bluuebunny/crossref_metadata_2025_split huggingface dataset:
Increased prevalence of kidney cysts in individuals carrying heterozygous COL4A3 or COL4A4 pathogenic variants
Authors: Furlano Mónica , Pilco-Teran Melissa , Pybus Marc , Martínez Víctor , Aza-Carmona Miriam , Rius Peris Asunción , Pérez-Gomez Vanessa , Berná Gerson , Mazon Jaime , Hernández Jonathan , Fayos de Arizón Leonor , Viera Elizabet , Gich Ignasi , Pérez Hugo Vergara , Gomá-Garcés Elena , Albero Dolon José Luis , Ars Elisabet , Torra Roser 
Published: February 2024

ABSTRACT

Background
Clinical variability among individuals with heterozygous pathogenic/likely pathogenic (P/LP) variants in the COL4A3/COL4A4 genes (also called autosomal dominant Alport syndrome or COL4A3/COL4A4-related disorder) is huge; many individuals are asymptomatic or show microhematuria, while others may develop proteinuria and chronic kidney disease (CKD). The prevalence of simple kidney cysts (KC) in the general population varies according to age, and patients with advanced CKD are prone to have them. A possible association between heterozygous COL4A3, COL4A4 and COL4A5 P/LP variants and KC has been described in small cohorts. The presence of KC in a multicenter cohort of individuals with heterozygous P/LP variants in the COL4A3/COL4A4 genes is assessed in this study.

Methods
We evaluated the presence of KC by ultrasound in 157 individuals with P/LP variants in COL4A3 (40.7%) or COL4A4 (53.5%) without kidney replacement therapy. The association between presence of KC and age, proteinuria, estimated glomerular filtration rate (eGFR) and causative gene was analyzed. Prevalence of KC was compared with historical case series in the general population.

Results
Half of the individuals with P/LP variants in COL4A3/COL4A4 showed KC, which is a significantly higher percentage than in the general population. Only 3.8% (6/157) had cystic nephromegaly. Age and eGFR showed an association with the presence of KC (P &lt; .001). No association was found between KC and proteinuria, sex or causative gene.

Conclusions
Individuals with COL4A3/COL4A4 P/LP variants are prone to develop KC more frequently than the general population, and their presence is related to age and to eGFR. Neither proteinuria, sex nor the causative gene influences the presence of KC in these individuals.
Incidence and outcomes of kidney replacement therapy for end-stage kidney disease due to primary glomerular disease in Europe: findings from the ERA Registry
Authors: Abd ElHafeez Samar , Kramer Anneke , Arici Mustafa , Arnol Miha , Åsberg Anders , Bell Samira , Belliere Julie , Corte Carmen Díaz , Fresnedo Gema Fernández , Hemmelder Marc , Heylen Line , Hommel Kristine , Kerschbaum Julia , Naumović Radomir , Nitsch Dorothea , Santamaria Rafael , Finne Patrik , Palsson Runolfur , Pippias Maria , Resic Halima , Rosenberg Mai , de Pablos Carmen Santiuste , Segelmark Mårten , Sørensen Søren Schwartz , Soler Maria Jose , Vidal Enrico , Jager Kitty J , Ortiz Alberto , Stel Vianda S 
Published: February 2024

ABSTRACT

Background
Primary glomerular disease (PGD) is a major cause of end-stage kidney disease (ESKD) leading to kidney replacement therapy (KRT). We aimed to describe incidence (trends) in individuals starting KRT for ESKD due to PGD and to examine their survival and causes of death.

Methods
We used data from the European Renal Association (ERA) Registry on 69 854 patients who started KRT for ESKD due to PGD between 2000 and 2019. ERA primary renal disease codes were used to define six PGD subgroups. We examined age and sex standardized incidence, trend of the incidence and survival.

Results
The standardized incidence of KRT for ESKD due to PGD was 16.6 per million population (pmp), ranging from 8.6 pmp in Serbia to 20.0 pmp in France. Immunoglobulin A nephropathy (IgAN) and focal segmental glomerulosclerosis (FSGS) had the highest incidences, of 4.6 pmp and 2.6 pmp, respectively. Histologically non-examined PGDs represented over 50% of cases in Serbia, Bosnia and Herzegovina, and Romania and were also common in Greece, Estonia, Belgium and Sweden. The incidence declined from 18.6 pmp in 2000 to 14.5 pmp in 2013, after which it stabilized. All PGD subgroups had 5-year survival probabilities above 50%, with crescentic glomerulonephritis having the highest risk of death [adjusted hazard ratio 1.8 (95% confidence interval 1.6–1.9)] compared with IgAN. Cardiovascular disease was the most common cause of death (33.9%).

