Aetiology of Papillon LeFevre Syndrome

Papillon LeFevre Syndrome, or PLS, was first described over 70 years ago. It is characterised by severe periodontal disease, typically leading to loss of teeth by adolescence, combined with palmoplantar hyperkeratosis. The fact that it is associated with consanguinity in particular ethnic groups suggests that genotype may contribute to the aetiology of this syndrome. Microbiological studies have been hampered by the rareness of the condition which makes prospective studies virtually impossible to perform. Numerous studies on small groups of patients, sometimes single cases, together suggest an association of recognised periodontal pathogens with PLS. Actinobacillus actinomycetemcomitans has been especially linked to PLS and raised levels of antibody to A.a. have been measured in some PLS patients, though not others. Porphyromonas gingivalis and Prevotella intermedia have also been detected in plaque samples from PLS, using monoclonal antibodies. Many other species have also been associated with PLS following culture and identification, as well as use of probes. Treatment has been attempted by eradication of periodontal pathogens so that teeth can erupt into a 'safe' environment. Successful treatment has needed intensive treatment and monitoring and good oral hygiene as well as thorough antibiotic therapy of patient, family members and even pets. Recently a Cathepsin C genotype has been strongly linked to PLS. However, this gene cannot account for all features of PLS and we can speculate that additional genes must be involved. It is concluded that PLS results from a combination of host and bacterial factors, including recessive human gene(s) associated with consanguinity, specific periodontal pathogens and lack of thorough oral hygiene. It is also believed that the human genetic component may merit examination as a 'host factor' in other bacterial infections. (C) 2001 Academic Press.

Wildlife-based tourism & increased support for nature conservation financially and otherwise: evidence from sea-turtle ecotourism at Mon Repos

The arguments of most conservationists supporting ecotourism have been based on the view that it is environmentally friendly as a resource-use and that receipts from it can counter demands to use the natural resources involved for more extractive economic purposes. But wildlife-based ecotourism can also have positive impacts in itself on the willingness of tourists to pay for wildlife conservation, strengthen the pro-conservation attitudes of tourists, and foster personal actions by them that contribute to wildlife conservation. These aspects are explored in this article on the basis of a survey of tourists visiting Mon Repos Beach near Bundaberg, Queensland, for the purpose of watching marine turtles. The results enable several of the conservation impacts of this experience on tourists to be quantified, and highlight important relationships between specific socio-economic variables and the willingness of tourists to pay for the protection of sea turtles. Furthermore, it is shown that the on-site experiences of ecotourists have positive impacts on the willingness of tourists to pay for the conservation of wildlife, and that willingness to pay is sensitive to whether or not wildlife is seen. It is suggested that in situ ecotourism is likely to be a more powerful force for fostering pro-conservation attitudes and actions among visitors than ex situ wildlife-based tourism in aquaria and zoos.

Masculine, feminine or neutral? In-company equal opportunities policies in Irish and French MNC retailing

Several problematic aspects of women's paid employment - e.g. low pay and lack of promotional opportunities - are exacerbated by the segregation of women and men into different occupations. In this article, the potential of in-store equal opportunities policies to break down such gender segregation will be explored, through consideration of the existence and implementation of these policies in twenty-two multinational retail companies in Dublin and Paris. It will be argued that, with one notable exception, the instore equal opportunities policies are effectively neutralized, and furthermore are neutralized in nationally specific ways which can be related to differences between France and Ireland in the organization of labour-market regulation and in women's labour-force participation (LFP). The case-study findings also suggest that the 'country' variable has a stronger effect on the existence and implementation of these policies than the extent of a shop's links to an overseas headquarters. The findings of this study have implications for both the equity of women's incorporation into the paid labour force and understanding of aspects of HRM in branches of multinational companies.

A segmentation-based and partial-volume-compensated method for an accurate measurement of lateral ventricular volumes on T-1-weighted magnetic resonance images

Lateral ventricular volumes based on segmented brain MR images can be significantly underestimated if partial volume effects are not considered. This is because a group of voxels in the neighborhood of lateral ventricles is often mis-classified as gray matter voxels due to partial volume effects. This group of voxels is actually a mixture of ventricular cerebro-spinal fluid and the white matter and therefore, a portion of it should be included as part of the lateral ventricular structure. In this note, we describe an automated method for the measurement of lateral ventricular volumes on segmented brain MR images. Image segmentation was carried in combination of intensity correction and thresholding. The method is featured with a procedure for addressing mis-classified voxels in the surrounding of lateral ventricles. A detailed analysis showed that lateral ventricular volumes could be underestimated by 10 to 30% depending upon the size of the lateral ventricular structure, if mis-classified voxels were not included. Validation of the method was done through comparison with the averaged manually traced volumes. Finally, the merit of the method is demonstrated in the evaluation of the rate of lateral ventricular enlargement. (C) 2001 Elsevier Science Inc. All rights reserved.

