Translations of new public management: a decentred approach to school governance in four OECD countries

Despite the prevalence of corporate and performative models of school governance within and across different education systems, there are various cases of uneven, hybrid expressions of New Public Management (NPM) that reveal the contingency of global patterns of rule. Adopting a ‘decentred approach’ to governance (Bevir, M. 2010. “Rethinking Governmentality: Towards Genealogies of Governance.” European Journal of Social Theory 13 (4): 423–441), this paper compares the development of NPM in four OECD countries: Australia, England, Spain, and Switzerland. A focus of the paper is how certain policy instruments are created and sustained within highly differentiated geo-political settings and through different multi-scalar actors and authorities yet modified to reflect established traditions and practices

Somatic mosaicism and common genetic variation contribute to the risk of very-early-onset inflammatory bowel disease

Abstract: Very-early-onset inflammatory bowel disease (VEO-IBD) is a heterogeneous phenotype associated with a spectrum of rare Mendelian disorders. Here, we perform whole-exome-sequencing and genome-wide genotyping in 145 patients (median age-at-diagnosis of 3.5 years), in whom no Mendelian disorders were clinically suspected. In five patients we detect a primary immunodeficiency or enteropathy, with clinical consequences (XIAP, CYBA, SH2D1A, PCSK1). We also present a case study of a VEO-IBD patient with a mosaic de novo, pathogenic allele in CYBB. The mutation is present in ~70% of phagocytes and sufficient to result in defective bacterial handling but not life-threatening infections. Finally, we show that VEO-IBD patients have, on average, higher IBD polygenic risk scores than population controls (99 patients and 18,780 controls; P < 4 × 10−10), and replicate this finding in an independent cohort of VEO-IBD cases and controls (117 patients and 2,603 controls; P < 5 × 10−10). This discovery indicates that a polygenic component operates in VEO-IBD pathogenesis