Expanding the spectrum of PTH1R mutations in patients with primary failure of tooth eruption

Objectives: Primary failure of tooth eruption (PFE) is a rare autosomal-dominant disease characterized by severe lateral open bite as a consequence of incomplete eruption of posterior teeth. Heterozygous mutations in the parathyroid hormone 1 receptor (PTH1R) gene have been shown to cause PFE likely due to protein haploinsufficiency. To further expand on the mutational spectrum of PFE-associated mutations, we report here on the sequencing results of the PTH1R gene in 70 index PFE cases. Materials and methods: Sanger sequencing of the PTH1R coding exons and their immediate flanking intronic sequences was performed with DNA samples from 70 index PFE cases. Results: We identified a total of 30 unique variants, of which 12 were classified as pathogenic based on their deleterious consequences on PTH1R protein while 16 changes were characterized as unclassified variants with as yet unknown effects on disease pathology. The remaining two variants represent common polymorphisms. Conclusions: Our data significantly increase the number of presently known unique PFE-causing PTH1R mutations and provide a series of variants with unclear pathogenicity which will require further in vitro assaying to determine their effects on protein structure and function. Clinical relevance: Management of PTH1R-associated PFE is problematic, in particular when teeth are exposed to orthodontic force. Therefore, upon clinical suspicion of PFE, molecular DNA testing is indicated to support decision making for further treatment options. © 2013 Springer-Verlag Berlin Heidelberg.

The management oversight and risk tree-MORT : including systems developed by the Idaho Operations Office and Aerojet Nuclear Company,

"February 12, 1973."

Brilliant mistake! Essays on incidents of management mistakes and mea culpa

Purpose – The purpose of this paper is to examine students’ perceptions of managerial mistakes and why (and why not) managers admit mistakes. Design/methodology/approach – This paper provides a reflective account of how students’ perceive management mistakes and deal with admitting “mea culpa” – “I am to blame”. Findings – The findings show a range of attitudes: they highlight the intermingling pressures associated with the cultural environment and mistakes; they identify media characteristics and its influences on mistakes and mea culpa; they highlight ceremonial processes and tasks that shape and influence the declaration of mea culpa; and they identify how the psychology and sociology of mistakes confronts and affects students. Taken together, the study highlights the varying degrees of wariness that is carried forward by the students from vicariously learning about management mistakes. Originality/value – This paper links up with recent discussions on retail failure and retail pedagogy. It is hoped that this paper will encourage more academics to address, and engage with, management mistakes creatively in their teaching.

Modernity and the 'failure' of crime control

Rather than understanding the recurrent failure of various attempts at crime control as unfortunate and undesirable aberrations, all too familiar glitches an otherwise uninterrupted teleological march to a better society, such failures are instead positioned as part of the fabric of late modernity itself. That is, society changes not according to a predetermined logic along neatly defined and clearly reasoned tracks, rather it hurtles from crisis to crisis, from failure to failure, and it is the regulation of that failure which produces new initiatives and new forms of governance. Utilising the example of the modern prison, this chapter contends that too great an emphasis upon this institution’s ‘failure’ results not only in a neglect of the many other functions that it serves in the regulation of difference, but also, and more generally, it results in an underestimation of the importance of failure in providing new impetus for social transformation.

The failure of Australian legislation on indirect discrimination to detect the systemic racism which prevents Aboriginal people from fully participating in the workforce

Government figures put the current indigenous unemployment rate at around 23%, 3 times the unemployment rate for other Australians. This thesis aims to assess whether Australian indirect discrimination legislation can provide a remedy for one of the causes of indigenous unemployment - the systemic discrimination which can result from the mere operation of established procedures of recruitment and hiring. The impact of those practices on indigenous people is examined in the context of an analysis of anti-discrimination legislation and cases from all Australian jurisdictions from the time of the passing of the Racial Discrimination Act by the Commonwealth in 1975 to the present. The thesis finds a number of reasons why the legislation fails to provide equality of opportunity for indigenous people seeking to enter the workforce. In nearly all jurisdictions it is obscurely drafted, used mainly by educated middle class white women, and provides remedies which tend to be compensatory damages rather than change to recruitment policy. White dominance of the legal process has produced legislative and judicial definitions of "race" and "Aboriginality" which focus on biology rather than cultural difference. In the commissions and tribunals complaints of racial discrimination are often rejected on the grounds of being "vexatious" or "frivolous", not reaching the required standard of proof, or not showing a causal connection between race and the conduct complained of. In all jurisdictions the cornerstone of liability is whether a particular employment term, condition or practice is reasonable. The thesis evaluates the approaches taken by appellate courts, including the High Court, and concludes that there is a trend towards an interpretation of reasonableness which favours employer arguments such as economic rationalism, the maintenance of good industrial relations, managerial prerogative to hire and fire, and the protection of majority rights. The thesis recommends that separate, clearly drafted legislation should be passed to address indigenous disadvantage and that indigenous people should be involved in all stages of the process.