Cash, customers, and care: The experience and meaning of differential payment for high care places in aged care facilities

The introduction of new asset/income tested charges for high care residents was the 1997-98 Commonwealth government policy response to concerns about financing residential aged care. This in-depth study of residents, families, staff and managers in three aged care facilities explores issues of equity, access and empowerment arising when some residents pay more for the same level of care and amenity. The study reports little evidence of financial contributions affecting access to high care places and the delivery of care, the potential for differential access to amenities such as single rooms linked to the extra payments, and no evidence of a sense of empowerment linked to payment of the new charges. The complexity of current financial arrangements, access to appropriate financial advice at the time of entry, and the potential for an informal two tier system in relation to the allocation Of amenities are identified as developing policy issues.

Longitudinal study of veterinarians from entry to the veterinary course to 10 years after graduation: attitudes to work, career and profession

Objective To describe the attitudes of veterinarians to their work, career and profession during the 10 years after graduation. Design Longitudinal study of students who started their course at The University of Queensland in 1985 and 1986, and who completed questionnaires in their first and fifth year as students, and after one, five and 10 years as veterinarians. Methods Data from 129 (96%) questionnaires completed after 10 years as a veterinarian were coded numerically then analysed, together with data from previous questionnaires, with SAS System 7 for Windows 95. Results After 10 years, almost all respondents were either very glad they had done the veterinary course (57%) or generally glad, though with some misgivings (37%). Despite this, only 55% would definitely become a veterinarian if they 'had to do it over again'. The responses for about one-third were different from those given five years earlier. The views of many were related to the level of support and encouragement received in their first job after graduation. There were 42% who were working less than half-time as veterinarians, and their main reasons were, in order, raising children, long hours of work, attitudes of bosses and clients, and poor pay. A majority was concerned about the ethics and competence of some colleagues, and almost all believed that consideration of costs must influence the type of treatment animals receive. Conclusions Most veterinarians were glad to have done the veterinary course, but for about one-quarter their career had not lived up to expectations and almost half would not do it again in another incarnation. Stress, hours of work, difficulties in balancing personal life with career and low income were important concerns for many. Low income may contribute to the low number of males entering the veterinary profession.

The effect of sodium chloride supplementation on the milk production of grazing Holstein Friesian cows during summer and autumn in a humid sub-tropical environment

Two studies were conducted to examine the effects of including NaCl at various rates in grain-based supplements for Friesian cows grazing established, dominant (>90%), rainfed kikuyu (Pennisetum clandestinum cv. Common) pastures during summer and autumn in a humid sub-tropical environment. In study 1 (19 January-27 March 1998), 48 cows (36 multiparous, 12 primiparous; 27-96 days postpartum) were allocated to one of four groups based on genetic merit, milk production, liveweight (LW) and days postpartum. They were fed (2.7 kg dry matter (DM) per cow, twice-a-day) one of four isoenergetic and isonitrogenous barley grain-based concentrates containing NaCl at concentrations (% as-fed) of either 0 (SC1), 1.1 (SC2), 2.2 (SC3) or 3.3 (SC4). Maximum temperature humidity index (THImax) was greater than or equal to78 during 50% of the experimental period. Concentrate NaCl content had no effect (P>0.05) on daily milk yield or LW change but daily yields of 4% fat corrected milk (FCM), fat and protein were higher (P0.05) among treatments at 7.6+/-1.24 kg DM per cow. In study 2 (18 January 1999-1 March 1999), 48 cows (32 pluriparous, 16 primiparous: 32-160 days postpartum) were fed (2.7 kg DM per cow twice-a-day) one of two isoenergetic and isonitrogenous barley grain-based concentrates containing NaCl at concentrations (% as-fed) of 0 (control) or 2.2 (HSC). THImax was greater than or equal to78 during 34% of days in the experimental period. Yields of milk, FCM, fat and protein were lower (P0.05) by concentrate NaCl content. These studies indicate that NaCl supplementation can be beneficial in terms of milk production during warm, humid conditions as opposed to milder conditions. (C) 2002 Elsevier Science B.V. All rights reserved.

