Techniques in Pediatric Surgery: Congenital Hyperinsulinism

For surgery in congenital hyperinsulinism (CHI), a distinct surgical strategy and technique is required for focal, diffuse and atypical CHI. In focal CHI, a confined, localized and parenchyma-sparing resection which is guided by the PET-CT is always indicated in order to cure the patient. In diffuse CHI, however, the results of surgical therapy are unpredictable and cure is an exception. Therefore, a strong tendency exists nowadays that medical therapy should be preferred in diffuse CHI. In atypical CHI the situation is more complex: if the focal lesion or the segmental mosaic are not too extensive, cure by resection should be possible. But care must be taken in atypical cases not to resect too much of the gland in order not to induce diabetes

Clinical and Genetic Evaluation of Patients with KATP Channel Mutations from the German Registry for Congenital Hyperinsulinism

Congenital hyperinsulinism (CHI) causes hypoglycemia due to irregular insulin secretion. In infants, a rapid diagnosis and appropriate management to avoid severe hypoglycemia is mandatory. CHI is a heterogeneous condition at the clinical and genetic level, and disease-causing genes have been identified in about half of the patients. The majority of mutations have been identified in the ABCC8 and KCNJ11 genes encoding subunits of the KATP channel responsible for two distinct histological forms. The diffuse form is caused by autosomal recessive or dominant inherited mutations, whereas the focal form is caused by a paternally transmitted recessive mutation and a second somatic event. We report on an unselected cohort of 136 unrelated patients from the German CHI registry. Mutations in either the ABCC8 or KCNJ11 gene were identified in 61 of these patients (45%). In total, 64 different mutations including 38 novel ones were detected in this cohort. We observed biparental (recessive) inheritance in 34% of mutation-positive patients, dominant inheritance in 11% and paternal transmission of a mutation associated with a focal CHI type in 38%. In addition, we observed inheritance patterns that do not exactly follow the classical recessive or dominant mode, further adding to the genetic complexity of this disease

Les mots et les choses : Johannes Schefferus et la philologie du réel entre Rhin et Laponie

Quand j’ai appris pour la première fois l’existence d’un catalogue électronique des manuscrits conservés en France, j’ai tout de suite regardé si Strasbourg disposait de manuscrits d’origine nordique. Je ne m’attendais pas à une grande découverte, mais l’un des manuscrits trouvés m’a profondément intrigué par l’improbabilité de son existence strasbourgeoise : il s’agit d’un rapport du 17e siècle écrit par un pasteur suédois, Olaus Graan, qui exerçait son office tout au nord du pays, à Piteå. ..

Das Kompetenznetzwerk Skandinavistik -Ein Modell für den Einsatz neuer Medien für die überregionale Kompetenzbündelung

Die Skandinavistik ist ein inhaltlich großes Fach mit geringen Ressourcen - sowohl personell als auch finanziell. Sie ist an den Universitäten Basel, Freiburg, Stras­bourg und Tübingen mit je einer Professur vertreten. Um ein inhaltlich vielfältiges und quali­tativ hochwertiges Studienangebot angesichts der Herausforderungen des Bologna­prozesses sicherstellen zu können, ist es nötig, die Ressourcen zu bündeln. Zugleich ist es sinnvoll, das Fach an möglichst vielen Hochschulen zu erhalten, da es immer wieder ein entscheidender Impulsgeber und Beiträger zu interdisziplinären Forschungs­projekten ist und im Kontext der Entstehung eines europäischen Arbeits­marktes wichtige Kompetenzen auch für Studierende anderer Fächer und Fakultäten vermittelt. Die skandinavistischen Abteilungen der Universitäten Basel, Freiburg, Strasbourg und Tübingen haben sich deshalb zu einem vertraglich abgesicherten, auf Nach­haltigkeit angelegten transnationalen Kompetenznetzwerk verbunden, das die an den Universitäten je vorhandenen Kompetenzen bündeln und diversifizieren soll. Mittels verschiedener eLearning-Szenarien wird Lehre zwischen den beteiligten Partnern regelmäßig ausgetauscht. Diese Veranstaltungen sind curricular inte­griert. Besonderes Augenmerk wird auf die Ebene der Master- und PhD-Studenten gelegt, da gerade hier zum einen fundierte Breite der Lehre von besonderer Bedeutung ist, zum anderen, da auf diesem Niveau eLearning-Szenarien, die auf selbstgesteuertes Lernen abzielen, besonders sinnvoll sind. Bewusst sollen die entwickelten Lehr-Modelle auf aufwändige multimediale Aufarbeitung verzichten, damit sie auch nach Ende der Förderungszeit mit geringem Aufwand an aktuelle Inhalte angepasst werden können. 25.02.2007 | Thomas MOHNIKE (Freiburg

