Bone morphogenetic protein receptor 2 in patients with idiopathic portal hypertension

In idiopathic portal hypertension (IPH) typical vascular lesions are present in the branches of the portal vein or in the perisinusoidal area of the liver. Similar histological alterations have been reported in the pulmonary vasculature of patients with idiopathic pulmonary artery hypertension (IPAH). As IPAH is associated with mutations of the bone morphogenetic protein receptor 2 (BMPR2) gene, the aim of this study was to investigate whether this association might also be found in patients with IPH. Twenty-three samples belonging to 21 unrelated caucasian patients with IPH followed in the hepatic haemodynamic laboratory of the Hospital Clinic in Barcelona were included in the study. All patients were studied for the entire open reading frame and splice site of the BMPR2 gene by direct sequencing and multiple ligation probe amplification (MLPA) in order to detect large deletions/duplications. None of the 23 patients had pulmonary artery hypertension. Four patients presented one single nucleotide polymorphism (SNP) in intron 5, four patients had a SNP in exon 12 and a SNP in exon 1 was found in two cases. Two patients had both intron 5 and exon 12 polymorphisms. All SNPs were previously described. Except for these three SNPs, neither mutations nor rearrangements have been identified in the BMPR2 gene in this population. We did not detect mutations or rearrangements in the coding region of the BMPR2 gene in our patients with IPH. These findings suggest that, in contrast to IPAH, mutations in BMPR2 are not involved in the pathogenesis of IPH.

National Values and Theatrical Values. The Battle of the Siege de Calais (1765)

This article underscores the complex relationship between national concerns and dramatic criticism by interrogating the role of theatre in the creation of a 'national culture' during the last few decades of the Ancien regime. The author focuses more specifically on the forms of patriotism proposed by Pierre-Laurent De Belloy, author of Le Siege de Calais, France's "first tragedy in which the nation is given the pleasure to take an interest in itself," as well as by his adversaries and his allies. The version of patriotism proffered by De Belloy - a 'fatherland' that he defines as both bourgeois and monarchical - renders problematic several aesthetic and political norms in place in 1765. The author thus responds modestly to one of the most essential questions posed by research on eighteenth-century political and cultural history: how did patriotism operate before the French Revolution?

Scrivere di architettura intorno al 1780: Andrea Memmo e Francesco Milizia tra il Veneto e Roma

Andrea Memmo ist in der Geschichte der Architekturtheorie vor allem deswegen bekannt, weil er durch die Veröffentlichung der 'Elementi d'architettura lodoliana, ossia l'arte del fabricare con solidità scientifica e con eleganza non capricciosa' (Rom 1786) entscheidend dazu beigetragen hat, die originellen architektonischen Konzepte der Nachwelt zu überliefern, die der venezianische Franziskaner Carlo Lodoli zwischen 1730 und 1750 mündlich verbreitet hatte. Der vorliegende Beitrag unternimmt es, anhand des bislang für verloren gehaltenen 'Piano Accademico', den Memmo um 1767 für die venezianische Akademie zum Zwecke der Unterrichtsreform der bildenden Künste (Skulptur, Malerei und Architektur) ausgearbeitet hatte, klarzustellen, welche wichtige Rolle der Aufenthalt in Rom für Memmo gespielt hat. Dies wird auch durch die Briefe belegt, die er während der Ausarbeitung seines Werkes über Lodoli verfasst hatte. Im Rom der 1780er Jahre waren für ihn ausschlaggebend 1. die Beziehungen zur dortigen Accademia di S. Luca und 2. seine Freundschaft zum spanische Botschafter José Nicolas de Azara. Azara hatte ihn dazu ermutigt, über den Padre Lodoli zu schreiben, um dessen Gedanken denen des mit Azara befreundeten Francesco Milizia gegenüberzustellen.

