601. A risk score to predict kidney survival in patients with autosomal recessive polycystic kidney disease at the age of two monthsKathrin Burgmaier, Samuel Kilian, Klaus Arbeiter, Bahriye Atmis, Olivia Boyer, Anja Katrin Buescher, Ismail Dursun, 2025, pregledni znanstveni članek Povzetek: Autosomal recessive polycystic kidney disease (ARPKD) is a severe hepatorenal fibrocystic disorder. Its rareness and the variability of disease courses have been major obstacles for the establishment of clinical trials on treatment of kidney disease in ARPKD. In this observational study we characterized kidney disease progression in a very large cohort of up to 658 patients with the clinical diagnosis of ARPKD and identified risk factors associated with rapid kidney disease progression. The estimated probability of kidney failure by the age of 20 years was 50.1% (95% confidence interval 42.2%‒57.0%), with earlier kidney failure in specific subgroups. Mean yearly estimated glomerular filtration rate decline after the first year of life was 1.3 ml/min per 1.73 m2 during childhood and adolescence in the overall cohort, ranging from 0.5 to 2.2 ml/min per 1.73 m2 in various subgroups. Furthermore, we developed prediction models for the relative risk of early kidney failure to be applied at the age of two months in daily clinical life. The finally chosen predictor set for a score based on a Cox model encompassed five factors: gestational age at oligo- or anhydramnios, gestational age at birth, functional genotype, serum creatinine (mg/dl) as well as documentation of arterial hypertension at the age of two months. The derived simple prognostic score showed good prediction performance, especially in the first three years of life. It reliably identified patients who are not at risk of early kidney failure and may be helpful to identify patients at risk of more rapid disease progression that could benefit from novel therapeutic interventions.
Ključne besede: ciliopathies, fibrocystic hepatorenal disease, fibrocystin, kidney survival, polycystic kidney disease
Objavljeno v DiRROS: 14.11.2025; Ogledov: 195; Prenosov: 78
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602. Intestinal involvement in graft versus host disease in children : an overview by the ESPGHAN Gastroenterology CommitteeMarina Vincent, Amit Assa, Osvaldo Borrelli, Matjaž Homan, Javier Martin-de-Carpi, Zrinjka Mišak, Maria Giovanna Puoti, Isabelle Scheers, Sara Sila, Caterina Strisciuglio, Christos Tzivinikos, Jernej Dolinšek, Emmanuel Mas, 2025, pregledni znanstveni članek Povzetek: Graft versus host disease (GVHD) is a complication that frequently occurs afterhaematopoietic stem cell transplantation and concerns many children in paediatrichaematology‐oncology and bone marrow transplantation departments. It can affectvarious organs, with the skin, gastrointestinal tract and liver being the most com-monly involved. To confirm intestinal GVHD and to rule out differential diagnosesendoscopy is frequently needed. Currently, there are no specific consensus rec-ommendations concerning the best method for endoscopic exploration and medicalmanagement of this disease in children, with limited studies available, including alow number of patients. Sigmoidoscopy could be initially proposed under sedation. Ifsigmoidoscopy is normal or if a general anaesthesia is required, colonoscopy andupper endoscopy should be planned, avoiding duodenal biopsy because of the riskof duodenal haematoma. Regarding therapeutic options, corticosteroids are thefirst‐line treatment for GVHD. Ruxolitinib, a Janus kinase inhibitor, is indicated forchildren aged 12 years and older with acute or chronic GVHD who have aninadequate response to corticosteroids or other systemic therapies. Nutritionalsupport has a key role in the management of intestinal GVHD and should beconsidered to guarantee the best possible evolution of intestinal GVHD.
Ključne besede: GVHD, children, complication, endoscopy, haematopoietic stem cell transplantation, intestine
Objavljeno v DiRROS: 14.11.2025; Ogledov: 146; Prenosov: 79
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608. What is a circular economy champion? Defining a home-grown concept in an emerging fieldDolores Modic, Sahana Swaroop Chetan, Shajara Ul-Durar, Noman Arshed, Marco De Sisto, Nadja Damij, 2025, izvirni znanstveni članek Povzetek: Circular economy, which aims to keep resources in production cycles and minimize waste, has emerged as a viable alternative to the traditional linear model. However, it is often criticized for being under-theorized and lacking indigenous, home-grown concepts, particularly regarding fundamental elements such as actors and actor types. This paper focuses on a specific actor: the circular economy champion. Circular economy champions can drive the transition toward a more circular economy, yet the concept remains under-conceptualized despite a growing body of literature. The aims of this study are twofold: first, to highlight the importance of home-grown concepts in emerging fields; and second, to address the ambiguity surrounding the terminology of “circular economy champion” by providing a novel conceptualization. The paper develops this concept using a family resemblance approach, employing differentiation criteria through a piecemeal method and underpinned by a theoretical review of the literature. By extending both the scope of actor types and, based on their traits, the range of their attributes, activities, and contributions to the circular economy, the concept of the circular economy champion is further delineated from related constructs and firmly defined. This contributes to circular economy discourse by proposing a novel definition of a key actor within the circular economy system, thus strengthening its theoretical foundations. Moreover, the proposed definition reduces ambiguity in understanding actors’ roles within the circular economy, further clarifying its microfoundations.
Ključne besede: circular economy, actor, circular economy champion, concept development
Objavljeno v DiRROS: 13.11.2025; Ogledov: 164; Prenosov: 85
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609. Quantum computing and the stable set problemAljaž Krpan, Janez Povh, Dunja Pucher, 2025, izvirni znanstveni članek Povzetek: Given an undirected graph, the stable set problem asks to determine the cardinality of the largest subset of pairwise non-adjacent vertices. This value is called the stability number of the graph, and its computation is an NP-hard problem. In this paper, we solve the stable set problem using the D-Wave quantum annealer. By formulating the problem as a quadratic unconstrained binary optimization problem with the penalty method, we show its optimal value equals the graph's stability number for specific penalty values. However, D-Wave's quantum annealer is a heuristic, so the solutions may be far from the optimum and may not represent stable sets. To address these, we introduce a post-processing procedure that identifies samples that could lead to improved solutions. Additionally, we propose a partitioning method to handle larger instances that cannot be embedded on D-Wave's quantum processing unit. Finally, we investigate how different penalty parameter values affect the solutions' quality. Extensive computational results show that the post-processing procedure significantly improves the solution quality, while the partitioning method successfully extends our approach to medium-size instances.
Ključne besede: stable set problem, quantum annealing, graph partitioning, D-Wave
Objavljeno v DiRROS: 13.11.2025; Ogledov: 198; Prenosov: 103
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