Familial hyperaldosteronism type II: Description of a large kindred and exclusion of the aldosterone synthase (CYP11B2) gene

Familial hyperaldosteronism type II (FH-II) is characterized by autosomal dominant inheritance and hypersecretion of aldosterone due to adrenocortical hyperplasia or an aldosterone-producing adenoma; unlike FH type I (FH-I), hyperaldosteronism in FH-II is not suppressible by dexamethasone. Of a total of 17 FH-II families with 44 affected members, we studied a large kindred with 7 affected members that was informative for linkage analysis. Family members were screened with the aldosterone/PRA ratio test; patients with aldosterone/PRA ratio greater than 25 underwent fludrocortisone/salt suppression testing for confirmation of autonomous aldosterone secretion. Postural testing, adrenal gland imaging, and adrenal venous sampling were also performed. Individuals affected by FH-II demonstrated lack of suppression of plasma A levels after 4 days of dexamethasone treatment (0.5 mg every 6 h). All patients had neg ative genetic testing for the defect associated with FH-I, the CYP11B1/CYP11B2 hybrid gene. Genetic linkage was then examined between FH-II and aldosterone synthase (the CYP11B2 gene) on chromosome 8q. A polyadenylase repeat within the 5'-region of the CYP11B2 gene and 9 other markers covering an approximately 80-centimorgan area on chromosome 8q21-8qtel were genotyped and analyzed for linkage. Two-point logarithm of odds scores were negative and ranged from -12.6 for the CYP11B2 polymorphic marker to -0.98 for the D8S527 marker at a recombination distance (theta) of 0. Multipoint logarithm of odds score analysis confirmed the exclusion of the chromosome 8q21-8qtel area as a region harboring the candidate gene for FH-II in this family. We conclude that FH-II shares autosomal dominant inheritance and hyperaldosteronism with FH-I, but, as demonstrated by the large kindred investigated in this report, it is clinically and genetically distinct. Linkage analysis demonstrated that the CYP11B2 gene is not responsible for FH-II in this family; furthermore, chromosome 8q21-8qtel most likely does not harbor the genetic defect in this kindred.

The effects of the Cannabis Expiation Notice System on the prevalence of cannabis use in South Australia: evidence from the National Drug Strategy Household Surveys 1985-95

This study sought to examine the impact of the Cannabis Expiation Notice (CEN) scheme on the prevalence of lifetime and weekly cannabis use in South Australia. Data from five National Drug Strategy Household Surveys between 1985 and 1995 were examined to test for differences in trends in self-reported: (1) lifetime cannabis use; and (2) current weekly cannabis use, after controlling for age and gender, between South Australia and the other states and territories. Between 1985 and 1995, rates of lifetime cannabis use increased in SA from 26% to 36%. There were also significant increases in Victoria (from 26% to 32%), Tasmania (from 21% to 33%) and New South Wales (from 26% to 33%). The increase in South Australia was significantly greater than the average increase throughout the rest of Australia, but the other Australian states differed in their rates of change. Victoria and Tasmania had similar rates of increase to South Australia; New South Wales, Queensland and Western Australia showed lower rates of increase; and the Northern Territory and the Australian Capital Territory had high rates that did not change during the period. There was no statistically significant difference between SA and the rest of Australia in the rate of increase in weekly cannabis use. While there was a greater increase in self- reported lifetime cannabis use in South Australia between 1985 and 1995 than in the average of the other Australian jurisdictions it is unlikely that this increase is due to the CEN system, because similar increases occurred in Tasmania and Victoria (where there was no change in the legal status of cannabis use), and there was no increase in the rate of weekly cannabis use in South Australia over the same period.

AGE DIFFERENCES IN THE PREVALENCE AND CO-MORBIDITY OF DSM-IV MAJOR DEPRESSIVE EPISODES: RESULTS FROM THE WHO WORLD MENTAL HEALTH SURVEY INITIATIVE