The strength and failure of silica optical fibers

The mechanical strength and failure behavior of conventional and microstructured silica optical fibers was investigated using a tensile test and fracture mechanics and numerical analyses. The effect of polymer coating on failure behavior was also studied. The results indicate that all these fibers fail in a brittle manner and failure normally starts from fiber surfaces. The failure loads observed in coated fibers are higher than those in bare fibers. The introduction of air holes reduces fiber strength and their geometrical arrangements have a remarkable effect on stress distribution in the longitudinal direction. These results are potentially useful for the design, fabrication and evaluation of optical fibers for a wide range of applications.

Export decisions within Taiwanese electrical and electronic SMEs : the role of management characteristics and attitudes

In this globalized environment, Taiwanese firms have been very successful in achieving growth via international market expansion. In particular, the Taiwanese electronics industry has shown a dynamism lacking in comparable industries around the world. However, in recent years there has been a move by many of the larger Taiwanese manufacturing firms to outsource their manufacturing to low-cost producers such as China in order to remain competitive. Conversely, most Taiwanese small- to medium-sized enterprises (SMEs) have retained their production facilities in Taiwan. These SMEs seek to expand their sales beyond the domestic market by employing an export strategy, making a significant socioeconomic contribution to the domestic and regional economies. This paper highlights the key dimensions such as enhancing factors (benefits/advantages), inhibiting factors (barriers/costs), and managerial factors (characteristics/commitment) that play an important role in the internationalization of SMEs located within the Taiwanese electronics industry. A logistic regression model is used to predict the probability of a firm being an exporter.

Developing a knowledge base for enterprise system success: the role of management and operational staff

Knowledge base is one of the emerging concepts in the Knowledge Management area. As there exists no agreed- upon standard definition of a knowledge base, this paper defines a knowledge base in terms of our research of Enterprise Systems (ES). The knowledge base is defined with reference to Learning Network Theory. Using this theoretical framework, we investigate the roles of management and operational staff in organisations and how their interactions can create a better ES-knowledge base to contribute to ES success. We focus on the post- implementation phase of ES as part of the ES lifecycle. Our findings will facilitate future research directions and contribute to better understandings of how the knowledge base can be integrated and how this integration leads to Enterprise System success.

An analysis of the qualitative characteristics of management commentary reporting by New Zealand companies

The narrative section of annual reports has considerable value to its user groups, such as financial analysts and investors (Tiexiera, 2004; Barlett and Chandler, 1997, IASB, 2006). This narrative section including chairpersons’/presidents’ statement contains twice the quantity of information than the financial statements section (Smith and Taffler, 2000). However, the abundance of information does not necessarily enhance the quality of such information (IASB, 2006). This issue of qualitative characteristics has been long foregone by researchers. This issue has attracted the attention of IASB (2006). Following the dearth in research in regard to qualitative characteristics of reporting this paper explores whether investors’ required qualitative characteristics as outlined by the IASB (2006) have been satisfied in management commentary section of New Zealand companies’ annual reports. Our result suggests that the principal stakeholders’, that is, investors’ qualitative characteristics requirements have been partially met in this section of annual reports. The qualitative characteristic of ‘relevance’ and ‘supportability’ have been satisfied in more annual reports compared to that of ‘balance’ and ‘comparability.’

Professional identity of management accountants : leadership in changing environments

The professional identity of management accountants (MAs) is evolving. According to 8,727 descriptors expressed by 1,158 participants, a range of characteristics of MAs are competing in shaping the identity of future MAs. Respondents strongly valued qualities such as professional principles, hard work, intelligence, analytical and forward thinking in MAs. Further, more innovative, dynamic and people-oriented qualities were strongly suggested for future MAs, with roles relating to multi-skilled business integrator, business partner/advisor, leader, change agent, and decision enabler/maker. Cultivating leadership qualities in the management accounting profession is critical according to participants. Projecting a positive image of the profession and CIMA, and innovative training in management and leadership skills can further support MAs to meet future challenges. Most of all, understanding business and continued personal development by individual MAs is highly valued in shaping the future leadership identity of MAs. Our quantitative data show positive relationships between the professional identification, image and reputation, and leadership qualities of MAs. This suggests that the more one identifies with the profession, the more one is likely to report higher levels of leadership qualities that support members to internalise the desired meaning of their profession and to create a positive image and reputation. After the financial crisis of 2008–2009, the image of financial professions has been tarnished and unpredictable markets and unstable employment opportunities have challenged the profession across all sectors. Many respondents, especially CIMA members, suggested that the turmoil of the financial crisis did not impact negatively but rather elevated the pivotal role of MAs in contributing to cost efficiency and value creation.