Paper money and national distress: William Huskisson and the early theories of credit, speculation and crises

This paper examines the explanation of commercial crises offered by William Huskisson in 1810 in the wake of the debate on the Bullion Report. Huskisson argued that the suspension of convertibility made it possible to extend issues of paper currency beyond its proper limits. Such an expansion, being in the interest of all parties concerned, would actually take place and stimulate excessive speculations, which would eventually prove unsustainable and bring generalized ruin and distress. Although some elements of this explanations were not new (having been anticipated by writers sucha as James Currie in 1793, William Roscoe in 1793, William Anderson in 1797 and an anonymous in 1796), Huskisson's explanation is more systematic and better organized, and his emphasis on the endogenous character of the crisis and on the instability of the dynamics of trade and credit makes it an interesting foreshadower of the theories of crises that were advanced half a century later.

Hepatic Manifestations of Wilson Disease - CHUV Experience 2005-2010

Background and aim: Wilson disease (WD) is an inherited disorder ofhepatic copper excretion leading to toxic accumulation of copper in theliver as well as the brain, cornea, and other organs. The defect is due tomutations of the copper-transporting ATPase ATP7B. Here, we describethe adult cases of hepatic WD diagnosed at the CHUV between 2005and 2010.Methods: Clinical manifestions, results of diagnostic tests, and follow-upof adult patients with hepatic WD were recorded systematically.Results: Seven new adult cases of hepatic WD were diagnosed in ourcenter between 2005 and 2010. Three were women and 4 men, with amedian a ge at d iagnosis o f 24 (range, 1 8-56) years. Three patientspresented with acute liver failure (ALF), three with persistently elevatedliver function tests, and one with a dvanced cirrhosis. None hadneurological manifestations. Only one patient, presenting with ALF, had aKayser-Fleischer corneal ring. Median ceruloplasmin levels at diagnosiswere 0.13 (range, <0.03-0.30) g/l, median 24 h urinary copper excretion6.3 (range, 0.4-62.0) μmol/24 h, and median hepatic copperconcentration 591 (range, 284-1049) μg/g. At least one mutation in theATP7B g ene was i dentified in a ll patients. Allelic frequency of t hecommon H1069Q mutation was 14%. Two patients presenting with ALFand the one with advanced cirrhosis underwent successful l ivertransplantation. One patient with ALF recovered under chelator therapy.D-penicillamine was used as first-line chelator treatment, with a switch totrientine due to adverse effects in 2 out of 4 patients u nder l ong-termtreatment.Conclusions: The clinical presentation of WD and the performance ofdiagnostic tests are variable. A high index of suspicion i n clinicallycompatible situations i s key, with a combination of tests allowing thediagnosis of WD.

La force motrice hydraulique au service du développement économique helvétique : L'exemple du réseau d'eau sous pression à Lausanne 1868-1914

The success story of hydroelectricity long influenced and dominated Swiss scholarly literature devoted to the history of technology. This means of conducting power, which emerged at the end of the 19th century and is still dominating today, has attracted much more attention than technologies that have been shadowed by its success. In spite of their important contribution to Swiss economic development, the distribution networks of pressurized water have been neglected by scholars. This article contributes to close this historiographic gap by analyzing the introduction of pressurized water distribution in 1876 in Lausanne, in the context of the building of the first Swiss cable funicular between Lausanne and Ouchy. This article shows how pressurized water distribution transformed socio-economic practices in the urban areas in which it was adopted. Indeed, this innovation, which allowed the use of distant hydraulic resources, enabled the rationalization of industrial and artisanal production as well as improved the density of the urban industrial base. By facilitating the introduction of electric lighting, pressurized water networks played a key role in the early development, and further successes, of the Swiss hydroelectric industry.

Mutation analysis of the ATP7B gene in a new group of Wilson's disease patients: contribution to diagnosis.

Wilson's disease (WD), an autosomal recessive disorder of copper transport with a broad range of genotypic and phenotypic characteristics, results from mutations in the ATP7B gene. Herein we report the results of mutation analysis of the ATP7B gene in a group of 118 Wilson disease families (236 chromosomes) prevalently of Italian origin. Using DNA sequencing we identified 83 disease-causing mutations. Eleven were novel, while twenty one already described mutations were identified in new populations in this study. In particular, mutation analysis of 13 families of Romanian origin showed a high prevalence of the p.H1069Q mutation (50%). Detection of new mutations in the ATP7B gene in new populations increases our capability of molecular analysis that is essential for early diagnosis and treatment of WD.