Background: Although depression appears to decrease in late life, this could be due to misattribution of depressive symptoms to physical disorders that increase in late we. Methods: We investigated this issue by studying age differences in co-morbidity of DSM-IV major depressive episodes (MDE) with chronic physical conditions in the WHO World Mental Health (WMH) surveys, a series of community epidemiological surveys of respondents in 10 developed countries (n = 52,485) and 8 developing countries (n = 37,265). MDE and other mental disorders were assessed with the Composite International Diagnostic Interview (CIDI). Organic exclusion rules were not used to avoid inappropriate exclusion of cases with physical co-morbidity. Physical conditions were assessed with a standard chronic conditions checklist. Results: Twelve-month DSM-IV/CIDI MDE was significantly less prevalent among respondents ages 65+ than younger respondents in developed but not developing countries. Prevalence of co-morbid mental disorders generally either decreased or remained stable with age, while co-morbidity of MDE with mental disorders generally increased with age. Prevalence of physical conditions, in comparison, generally increased with age, while co-morbidity of MDE with physical conditions generally decreased with age. Depression treatment was lowest among the elderly in developed and developing countries. Conclusions: The weakening associations between MDE and physical conditions with increasing age argue against the suggestion that the low estimated prevalence of MDE among the elderly is due to increased confounding with physical disorders. Future study is needed to investigate processes that might lead to a decreasing impact of physical illness on depression among the elderly. Depression and Anxiety 27:351-364, 2010. (C) 2009 Wiley-Liss, Inc.

Fighting climate change in the air: lessons from the EU directive on global aviation

The European Union's (EU) decision to include aviation into the Emissions Trade Scheme was heatedly contested. Countries around the world, but mainly the Brazil, Russia, India, China and South Africa group (BRICS) and the US, denounced the EU's initiate as illegal and unilateral. Following a decade of frustrated negotiations at the International Civil Aviation Organization (ICAO), this paper interrogates why such measure, in principle climate-friendly, inspired so much global resentment. I argue that concerns with competitiveness and risks of legal inconsistency are important, but insufficient elements to explain the core of the conflict. The paper suggests that the EU was strongly criticized because third countries perceived this action as an imposed solution, which fostered an environment of distrust. Therefore, I claim that the problem has more to do with a normative divide than with a substantive divergence on what should be done regarding aviation emissions. My analysis is informed by the present literature on the links between trade and climate change, but gives particular weight to first-hand information through interviews with key stakeholders. The paper is divided in three parts. First, it presents the scope of the EU directive in historical perspective. Second, it explores the EU's measure through three different angles: legal, economical and political. The final part explores some possible solutions to overcome these divergences.

Reflections on the legal framework for relations between cooperatives and subsidiaries.

In the light of Portuguese legal system, cooperative enterprises may include an enterprise carried out by a subsidiary, provided they conform to certain requirements. The aim of this paper is to reflect on the issue of the legal framework of the relationship between the cooperative and the subsidiary. There are several problems to be addressed in this paper: (i) How to qualify such a relationship since corresponding to mere investments made by the cooperative? Should it be classified as non-member cooperative transactions or as extraordinary activities? (ii) How to qualify such a relationship when related to the development of preparatory or complementary activities for the economic activity developed between the cooperative and its members? May we speak, in this situation, of a concept of “indirect mutuality”, as provided in other legal systems? (iii) How should we classify and what is the regime of the economic results from the activity developed by the subsidiary? We will conclude, advocating: (i) That the cooperative enterprise may include an enterprise carried out by a subsidiary if this is deemed necessary to satisfy the interests of the members; (ii) The inadmissibility of the concept of “indirect mutuality”; (iii) The inadequacy of qualifying the legal relationship between the cooperative partner (iv) The application, to the economic results coming from the activity developed by the subsidiary, of the regime provided for in the Portuguese Cooperative Code to the results from non-member cooperative transactions; (v) The economic results coming from the activity developed by the subsidiary cannot be appropriated by individual co-operators members, and so should be allocated to indivisible reserves.

Impact of taxes on competition: the legal status quo in the European Union

Dissertação de mestrado em Direito dos Negócios, Europeu e Transnacional

Nutritional composition, quality and spoilage capacity of orange roughy (Hoplostethus atlanticus) from the North East Atlantic

