Wirkungsevaluation in der Entwicklungspolitik

Über die Wirksamkeit der Entwicklungspolitik wurde in den letzten Jahren viel spekuliert. Vor allem deshalb, weil hierzu - trotz vielfältiger Evaluationsaktivitäten - sowohl in der Wissenschaft als auch bei den Geberorganisationen selbst nur wenige profunde Ergebnisse vorlagen. Mittlerweile wird dem Problem nicht nur in der Wissenschaft, sondern vor allem auch in den Geberorganisationen erhebliche Aufmerksamkeit geschenkt. Reformen im Evaluationssystem der deutschen Geber- und Durchführungsorganisationen haben das Ziel, Evaluationen als Managementinstrument sowie als Mittel zur Legitimierung der Entwicklungszusammenarbeit zu stärken. Insgesamt soll die Wirkungsbeobachtung im Rahmen projekt- und programmbegleitender Monitoringaktivitäten als auch bei der Durchführung von Evaluationen gestärkt werden. Hierfür werden zunehmend auch wissenschaftliche Konzepte miteinbezogen. Der Reformprozess hat gerade erst begonnen und muss konsequent weitergeführt werden

Diffuse cutaneous mastocytosis with novel somatic KIT mutation K509I and association with tuberous sclerosis.

Diffuse cutaneous mastocytosis (DCM) is a rare but potentially fatal condition when diagnosis and targeted treatments are delayed. This case illustrates the life-threatening complications in DCM and reviews the currently available treatments. To our knowledge, this is the first report of mastocytosis with somatic K509I mutation and concomitant tuberous sclerosis

A new TRPV3 missense mutation in a patient with Olmsted syndrome and erythromelalgia

IMPORTANCE: Olmsted syndrome (OS) is a rare keratinizing disorder characterized by excessive epidermal thickening of the palms and soles, with clinical and genetic heterogeneity. Approximately 50 cases have been reported, with the molecular basis described in only 9. Recently, TRPV3 (transient receptor potential vanilloid 3) mutations were identified in autosomal-dominant OS in 7 sporadic cases and 1 familial case, whereas an MBTPS2 (membrane-bound transcription factor protease, site 2) mutation was reported in X-linked recessive OS. We report a new sporadic case of severe, atypical OS and its underlying genetic basis. OBSERVATIONS: Our patient is a young girl with severe nonmutilating (palmo)plantar keratoderma without periorificial keratotic plaques associated with intense acute flares of inflammation, itching, burning pain, vasodilatation, and redness of the extremities consistent with erythromelalgia. Whole exome sequencing of patient DNA identified a novel de novo heterozygous missense mutation within TRPV3, p.Leu673Phe, predicted to be damaging. CONCLUSIONS AND RELEVANCE: This case study further implicates TRPV3 in OS pathogenesis. In addition, previous reports of OS have not described erythromelalgia as a clinical feature. Its occurrence in our patient could be a chance event, but, if associated with OS, the features of erythromelalgia may expand the phenotypic spectrum of this rare syndrome. Copyright 2014 American Medical Association. All rights reserved

Making Ordinary People Actors in National History: ‘Hanoi life under the subsidy economy’ in the Vietnam Museum of Ethnology

This paper analyzes a recent exhibit in Vietnam’s national ethnology museum which depicted ordinary people’s everyday experiences during the postwar, pre-reform era in Hanoi, an era which was full of hardship and had rarely been  discussed publicly prior to this. The exhibit narrated a critical yet nostalgic representation which favored the experiences of ‘ordinary citizens’’ above official discourses about this period. I argue that the exhibit designers aimed to pose ordinary citizens as significant actors in national history, thereby narrating Vietnam’s modern history in a new way. In the course of critically examiningthe past and the present, the exhibit pushed the boundaries of social criticism and public discourse, and showed that museum exhibits are not neutral spacesfor the production of knowledge but, rather, can become sites which mediate indirect negotiation between people and the state and re-interpret the past forthe present and the future.   Keywords: museums, politics of memory, personal narratives, subsidy system, Vietna

The Default Art of Classifying the Occult

Even as the world of information moves increasingly away from print, academic librarians and patrons spend significant time in neighborhoods of books organized by Library of Congress Classification. Close attention to specific neighborhoods and historical inquiry into their organization opens windows into past and present constructions of knowledge. This essay examines one such neighborhood – the latter ranges of BF, and particularly its tail end, Occult Sciences. It explores the creation of this neighborhood in a deliberately recursive fashion, and the journey to determine how such content came to be collocated by the Library of Congress leads through other classification systems, bookseller catalogs, and even literature. As food for further thought many comments from the essay manuscript’s anonymous reviewers are appended