Un Grec du Nord

Comment décrire la poésie de Jesper Svenbro ? Comment le décrire lui-même ? Poète suédois, de cœur français et d’inspiration hellénique ? Européen métapoétique ? Né en 1944 dans le Grand Sud de la Suède, il est spécialiste de littérature grecque ancienne, directeur de recherche au CNRS à Paris et, depuis 2006, membre de l’Académie suédoise. Comme chercheur, il utilise souvent la langue française pour écrire sur l’origine de la poésie grecque (La Parole et le marbre : aux origines de la poétiq..

Narrating the North. Towards a theory of mythemes of social knowledge in cultural circulation

When studying the history of geographical imaginations of the North, scholars often note that in spite of the wealth of cultural, artistic and societal contexts that are analysed, the same sets of elements representing the North can be found in constantly evolving combinations. In order to understand the sign system that is the North in social knowledge, the present article argues that we should change perspective and start by observing the smallest parts of the system, describing their nature and their ability to connect with other units of discourse. I propose to call these smallest units of discourse ‘mythemes of social knowledge’, and the laws that determine the possibilities to combine mythemes at a certain moment in time their ‘discursive grammar’. This approach allows us to understand the perceived stability not as a historical fact per se, but as an effect of family resemblance between mythemes changing through use and cultural circulation, and it permits to trace the history and geography of these transformations. In spite of the apparent simplicity of the approach, it proves to be quite complex as soon as we want to describe the changing repertoire of mythemes of the North in cultural circulation because of the number of sources and mythemes that should be considered. It requires data management that goes beyond traditional pen-and-paper analysis. It demands the use of relational databases, best informed by digital text retrieval with tools still to be developed. The last part of the article investigates the challenges and possible solutions for the sketched problem and presents first experimental results. It also shows that digital analysis not only allows us to handle the apparent complexity, but also helps to avoid the arbitrary definitions of individual mythemes because it forces us to work in a counterintuitive fashion, by objectivizing intuitive knowledge

Somatostatin receptors in congenital hyperinsulinism:Biology to bedside

Congenital hyperinsulinism (CHI), although a rare disease, is an important cause of severe hypoglycemia in early infancy and childhood, causing preventable morbidity and mortality. Prompt diagnosis and appropriate treatment is necessary to prevent hypoglycaemia mediated brain damage. At present, the medical treatment of CHI is limited to diazoxide as first line and synthetic somatostatin receptor ligands (SRLs) as second line options; therefore understanding somatostatin biology and treatment perspectives is important. Under healthy conditions, somatostatin secreted from pancreatic islet δ-cells reduces insulin release through somatostatin receptor induced cAMP-mediated downregulation and paracrine inhibition of β- cells. Several SRLs with extended duration of action are now commercially available and are being used off-label in CHI patients. Efficacy remains variable with the present generation of SRLs, with treatment effect often being compromised by loss of initial response and adverse effects such as bowel ischaemia and hepatobiliary dysfunction. In this review we have addressed the biology of the somatostatin system contexualised to CHI. We have discussed the clinical use, limitations, and complications of somatostatin agonists and new and emerging therapies for CHI