theory may solve the factual problem of the generality re- quirement, it does not solve its normative problem which I characterise as a first-personal implication of the question, ‘why should I obey the law?’</span><span>] </span></p></div></div></div>
ANOTHER MODEST PROPOSAL
Authors: MANDERSON DESMOND 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>[</span><span>In essays first published earlier this year in mainstream Australian media to considerable fanfare, Bagaric and Clarke, two Australian academics, develop a modest proposal for the justification of torture under excep- tional circumstances. This essay rebuts the proposal and defends the abso- lute prohibition against torture. Their attempt to abstract torture from the social context – including the “war on terror” – in which the question of government sanctioned torture is now being raised, is condemned as in- genuous. A rhetorical analysis further demonstrates that the authors them- selves do not believe their argument is either hypothetical or limited. Furthermore, when the actual “defence of torture” is examined, it is shown to be illogical, incoherent, and lacking any sophisticated under- standing of the nature, purpose, or effects of torture. This is not the first time that half-baked reasoning and careless analogies have been devel- oped in order to defend the indefensible. Drawing on Voltaire and Jona- than Swift as well as Guantanamo Bay, this essay puts an important social issue into its immediate and its larger historical context</span><span>.] </span></p></div></div></div>
A CRITICAL EVALUATION OF THE KOORI COURT DIVISION OF THE VICTORIAN MAGISTRATES’ COURT
Authors: MCASEY BRIDGET 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>[</span><span>The Koori Court Division of the Magistrates’ Court in Victoria has been in operation since 2002. This article seeks to assess its development and operation, with the perspective that the Division has the potential to ad- dress problems Aboriginal people face in the criminal justice system and society generally. The author takes the view, however, that to fulfil this po- tential, the Division’s development and operation must function in a way that makes some effort to adjust the power imbalance between the Abo- riginal and non-Aboriginal community, The author sees a critical ap- proach to an evaluation of the Division as crucial, considering the background of treatment Aboriginal people have received at the hands of the criminal justice system and Australian society as a whole, and the negative impact of previous government policies.</span><span>] </span></p></div></div></div>
W. WESLEY PUE AND DAVID SUGARMAN, LAWYERS & VAMPIRES CULTURAL HISTORIES OF LEGAL PROFESSIONS (OXFORD: HART PUBLISHING, 2003) 399 PAGES ISBN: 1-84113-312-4
Authors: RENAUD GILLES 
Published: April 2016

<div class="page" title="Page 1"><div class="layoutArea"><div class="column"><p><span>As is noted in the preface, this “book seeks to develop understandings of the his- torical and social functions of legal professions on a comparative basis and through interpretative lenses sensitive to the urges of ‘cultural history”. The editors go on to note the “essence of a comparative ‘cultural history’ of legal professions is the pursuit of an understanding transcending the spatial, temporal or disciplinary boxes that conventionally constrain scholarship.” </span></p></div></div></div>
Police Liability for Negligent Investigations: When Will a Duty of Care Arise?
Authors: Shircore Mandy 
Published: April 2016

<p>In 1988 in Hill v Chief Constable of West Yorkshire, the House of Lords denied a duty of care was owed by police to a victim of a serial murderer. The case was interpreted as immunity from negligence actions for police when involved in the suppression and investigation of crime’. Recent cases in Australia and the United Kingdom have confirmed that while blanket immunity from negligence actions for police involved in investigatory functions does not exist, plaintiffs alleging negligence will face considerable difficulties establishing a duty of care was owed. The recent cases provide an opportunity to re-examine the appropriate tests to apply in determining the existence and scope of police duty of care and to reassess the policy grounds underlying the decisions. In doing so the article argues that Australian courts should avoid the UK approach of applying a<br />broad exclusionary rule particular to police, as sufficient safeguards already exist in the Australian High Court’s duty of care formulation for public authorities.</p>
Preparing Australia's Future Lawyers: An Exposition of Changing Values Over Time In The Context Of Teaching About Ethical Dilemmas
Authors: Evans Adrian , Palermo Josephine 
Published: April 2016

<p>This study investigated what values may be influential to decision making in relation to ethical behaviour for early career lawyers. It adopted a longitudinal approach to investigate how values develop or degrade over time as final year law students move into their first two years of employment or further study. To this end, the study investigated the role that tertiary education and employers fulfill in building and perpetuating ‘appropriate’ professional values? Results demonstrate that, in general, ethical behaviour was not uniformly reinforced over time in the workplace. The undertaking of pro bono work stands out here. Results suggested that certain behaviour relevant values may develop or degrade over the early years of the<br />Australian lawyer’s career. The implications of results are discussed in the contexts of ethics education in a tertiary context and the continuing education and regulation of the legal profession.</p>
Driving While Disqualified or Suspended under s 30 of the Road Safety Act 1986 (Vic): Abolition of the Mandatory Sentencing Provision?
Authors: Coleman Belinda 
Published: April 2016

<p>In an earlier article in 2001, Edney and Bagaric argued that the mandatory sentencing of persons to imprisonment pursuant to s 30 of the Road Safety Act 1986 (Vic) for second or subsequent offences of Driving While Disqualified or Suspended cannot be justified and that reform is required. Since then the topic of mandatory sentencing for Driving While Disqualified or Suspended has assumed even greater importance having regard to (a) an increase in the number of administrative ways a person can now have their licence cancelled or disqualified; (b) the availability of recent empirical data demonstrating the number of persons sentenced to imprisonment for this offence; (c) the results of a major review of Victorian sentencing law and (d) an increase in the different ways a sentence of imprisonment can in fact (and in law) be served. In view of these developments, this article re-examines the use of mandatory sentencing for Driving While Disqualified or Suspended and argues that the arguments put forward by Edney and Bagaric are even more compelling five years down the track.</p>
Incorporation of Unusual or Unreasonable Terms Into Contracts: The Red Hand Rule and Signed Documents
Authors: Kapnoullas Steve , Clarke Bruce 
Published: April 2016

<p>This article analyses case law relating to the red hand rule. In particular, there is an examination of the decision of the High Court in Toll v Alphapharm Pty Ltd. The judgment of the court signals a clear answer as to whether the red hand rule applies to signed documents. In this context, the authors also consider whether section 52 of the Trade Practices Act, and statutory provisions relating to unconscionability, are available to protect consumers against unusual or unreasonable clauses incorporated into signed contracts. Two recent cases involving share trader David Tweed are also analysed.</p>