Pathways that make voices - White matter changes in auditory hallucinations

BACKGROUND: The origin of auditory hallucinations, which are one of the core symptoms of schizophrenia, is still a matter of debate. It has been hypothesized that alterations in connectivity between frontal and parietotemporal speech-related areas might contribute to the pathogenesis of auditory hallucinations. These networks are assumed to become dysfunctional during the generation and monitoring of inner speech. Magnetic resonance diffusion tensor imaging is a relatively new in vivo method to investigate the directionality of cortical white matter tracts. OBJECTIVE: To investigate, using diffusion tensor imaging, whether previously described abnormal activation patterns observed during auditory hallucinations relate to changes in structural interconnections between the frontal and parietotemporal speech-related areas. METHODS: A 1.5 T magnetic resonance scanner was used to acquire twelve 5-mm slices covering the Sylvian fissure. Fractional anisotropy was assessed in 13 patients prone to auditory hallucinations, in 13 patients without auditory hallucinations, and in 13 healthy control subjects. Structural magnetic resonance imaging was conducted in the same session. Based on an analysis of variance, areas with significantly different fractional anisotropy values between groups were selected for a confirmatory region of interest analysis. Additionally, descriptive voxel-based t tests between the groups were computed. RESULTS: In patients with hallucinations, we found significantly higher white matter directionality in the lateral parts of the temporoparietal section of the arcuate fasciculus and in parts of the anterior corpus callosum compared with control subjects and patients without hallucinations. Comparing patients with hallucinations with patients without hallucinations, we found significant differences most pronounced in the left hemispheric fiber tracts, including the cingulate bundle. CONCLUSION: Our findings suggest that during inner speech, the alterations of white matter fiber tracts in patients with frequent hallucinations lead to abnormal coactivation in regions related to the acoustical processing of external stimuli. This abnormal activation may account for the patients' inability to distinguish self-generated thoughts from external stimulation.

Meditationen über die Verhandlungen in der holsteinischen Ständekammer in Betreff der Petition mosaischer Glaubensgenossen wegen Ertheilung des Bürgerrechts, in der 41sten Sitzung am 22. November 1838

von [Salomon Ludwig] Steinheim

Aperçu général de la gestion de 1825 à 1838 : Rapport présenté par la commission administrative

Société d'encouragement au travail en faveur Israélites indigents du Bas-Rhin

Schilderungen aus dem Prerauer Ghettoleben vom Jahre 1838 - 1848 : mit Streiflichtern bis in die Gegenwart und Jugenderinnerungen eines 78jährigen

von Ignaz Briess

Familia honor y poder. La nobleza de la ciudad de Quito durante la época colonial tardía (1765- 1822)

Con este trabajo sobre la nobleza de la ciudad de Quito, Christian Büschges amplía el campo de los estudios socio-económicos y políticos sobre las así llamadas élites coloniales, con un análisis que se enfoca de manera especial en las mentalidades y representaciones estamentales, que tan fuertemente marcaron las relaciones sociales del antiguo régimen colonial.

Factors affecting cognitive outcome in early pediatric stroke

OBJECTIVE We examined cognitive performance in children after stroke to study the influence of age at stroke, seizures, lesion characteristics, neurologic impairment (NI), and functional outcome on cognitive outcome. METHODS This was a prospectively designed study conducted in 99 children who sustained an arterial ischemic stroke (AIS) between the age of 1 month and 16 years. All children underwent cognitive and neurologic follow-up examination sessions 2 years after the insult. Cognitive development was assessed with age-appropriate instruments. RESULTS Although mean cognitive performance was in the lower normative range, we found poorer results in subtests measuring visuoconstructive skills, short-term memory, and processing speed. Risk factors for negative cognitive outcome were young age at stroke, seizures, combined lesion location (cortical and subcortical), as well as marked NI. CONCLUSIONS We recommend that all children with a history of AIS undergo regularly scheduled neuropsychological assessment to ensure implementation of appropriate interventions and environmental adjustments as early as possible.