The nutritional composition o f orange roughy (collected from the Northeast Atlantic near the Rockall Trough) was studied on a seasonal basis. In addition samples were aged and stability assessed. Protein levels (16.68-16.21% w/w) were found to be slightly higher than those recorded for the N ew Zealand species o f orange roughy and compared favourably with protein values for fish muscle in general. Statistically results show a significant seasonal variation with no variation from fish to fish or in the location within the fish. Lipid content (3.6-4.5% w/w) was found to be much lower than that recorded for New Zealand. As with protein statistically results show a significant seasonal variation and no variation from fish to fish or in the location within the fish. Moisture levels (77.3_79.6%w/w) compared favourably with values obtained from other studies. Again statistically results show a significant seasonal variation with no variation from fish to fish or within the fish. Iodine values (74.63-79.54) indicate the likely presence o f a high level o f mono unsaturated fatty acids. Statistically results show no significant seasonal variation and no sample variation or variation within fish. Thin layer chromatography o f the extracted fat showed the major type to be wax esters with a much lower amount o f triglycerides and smaller amounts of polar lipids, free sterols and free fatty acids. Total fatty acid composition was found to be very similar to that recorded from other studies and showed that most o f the oils extracted from the fish muscle contained a high percentage o f mono unsaturates namely 16:1,18:1, 20:1 and 22:1 (85.63 - 91.14% ) with 16:1 present in the smallest amounts and 18:1 the major one. The only saturated fatty M.Sc. in Biochemistry III Nutritional Composition, Quality and Spoilage Capacity of Specific Deep Sea Fish acids present in significant quantities were 14:0, 16:0 and 18:0, the total varied from a seasonal average high o f 4.05 % to an average low o f 2.27%. The polyunsaturated fatty acids linoleic and arachidonic acid were present in small quantities varying in total from 0.89% to 1.50%. Docosapentaenoic acid (D P A ) was found only in trace quantities in spring, autumn and winter samples and undetected in summer. Levels o f Eicosapentaenoic acid (EPA ) and Docosahexaenoic acid (D H A ) were also found in very low percentages and varied on a seasonal basis with average values ranging from 0.41% in summer to 1.03 % in autumn for EPA and from 1.44 % in summer to3.20 % in autumn for D H A . Again statistically results show a significant seasonal variation with no variation from fish to fish or location within the fish. Levels o f freshness were measured using the Thiobarbituric acid (T B A ), Total volatile base nitrogen (T V B -N ) and Trimethylamine (T M A ) techniques. The quality o f the fish upon arrival was excellent and well below legal/acceptable lim its.T V B -N values ranged from 6.88-8.91 mg/lOOg and T M A values from 4.82-6.46 mg/lOOg Values for T B A ranged from 0.18-0.35 mg Malonaldehyde/kg fish. The summer values were higher than the other seasons. Seasonal variation was significant for all methods with no variation from fish to fish or within the fish. Fish aged at +4°C in air did not exceed the T V B N lim it o f 35mg/100g until day 6 whereas the T V B N lim it was extended to 8 days for fish aged at +4°C in vacuum. However the T M A lim it o f 12mg/100g was reached on day 4 for fish stored at +4°C in air and on day 5 for vacuum packed samples stored at +4°C . Fish stored at -5°C in air and vacuum packed did not reach the T V B N lim it until day 61 but the T M A limit was reached on day 24 for fish stored at -5°C in air and was extended to 31 days for vacuum packed fish stored at-5°C. Prolonged storage at -18°C caused some deterioration o f the frozen fish muscle. Upon thawing the shelf life o f fish stored for 12 months was much shorter than that stored for 6 M.Sc. in Biochemistry IV Nutritional Composition, Quality and Spoilage Capacity of Specific Deep Sea Fish months. This in turn deteriorated faster than fresh fish held at refridgeration temperature in air. Orange roughy were found to be a good source of protein with moisture levels similar to that o f other fish. They were o f medium fat content but have a very poor content o f the essential omega 3 and omega 6 fatty acids. Orange roughy can be stored at -18°C but its subsequent refridgerated shelf life will be shorter than that o f unfrozen orange roughy stored at refridgeration temperature. Orange roughy are a very important part o f the ecosystem. Their composition is less nutritionally beneficial than more readily available fish for human consumption and therefore should not be fished at all

β-Glucan antigenemia assay for the diagnosis of invasive fungal infections in patients with hematological malignancies: a systematic review and meta-analysis of cohort studies from the Third European Conference on Infections in Leukemia (ECIL-3).

BACKGROUND: Invasive fungal infections (IFIs) are life-threatening complications in patients with hemato-oncological malignancies, and early diagnosis is crucial for outcome. The compound 1,3-β-D-glucan (BG), a cell wall component of most fungal species, can be detected in blood during IFI. Four commercial BG antigenemia assays are available (Fungitell, Fungitec-G, Wako, and Maruha). This meta-analysis from the Third European Conference on Infections in Leukemia (ECIL-3) assessed the performance of BG assays for the diagnosis of IFI in hemato-oncological patients. METHODS: Studies reporting the performance of BG antigenemia assays for the diagnosis of IFI (European Organization for Research and Treatment of Cancer and Mycoses Study Group criteria) in hemato-oncological patients were identified. The analysis was focused on high-quality cohort studies with exclusion of case-control studies. Meta-analysis was performed by conventional meta-analytical pooling and bivariate analysis. RESULTS: Six cohort studies were included (1771 adult patients with 414 IFIs of which 215 were proven or probable). Similar performance was observed among the different BG assays. For the cutoff recommended by the manufacturer, the diagnostic performance of the BG assay in proven or probable IFI was better with 2 consecutive positive test results (diagnostic odds ratio for 2 consecutive vs one single positive results, 111.8 [95% confidence interval {CI}, 38.6-324.1] vs 16.3 [95% CI, 6.5-40.8], respectively; heterogeneity index for 2 consecutive vs one single positive results, 0% vs 72.6%, respectively). For 2 consecutive tests, sensitivity and specificity were 49.6% (95% CI, 34.0%-65.3%) and 98.9% (95% CI, 97.4%-99.5%), respectively. Estimated positive and negative predictive values for an IFI prevalence of 10% were 83.5% and 94.6%, respectively. CONCLUSIONS: Different BG assays have similar accuracy for the diagnosis of IFI in hemato-oncological patients. Two consecutive positive antigenemia assays have very high specificity, positive predictive value, and negative predictive value. Because sensitivity is low, the test needs to be combined with clinical, radiological, and microbiological findings.

