Socially sensitive court cases are often discussed within the framework of democratic legitimacy. According to critics, problems of democratic legitimacy would not only manifest themselves... Show moreSocially sensitive court cases are often discussed within the framework of democratic legitimacy. According to critics, problems of democratic legitimacy would not only manifest themselves empirically (a lack of societal acceptance), but also normatively (a lack of justification of political power). Against the background of citizens and interest groups aiming to transform a social conflict into a legal one that is suitable for a criminal trial, this paper argues that a third conception of legitimacy is needed: both the common empirical focus on perceived legitimacy, as well as the pure normative focus on objective qualities of powerholders obscure the social dynamics of powerholders making claims to legitimacy and their audiences judging these claims. Drawing on recent theoretical work on legitimacy, this paper argues that democratic legitimacy neither resides in individuals’ minds nor in objective qualities of powerholders. Instead, it will always need to be constructed in a constant dialogue between multiple audiences, who express their problems and conflicts, and powerholders that respond to them within the normative frameworks in which they operate. This dialogic approach to legitimacy thus implies conducting both empirical and normative research. After empirically studying the social practices of claims-making, dialogue and judgment through a media analysis, this paper turns to democratic theory in order to spark our institutional imagination on how to make the responses of courts more democratic, while still accounting for the normative limits in which they operate. Show less
Tange, F.P.; Verduijn, P.S.; Mulder, B.G.S.; Capelle, L. van; Koning, S.; Driessen, C.; ... ; Vorst, J.R. van der 2023
Background: A common complication after a DIEP flap reconstruction is the occurrence of fat necrosis due to inadequate flap perfusion zones. Intraoperative identification of ischemic zones in the... Show moreBackground: A common complication after a DIEP flap reconstruction is the occurrence of fat necrosis due to inadequate flap perfusion zones. Intraoperative identification of ischemic zones in the DIEP flap could be opti-mized using indocyanine green near-infrared fluorescence angiography (ICG-NIR-FA). This randomized controlled trial aims to determine whether intraoperative ICG-NIR-FA for the assessment of DIEP flap perfusion decreases the occurrence of fat necrosis. Design/methods: This article describes the protocol of a Dutch multicenter randomized controlled clinical trial: the FAFI-trial. Females who are electively scheduled for autologous breast reconstruction using DIEP or muscle -sparing transverse rectus abdominis muscle (msTRAM) flaps are included. A total of 280 patients will be included in a 1:1 ratio between both study arms. In the intervention arm, the intraoperative assessment of flap perfusion will be based on both regular clinical parameters and ICG-NIR-FA. The control arm consists of flap perfusion evaluation only through the regular clinical parameters, while ICG-NIR-FA images are obtained during surgery for which the surgeon is blinded. The main study endpoint is the difference in percentage of clinically relevant fat necrosis between both study arms, evaluated two weeks and three months after reconstruction. Conclusion: The FAFI-trial, a Dutch multicenter randomized controlled clinical trial, aims to investigate the clinical added value of intraoperative use of standardized ICG-NIR-FA for assessment of DIEP/msTRAM flap perfusion in the reduction of fat necrosis. Clinical trial registration number: NCT05507710; NL 68623.058.18. Show less
Weerts, M.J.A.; Lanko, K.; Guzman-Vega, F.J.; Jackson, A.; Ramakrishnan, R.; Cardona-Londono, K.J.; ... ; Genomics England Res 2021
Purpose Pathogenic variants in SETD1B have been associated with a syndromic neurodevelopmental disorder including intellectual disability, language delay, and seizures. To date, clinical features... Show morePurpose Pathogenic variants in SETD1B have been associated with a syndromic neurodevelopmental disorder including intellectual disability, language delay, and seizures. To date, clinical features have been described for 11 patients with (likely) pathogenic SETD1B sequence variants. This study aims to further delineate the spectrum of the SETD1B-related syndrome based on characterizing an expanded patient cohort. Methods We perform an in-depth clinical characterization of a cohort of 36 unpublished individuals with SETD1B sequence variants, describing their molecular and phenotypic spectrum. Selected variants were functionally tested using in vitro and genome-wide methylation assays. Results Our data present evidence for a loss-of-function mechanism of SETD1B variants, resulting in a core clinical phenotype of global developmental delay, language delay including regression, intellectual disability, autism and other behavioral issues, and variable epilepsy phenotypes. Developmental delay appeared to precede seizure onset, suggesting SETD1B dysfunction impacts physiological neurodevelopment even in the absence of epileptic activity. Males are significantly overrepresented and more severely affected, and we speculate that sex-linked traits could affect susceptibility to penetrance and the clinical spectrum of SETD1B variants. Conclusion Insights from this extensive cohort will facilitate the counseling regarding the molecular and phenotypic landscape of newly diagnosed patients with the SETD1B-related syndrome. Show less