Complete mitochondrial genomes confirm the distinctiveness of the horse-dog and sheep-dog strains of Echinococcus granulosus

Unlike other members of the genus, Echinococcus granulosus is known to exhibit considerable levels of variation in biology, physiology and molecular genetics. Indeed, some of the taxa regarded as 'genotypes' within E. granulosus might be sufficiently distinct as to merit specific status. Here, complete mitochondrial genomes are presented of 2 genotypes of E. granulosus (G1-sheep-dog strain: G4-horse-dog strain) and of another taeniid cestode, Taenia crassiceps. These genomes are characterized and compared with those of Echinococcus multilocularis and Hymenolepis diminuta. Genomes of all the species are very similar in structure, length and base-composition. Pairwise comparisons of concatenated protein-coding genes indicate that the G1 and G4 genotypes of E. granulosus are almost as distant from each other as each is from a distinct species, E. multilocularis. Sequences for the variable genes atp6 and nad3 were obtained from additional genotypes of E. granulosus, from E. vogeli and E. oligarthrus. Again, pairwise comparisons showed the distinctiveness of the G1 and G4 genotypes. Phylogenetic analyses of concatenated atp6, nad1 (partial) and cox1 (partial) genes from E. multilocularis, E. vogeli, E. oligarthrus, 5 genotypes of E. granulosus, and using T. crassiceps as an outgroup, yielded the same results. We conclude that the sheep-dog and horse-dog strains of E. granulosus should be regarded as distinct at the specific level.

A estrat??gia de Lisboa e as suas implica????es para a reforma institucional da Uni??o Europ??ia

Como manter a estrat??gia europ??ia de desenvolvimento atualizada, e ?? luz dos valores europeus, para reagir aos novos desafios decorrentes da globaliza????o, da mudan??a tecnol??gica e do envelhecimento das popula????es? Um novo paradigma emerge, em que o conhecimento e a inova????o s??o as principais fontes de riqueza e tamb??m de diferen??as entre as na????es, as empresas e as pessoas. Era preciso definir a via europ??ia de transi????o para essa economia baseada na inova????o e no conhecimento, com marcas distintas que v??o da salvaguarda da coes??o social e da diversidade cultural ??s pr??prias escolhas tecnol??gicas. Criar uma nova plataforma competitiva ?? fundamental para sustentar o modelo social europeu, que tamb??m dever?? ser renovado. Este artigo nos traz o debate dos pa??ses membros da Uni??o Europ??ia que precedeu a Cimeira de Lisboa (23 e 24 de Mar??o de 2000).

Levantamento te??rico sobre as causas dos insucessos das tentativas de reforma administrativa

O presente artigo tem como objetivo identificar e analisar os principais motivos apontados na literatura pertinente como causadores dos sucessivos e reiterados fracassos das tentativas de reforma da administra????o p??blica brasileira. As referidas tentativas foram empreendidas em 1938, quando da cria????o do Departamento Administrativo do Servi??o P??blico (DASP), durante a ditadura do Estado Novo; em 1963, quando Ernani do Amaral Peixoto foi designado ministro extraordin??rio para a Reforma Administrativa; em 1967, quando da edi????o do Decreto-Lei n?? 200, de 25/2/1967 e, tamb??m, em 1986, 1990 e 1995, nos Governos Sarney, Collor e Cardoso, respectivamente. Todas elas visavam, basicamente, a implantar a meritocracia na administra????o p??blica brasileira. Segundo os autores consultados, essas tentativas foram malsucedidas devido aos seguintes fatores: a quest??o da depend??ncia da trajet??ria, as caracter??sticas das organiza????es que dificultam a ocorr??ncia de altera????es institucionais, os elementos de natureza sociol??gica, o fato de a reforma administrativa ser bem p??blico sujeito a problemas de a????o coletiva e, finalmente, as peculiaridades do sistema pol??tico brasileiro.

Crise e reforma do Estado: uma quest??o de cidadania e valoriza????o do servidor

O artigo discute a evolu????o recente da problem??tica da crise do Estado a partir dos tr??s movimentos voltados para a sua supera????o: a busca da efici??ncia, a melhoria da qualidade e o resgate da esfera p??blica como instrumento do exerc??cio da cidadania. Em seguida s??o analisadas as limita????es impostas pelo modelo burocr??tico de administra????o p??blica e apresentadas as experi??ncias recentes de reforma nos EUA, Gr??-Bretanha, Fran??a e Brasil. S??o tamb??m avaliados os impactos do novo modelo de gest??o p??blica nas ??reas de planejamento, controle e desenvolvimento de pessoal. Finalmente o artigo discute o contexto de mudan??a e transforma????o do Estado destacando a necessidade de vincular os conceitos de governabilidade e governan??a aos de cidadania e valoriza????o do servidor p??blico.

Meritocracia ?? brasileira: o que ?? desempenho no Brasil?