Sex, politics and social change in the eighteenth and the nineteenth centuries: Evidence from the Swiss Alps

The 18th century "sexual revolution" can not simply be explained as a consequence of economic or institutional factors - industrialization, agricultural revolution, secularization or legal hindrances to marriages: the example of western Valais (Switzerland) shows that we have to deal with a complex configuration of factors The micro-historical approach reveals that in the 18th and 19th century sexuality - and above all illicit sexuality - was a highly subversive force which was considerably linked to political innovation and probably more generally to historical change. Non-marital sexuality was clearly tied to political dissent ant to innovative ways of behaviour, both among the social elites and the common people. This behaviour patterns influenced crucial evolutions in the social, cultural and economic history of the region.

Description of Pintomyia (Pifanomyia) brazilorum sp. nov. a new fossil species from the Dominican Republic (Diptera: Psychodidae: Phlebotominae)

A sand fly fossil was found in amber, a vegetal resin, which allows all the external phlebotomine structures to be seen. The piece that contains the new species is 14 mm long ´ 8 mm wide ´ 3 mm high. All the structures from the head, thorax, and abdomen were examined under the microscope and measured with a calibrated micrometric eye-piece. The morphological aspects of the new species suggest its inclusion in the Pintomyia genus, Pifanomyia subgenus though it is not possible to include it in any of the series known for this subgenus. The presence of two atrophied spines on the gonostyles and gonocoxites without tufts of setae permit the exclusion of the new species from the other species of the subgenus Pifanomyia. The new species is named Pintomyia (Pifanomyia) brazilorum sp. nov.

Design effect and interviewer effects: Evidence from the Spanish ESS3-2006 Data

In 2007 the first Quality Enhancement Meeting on sampling in the European SocialSurvey (ESS) took place. The discussion focused on design effects and inteviewereffects in face-to-face interviews. Following the recomendations of this meeting theSpanish ESS team studied the impact of interviewers as a new element in the designeffect in the response s variance using the information of the correspondent SampleDesign Data Files. Hierarchical multilevel and cross-classified multilevel analysis areconducted in order to estimate the amount of responses variation due to PSU and tointerviewers for different questions in the survey. Factor such as the age of theinterviewer, gender, workload, training and experience and respondent characteristicssuch as age, gender, renuance to participate and their possible interactions are alsoincluded in the analysis of some specific questions like trust in politicians and trustin legal system . Some recomendations related to future sampling designs and thecontents of the briefing sessions are derived from this initial research.

Wireless privacy and personalized location-based services: the challenge of translating the legal framework into business practices

L'avancement des communications sans-fil permet l'obtention de nouveaux services bases sur l'habileté des fournisseurs de services sans-fil à déterminer avec précision, et avec l'utilisation de technologies de pistage, la localisation et position géographiquement d'appareils sans-fil Cette habileté permet d'offrir aux utilisateurs de sans-fil de nouveaux services bases sur la localisation et la position géographique de leur appareil. Le développement des services basés sur la localisation des utilisateurs de sans-fil soulevé certains problèmes relatifs à la protection de la vie privée qui doivent être considérés. En effet, l'appareil sans-fil qui suit et enregistre les mouvements de I 'utilisateur permet un système qui enregistre et entrepose tous les mouvements et activités d'un tel utilisateur ou encore qui permet l'envoi de messages non anticipes à ce dernier. Pour ce motif et afin de protéger la vie privée des utilisateurs de sans-fil, une compagnie désirant développer ou déployer une technologie permettant d'offrir ce genre de services personnalisés devra analyser l'encadrement légal touchant la protection des données personnelles--lequel est dans certains cas vague et non approprié à ce nouveau contexte--ainsi que la position de l'industrie dans ce domaine, et ce, afin d'être en mesure de traduire cet encadrement en pratiques commerciales. Cette analyse permettra d'éclairer le fournisseur de ces services sur la façon d'établir son modèle d'affaires et sur le type de technologie à développer afin d'être en mesure de remédier aux nouveaux problèmes touchant la vie privée tout en offrant ces nouveaux services aux utilisateurs de sans-fil.

