Refining Kidney Survival in 383 Genetically Characterized Patients With Nephronophthisis

Introduction: Nephronophthisis (NPH) comprises a group of rare disorders accounting for up to 10% of end-stage kidney disease (ESKD) in children. Prediction of kidney prognosis poses a major challenge. We assessed differences in kidney survival, impact of variant type, and the association of clinical characteristics with declining kidney function. Methods: Data was obtained from 3 independent sources, namely the network for early onset cystic kidney diseases clinical registry (n = 105), an online survey sent out to the European Reference Network for Rare Kidney Diseases (n = 60), and a literature search (n = 218). Results: A total of 383 individuals were available for analysis: 116 NPHP1, 101 NPHP3, 81 NPHP4 and 85 NPHP11/TMEM67 patients. Kidney survival differed between the 4 cohorts with a highly variable median age at onset of ESKD as follows: NPHP3, 4.0 years (interquartile range 0.3–12.0); NPHP1, 13.5 years (interquartile range 10.5–16.5); NPHP4, 16.0 years (interquartile range 11.0–25.0); and NPHP11/TMEM67, 19.0 years (interquartile range 8.7–28.0). Kidney survival was significantly associated with the underlying variant type for NPHP1, NPHP3, and NPHP4. Multivariate analysis for the NPHP1 cohort revealed growth retardation (hazard ratio 3.5) and angiotensin-converting enzyme inhibitor (ACEI) treatment (hazard ratio 2.8) as 2 independent factors associated with an earlier onset of ESKD, whereas arterial hypertension was linked to an accelerated glomerular filtration rate (GFR) decline. Conclusion: The presented data will enable clinicians to better estimate kidney prognosis of distinct patients with NPH and thereby allow personalized counseling

Sport and ethno-racial formation: imagined distance in Fiji

Significant research depicts the implications of sport’s role in racial formation located mainly in the ‘Global North’. Yet, there has been less attention afforded to the related role of sport in the ‘Global South’, particularly in divided societies, where the consequences of sports’ influence on ethno-racial relations, are also significant. This study relies on empirical evidence gathered during an in-depth exploration into the role of soccer and rugby in Fijian intergroup relations. Sport is analysed as an arena that not only plays host to ethno-racial groupings but one which is also instrumental in their maintenance and reimagining. In Fiji at least, the organisation and positioning of sport in popular culture and discourse means that it becomes an emblematic sphere, active in the reconfirmation and preservation of ethno-racial division. Through this discussion, this study contributes to sport and racial formation theory, widening the gaze to diverse and divided socio-cultural settings

Renewable energy from Cyanobacteria: energy production optimization by metabolic pathway engineering

The need to develop and improve sustainable energy resources is of eminent importance due to the finite nature of our fossil fuels. This review paper deals with a third generation renewable energy resource which does not compete with our food resources, cyanobacteria. We discuss the current state of the art in developing different types of bioenergy (ethanol, biodiesel, hydrogen, etc.) from cyanobacteria. The major important biochemical pathways in cyanobacteria are highlighted, and the possibility to influence these pathways to improve the production of specific types of energy forms the major part of this review

Economic modeling and budget impact analysis in abdominal surgery: the case of Mesh

The chapter describes the Budget Impact Analysis (BIA) which is increasingly demanded by regulators as an essential part of a comprehensive economic evaluation of a new healthcare intervention. An example of BIA is presented considering the case of prostheses for incisional hernia repairs. The objective of this chapter is to give an overview of an ongoing project with the aim to estimate the current economic impact of the management of patients with incisional abdominal hernia through biosynthetic, synthetic or biologic meshes and to evaluate changes in the healthcare budget considering a future scenario with increased utilization rates of biosynthetic meshes in the next five years. The analysis is performed from the hospital perspective in Italy