La maladie de Wilson: un caméléon clinique auquel il faut penser [A primer on Wilson disease for the general practitioner].

Wilson disease (WD) is an inherited disorder of hepatic copper excretion leading to toxic accumulation of copper in the liver as well as the brain, cornea, and other organs. The defect is due to mutations of the copper-transporting ATPase ATP7B. Clinical manifestations are highly variable and comprise acute liver failure, chronic hepatitis and cirrhosis as well as neurological or psychiatric symptoms. The Kayser-Fleischer corneal ring is pathognomonic but absent in about 50% of patients with hepatic manifestations alone. A high index of suspicion in clinically compatible situations is key, with a combination of laboratory tests allowing the diagnosis of WD. Treatment is based on the use of chelating agents, D-penicillamine or trientine. Liver transplantation should be considered for patients with acute liver failure or advanced cirrhosis.

Fonctions psychologiques du religieux: Cent ans après "Varieties" de William James

La première traduction en français de « The Varieties of Religious Experience » fut publiée en 1906 et elle connut aussitôt un très grand retentissement. Dans cet ouvrage, William James affirme: « D'un point de vue tout psychologique, la religion est une fonction éternelle de l'esprit humain ». Toutefois, caractériser la religion par une fonction si fondamentale, sous-jacente à tous les systèmes de croyances, ne conduit pas James à porter un regard dévalorisant sur la multiplicité des formes religieuses. Au contraire, il y voit l'expression de la variété des combinaisons possibles du religieux avec la vie psychique sous ses différents aspects. Ce livre a pour but non seulement de relire William James un siècle après la publication de Varieties, mais aussi de préciser, sur la base des connaissances actuelles, quelles sont les fonctions psychologiques du religieux.

L'analyse économique face à l'épuisement des ressources naturelles, de William Stanley Jevons à Harold Hotelling (1865-1931) : le cas de énergies fossiles

L'épuisement des énergies fossiles est un thème d'actualité dont les prémices datent, selon l'opinion courante, des années 1970 et du premier choc pétrolier. En réalité, c'est une préoccupation plus ancienne, intimement liée à l'ère industrielle. Dans la deuxième partie du XIXème siècle, les économistes se sont penchés sur la question de l'épuisement des minerais, 'objet non identifié' jusqu'alors et nécessitant la mise sur pied de nouveaux outils d'analyse (effet-rebond chez Jevons, rente minière chez Marshall-Einaudi notamment). Avec le progrès des techniques et l'apparition de nouvelles énergies (pétrole, hydro-électricité), leurs craintes de déclin industriel se sont progressivement dissipées dans les années 1910 et 1920. Mais ces évolutions tenant à l'histoire des faits ne sont pas les seules à considérer. Des facteurs internes à la discipline économique, comme l'émergence du marginalisme dans les années 1870 et de la théorie de l'épargne et du capital dans les années 1890, ont aussi changé le regard des économistes sur la question de l'épuisement des ressources. Pourquoi ? Comment ? Quels enseignements peut-on en tirer pour les défis environnementaux d'aujourd'hui ? Voilà les questions qui sont traitées dans ce travail de thèse.

EASL Clinical Practice Guidelines: Wilson's disease.

This Clinical Practice Guideline (CPG) has been developed to assist physicians and other healthcare providers in the diagnosis and management of patients with Wilson's disease. The goal is to describe a number of generally accepted approaches for diagnosis, prevention, and treatment of Wilson's disease. Recommendations are based on a systematic literature review in the Medline (PubMed version), Embase (Dialog version), and the Cochrane Library databases using entries from 1966 to 2011. The Grades of Recommendation, Assessment, Development, and Evaluation (GRADE) system used in other EASL CPGs was used and set against the somewhat different grading system used in the AASLD guidelines (Table 1A and B). Unfortunately, there is not a single randomized controlled trial conducted in Wilson's disease which has an optimal design. Thus, it is impossible to assign a high or even a moderate quality of evidence to any of the questions dealt with in these guidelines. The evaluation is mostly based on large case series which have been reported within the last decades.