A Comprehensive Analysis of the Legal Issues Relating to Nominee Directors

Dans la foulée des scandales financiers ayant secoué le milieu des affaires ces dernières années, l’efficacité des pratiques de régie d’entreprise, et, en particulier celles liées à l’indépendance des administrateurs, a été passée au crible. L’administrateur désigné par une partie pour la représenter est un type d’administrateur que l’on rencontre fréquemment au sein des conseils d’administration des entreprises. Toutefois, l’on peut se questionner sur l’indépendance réelle de ces administrateurs, considérant leur loyauté envers la personne les ayant désignés, laquelle détient habituellement un intérêt à titre d’actionnaire ou de partie prenante dans l’entreprise visée. En outre, alors que les principes légaux requièrent que les administrateurs agissent dans le meilleur intérêt de l’entreprise, la réalité pratique est parfois toute autre: aux prises avec les instructions ou les souhaits de la personne les ayant nommés, les administrateurs désignés se retrouvent placés en situation inhérente de conflit d’intérêts. Ce texte vise à offrir une analyse détaillée au sujet de l’administrateur désigné et du conflit d’intérêts résultant de cette double exigence de loyauté. L’objectif est de présenter un examen approfondi des diverses difficultés résultant de la nomination d’un administrateur désigné ou associées à celle-ci, ainsi que des réponses judiciaires et législatives liées à cette problématique. Cette réflexion mènera à une exploration de certains systèmes législatifs et légaux, en particulier ceux du Royaume-Uni, de l’Australie et de la Nouvelle-Zélande, afin d’obtenir une meilleure compréhension et d’offrir une perspective éclairée quant aux enjeux analysés par la présente.

The Legal Implications of Internet Marketing : Exploiting the Digital Marketplace Within the Boundaries of the Law

Au cours des dernières années, le domaine de la consommation a grandement évolué. Les agents de marketing ont commencé à utiliser l’Internet pour influencer les consommateurs en employant des tactiques originales et imaginatives qui ont rendus possible l’atteinte d'un niveau de communication interpersonnelle qui avait précédemment été insondable. Leurs interactions avec les consommateurs, en utilisant la technologie moderne, se manifeste sous plusieurs formes différentes qui sont toutes accompagnés de leur propre assortiment de problèmes juridiques. D’abord, il n'est pas rare pour les agents de marketing d’utiliser des outils qui leur permettent de suivre les actions des consommateurs dans le monde virtuel ainsi que dans le monde physique. Les renseignements personnels recueillis d'une telle manière sont souvent utilisés à des fins de publicité comportementale en ligne – une utilisation qui ne respecte pas toujours les limites du droit à la vie privée. Il est également devenu assez commun pour les agents de marketing d’utiliser les médias sociaux afin de converser avec les consommateurs. Ces forums ont aussi servi à la commission d’actes anticoncurrentiels, ainsi qu’à la diffusion de publicités fausses et trompeuses – deux pratiques qui sont interdites tant par la loi sur la concurrence que la loi sur la protection des consommateurs. Enfin, les agents de marketing utilisent diverses tactiques afin de joindre les consommateurs plus efficacement en utilisant diverses tactiques qui les rendent plus visible dans les moteurs de recherche sur Internet, dont certaines sont considérés comme malhonnêtes et pourraient présenter des problèmes dans les domaines du droit de la concurrence et du droit des marques de commerce. Ce mémoire offre une description détaillée des outils utilisés à des fins de marketing sur Internet, ainsi que de la manière dont ils sont utilisés. Il illustre par ailleurs les problèmes juridiques qui peuvent survenir à la suite de leur utilisation et définit le cadre législatif régissant l’utilisation de ces outils par les agents de marketing, pour enfin démontrer que les lois qui entrent en jeu dans de telles circonstances peuvent, en effet, se révéler bénéfiques pour ces derniers d'un point de vue économique.

Absent From the Convention: Libraries, Law and Political Philosophy: John Adams and Thomas Jefferson

Presentation by Professor Mary Sarah Bilder, as commentator, at the conference "John Adams & Thomas Jefferson: Libraries, Leadership & Legacy," held in Boston and Charlottesville, June 21-17, 2009.