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Introduction: Since 2016, kidney transplantation in patients with atypical hemolytic uremic syndrome (aHUS) in the Netherlands is performed without eculizumab prophylaxis. Eculizumab is given in case of posttransplant aHUS recurrence. Eculizumab therapy is monitored in the CUREiHUS study. Methods:All participating kidney transplant patients who received eculizumab therapy for a suspected posttransplant aHUS recurrence were evaluated. Overall recurrence rate was monitored prospectively at Radboud University Medical Center. Results:In the period from January 2016 until October 2020, we included 15 (12 females, 3 males; median age 42 years, range 24−66 years) patients with suspected aHUS recurrence after kidney transplantation in this study. The time interval to recurrence showed a bimodal distribution. Seven patients presented early after transplantation (median 3 months, range 0.3−8.8 months), with typical aHUS features: rapid loss of estimated glomerular filtration rate (eGFR) and laboratory signs of thrombotic microangiopathy (TMA). Eight patients presented late (median 46 months, range 18−69 months) after transplantation. Of these, only 3 patients had systemic TMA, whereas 5 patients presented with slowly deteriorating eGFR without systemic TMA. Treatment with eculizumab resulted in improvement or stabilization of eGFR in 14 patients. Eculizumab discontinuation was tried in 7 patients; however, it was successful only in 3. At the end of the follow-up (median 29 months, range 3–54 months after start of eculizumab), 6 patients had eGFR <30 ml/min per 1.73 m2. Graft loss had occurred in 3 of them. Overall, aHUS recurrence rate without eculizumab prophylaxis was 23%. Conclusions: Rescue treatment of posttransplant aHUS recurrence is effective; however, some patients suffer from irreversible loss of kidney function, likely caused by delayed diagnosis and treatment and/or too a

It is common in architecture education to quantify the quality of assignments into grades, often done by one or two teachers using rubrics. However, this can have several downsides. It suggests an objective preciseness that is debatable for the creative assignments in the field of architecture, and the assessment is dependent on the judgement of only one or two people. Comparative judgement (CJ) offers an alternative to rubric-based assessment by applying pairwise comparison to student assignments, resulting in a ranking instead of a grade. We used a mixed methods approach to compare the reliability, time efficiency, and fairness of CJ in the selection of students for an undergraduate architecture programme at Delft University of Technology in the Netherlands. Teachers involved in the rubric-based approach for student selection were asked to re-assess a random selection of the assignments using CJ. Reliability and time investments for both methods were compared, and the involved assessors were asked in a focus group setting which of the two methods they perceived as more reliable and fair. Comparing rubric-based assessment to CJ is new, as previous studies have only looked at these assessment methods in isolation. Findings indicate that CJ can be serve as a more reliable and time efficient alternative to rubric-based assessment. However, teachers still perceive rubrics as having higher reliability and fairness. Though this research is particularly relevant in the context of architecture, it contributes to wider discussions about reliable and fair assessment of creative student assignments., Education and Student Affairs, Urban Development Management

Objective To codesign an online support intervention for families after sudden cardiac death (SCD) in the young (<35 years). Design Codesign of an SCD family intervention by stakeholder focus groups. Setting Families and healthcare professionals with experience in SCD in the young. Participants Semistructured online focus groups were held with key stakeholders, that is, family members who had experienced young SCD, healthcare professionals and researchers based in New South Wales, Australia. Guided discussions were used to develop an online support intervention. Thematic analysis of discussions and iterative feedback on draft materials guided content development. Results Four focus groups were held (4-6 participants per group, 12 unique participants). Stakeholder involvement facilitated development of high-level ideas and priority issues. Creative content and materials were developed based on user preference for stories, narratives and information reflecting everyday experience of families navigating the legal and medical processes surrounding SCD, normalising and supporting grief responses in the context of family relationships and fostering hope. Emphasis on accessibility led to the overarching need for digital information and online engagement. These insights allowed development of an online intervention - COPE-SCD: A COmmunity suPporting familiEs after Sudden Cardiac Death - which includes a website and online support programme. Conclusion Using codesign with stakeholders we have developed a support intervention that addresses the needs of SCD families and aims to fill a large gap in existing healthcare. We will evaluate COPE-SCD to determine whether this is an effective intervention for support of families following a young SCD.

Sudden cardiac death (SCD) is a devastating complication of genetic heart diseases. The psychological impact on surviving family members is significant and lifelong. Previous needs analyses found medical and psychological needs are of high importance but remain unmet in some cases. We used co-design focus groups with stakeholders (healthcare professionals and family members) to develop a support intervention: COPESCD - an online community supporting families after SCD in the young. The intervention contains two parts: (1) a website and (2) a series of four, online support sessions (covering: general information, uncertainty, grief and loss, and family coping).

Traditionally, challenges of natural resource management have been addressed with a sectoral policy approach. However, it is increasingly recognised that different sectors are interconnected in a complex and mutually interacting system. A nexus approach is proposed to identify synergies and trade-offs between sectors and to foster the sustainable and efficient use of resources, particularly in light of climate change. The nexus approach has led to studies identifying interactions between policy objectives across nexus sectors, but the physical interactions between nexus sectors that can be the result of policy interactions, have received less attention. Nevertheless, such interactions can have severe consequences for the environment, affecting ecosystems and the services they provide. Integrating the nexus approach and the ecosystem service concept may help to better understand pressures and impacts related to a resource nexus and to address trade-offs. In this study, literature and expert assessment are used to analyse the water-energy-food-land-climate nexus in Sweden through the lens of the ecosystem services concept to gain insights into interactions between the nexus sectors. By demonstrating how anthropogenic pressures originating from the nexus sectors affect ecosystem functions and services, this paper serves as a foundation to further inform policy making (within and outside Sweden) when considering the water-energy-food-land-climate nexus.