O artigo trata da quest??o da meritocracia e da avalia????o de desempenho no setor p??blico brasileiro e na sociedade sob uma perspectiva mais ampla, enfatizando a diferen??a entre a exist??ncia de sistemas meritocr??ticos formais e sua legitimidade social. Para a autora, o cerne desta discuss??o passa pelo entendimento da diferen??a entre sistemas meritocr??ticos e ideologia da meritocracia e pela compreens??o dos pressupostos culturais que est??o informando este debate. O artigo situa a quest??o da meritocracia em contextos mais amplos, como a teoria da administra????o, a perspectiva intercultural e a ??tica hist??ricosociol??gica, sem perder de vista as especificidades da sociedade brasileira.

Fortalecer o servi??o p??blico e valorizar a cidadania: a op????o australiana

O artigo analisa o processo de reforma administrativa empreendida na Austr??lia ao longo das d??cadas de 70-80, destacando duas de suas principais tend??ncias: o fortalecimento da ???Fun????o Executiva Superior???e a valoriza????o da cidadania. Nele s??o descritos os princ??pios b??sicos norteadores da reforma ao longo dos anos 70, bem como, os objetivos visados com a segunda fase das reformas executadas a partir da d??cada de 80, implementadas por governos trabalhistas. Destaca-se a organiza????o e o funcionamento do sistema das Ag??ncias de Ombudsmen e dos Comiss??rios de Contas respons??veis pela fiscaliza????o e gest??o or??ament??ria. A seguir, s??o descritas algumas das a????es encaminhadas pela Public Service Commission (PSC) no sentido de aperfei??oar o gerenciamento e o melhor desempenho dos recursos humanos, destacando a introdu????o do importante conceito de Novo Profissionalismo, assentado em valores como responsabilidade para com o governo, foco nos resultados, m??rito como valor b??sico, altos n??veis de integridade e probidade na conduta, desempenho com accountability e melhoramento cont??nuo do desempenho das equipes e indiv??duos. Outro importante instrumento analisado ?? o Human Resource Management, atrav??s do qual as autoridades australianas introduziram novas pr??ticas como negocia????o no local de trabalho, plano de oportunidades igualit??rias no local de trabalho e o plano de treinamento e especializa????o. Para finalizar, o artigo enfoca a pol??tica de contratualiza????o de servi??os p??blicos adotada na Austr??lia ??? os Competitive Tendering and Contracting, evidenciando os fatores indutores da ado????o desta nova pr??tica de gest??o.

Reforma administrativa: o caso do Reino Unido

O artigo apresenta uma an??lise das principais caracter??sticas da reforma administrativa empreendida no Reino Unido a partir da do primeiro governo Thatcher em 1979. Inicialmente, s??o descritos dois aspectos peculiares que, segundo os autores, explicam a intensidade das reformas administrativas a?? empreendidas: seu sistema pol??tico, no qual sobressai sobremaneira a alta capacidade decis??ria do Executivo, e as debilidades de seu sistema administrativo, alvo de cr??ticas reiteradas desde o Relat??rio do Comit?? Fulton, publicado em 1968. A partir disso, os autores descrevem tr??s fases recentes na reforma administrativa inglesa p??s-Thatcher. Nesta descri????o, s??o enfocadas as principais caracter??sticas e experi??ncias inovadoras adotadas, enfatizando, entre outros: 1) os chamados ???escrut??neos de Rayner???; 2) os sistemas de informa????es gerenciais adotados (Management Information System for Ministers e o Management Accounting System); 3) o programa Citizen???s Charter; 4) o processo de privatiza????o ingl??s; 5) a experi??ncia de contrata????o externa de servi??os (com a ado????o de instrumentos como a ???licita????o competitiva???, que permite aos pr??prios servidores p??blicos apresentarem propostas para presta????o de servi??os em competi????o com as empresas privadas, al??m dos sistemas Market Testing e Competing for Quality) e, por fim; 6) a pol??tica de gest??o de recursos humanos, com destaque para o forte processo de demiss??es no servi??o p??blico, o sistema de avalia????o de desempenho dos funcion??rios e de remunera????o por performance adotados no Reino Unido.

As reformas administrativas no Brasil: modelos, sucessos e fracassos

O artigo rev??, com base na bibliografia especializada, as reformas e tentativas de reformas administrativas realizadas no pa??s, desde o Estado Novo, passando pelas tentativas dos governos militares e pelas propostas mais recentes da Nova Rep??blica, e chegando at?? a reforma Bresser Pereira. O balan??o cr??tico ?? bastante negativo, sobretudo, porque as grandes reformas, precisamente pela sua abrang??ncia, nunca foram efetivamente implementadas. O sistema do m??rito no servi??o p??blico federal ?? um bom exemplo. As dificuldades de implementa????o das grandes reformas, advindas principalmente de sua grande complexidade, resultam tamb??m de sua intera????o negativa com o contexto pol??tico mais geral, pela carga ret??rica de que se fazem acompanhar, e pelo fato de subestimarem os aspectos t??cnicos necess??rios ?? sua implementa????o.