Conclusion
The incidence of KRT for ESKD due to PGD showed large differences between countries and was highest and increasing for IgAN and FSGS. Lack of kidney biopsy facilities in some countries may have affected accurate assignment of the cause of ESKD. The recognition of the incidence and outcomes of KRT among different PGD subgroups may contribute to a more individualized patient care approach.
A predictive mortality score in ANCA-associated renal vasculitis
Authors: Fage Nicolas , Quéméneur Thomas , Riou Jérémie , Boud'hors Charlotte , Desouche Alice , Vinatier Emeline , Samoreau Clément , Coindre Jean-Philippe , Djema Assia , Henry Nicolas , Gnemmi Viviane , Copin Marie-Christine , Piccoli Giorgina Barbara , Vandenbussche Cyrille , Augusto Jean-François , Brilland Benoit ,   the Maine-Anjou Registry Research Group, Augusto Jean-François , Beauvillain Céline , Coindre Jean-Philippe , Copin Marie-Christine , Cousin Maud , Croué Anne , Djema Assia , Guibert Fanny , Henry Nicolas , Piccoli Giorgina Barbara , Pouteau Lise-Marie , Wacrenier Samuel , Vinatier Emeline 
Published: February 2024

ABSTRACT

Background
Several scores have been developed to predict mortality at anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) diagnosis. Their prognostic value in Caucasian patients with kidney involvement (AAV-GN) remains uncertain as none has been developed in this specific population. We aimed to propose a novel and more accurate score specific for them.

Methods
This multicentric study included patients diagnosed with AAV-GN since January 2000 in four nephrology centers (recorded in the Maine-Anjou AAV-GN Registry). Existing scores and baseline characteristics were assessed at diagnosis before any therapeutic intervention. A multivariable analysis was performed to build a new predictive score for death. Its prognosis performance (area under receiving operating curve and C-index) and accuracy (Brier score) was compared with existing scores. One hundred and eighty-five patients with AAV-GN from the RENVAS registry were used as a validation cohort.

Results
A total of 228 patients with AAV-GN from the Maine-Anjou registry were included to build the new score. It included the four components most associated with death: age, history of hypertension or cardiac disease, creatinine and hemoglobin levels at diagnosis. Overall, 194 patients had all the data available to determine the performance of the new score and existing scores. The new score performed better than the previous ones in the development and in the validation cohort. Among the scores tested, only Five-Factor Score and Japanese Vasculitis Activity Score had good performance in predicting death in AAV-GN.

Conclusions
This original score, named DANGER (Death in ANCA Glomerulonephritis—Estimating the Risk), may be useful to predict the risk of death in AAV-GN patients. Validation in different populations is needed to clarify its role in assisting clinical decisions.
Sustainability of an Opt-Out Electronic-Health Record-Based Tobacco Treatment Consult Service at a Large Safety-Net Hospital: A 6-Year Analysis
Authors: Flores Adriana , Wiener Renda Soylemez , Hon Stephanie , Wakeman Cornelia , Howard Jinesa , Virani Nikita , Mattus Bruce , Foreman Alexis Gallardo , Singh Johar , Rosen Linda , Bulekova Katia , Kathuria Hasmeena 
Published: February 2024

Abstract

Introduction
Our safety-net hospital implemented a hospital-based tobacco treatment intervention in 2016. We previously showed the intervention, an “opt-out” Electronic Health Record (EHR)-based Best Practice Alert (BPA)+ order-set that triggers consultation to an inpatient Tobacco Treatment Consult (TTC) service for all patients who smoke, improves smoking abstinence. We now report on sustainability, 6 years after inception.

Aims and Methods
We analyzed data collected between July 2016–June 2022 of patients documented as “currently smoking” in the EHR. Across the 6 years, we used Pearson’s correlation analysis to compare Adoption (clinician acceptance of the BPA+ order-set, thus generating consultation to the TTC service); Reach (number of consultations completed by the TTC service); and Effectiveness (receipt of pharmacotherapy orders between patients receiving and not receiving consultations).

Results
Among 39 558 adult admissions (July 2016–June 2022) with “currently smoking” status in the EHR for whom the BPA triggered, clinicians accepted the TTC order set on 50.4% (19 932/39 558), though acceptance varied across services (eg, Cardiology [71%] and Obstetrics-Gynecology 12%]). The TTC service consulted on 17% (6779/39 558) of patients due to staffing constraints. Consultations ordered (r = −0.28, p = .59) and completed (r = 0.45, p = .37) remained stable over 6-years. Compared to patients not receiving consultations, patients receiving consultations were more likely to receive pharmacotherapy orders overall (inpatient: 50.8% vs. 35.1%, p &lt; .0001; at discharge: 27.1% vs. 10%, p &lt; .0001) and in each year.

Conclusions
The “opt-out” EHR-based TTC service is sustainable, though many did not receive consultations due to resource constraints. Health care systems should elevate the priority of hospital-based tobacco treatment programs to increase reach to underserved populations.

Implications
Our study shows that opt-out approaches that utilize the EHR are a sustainable approach to providing evidence-based tobacco treatment to all hospitalized individuals who smoke, regardless of readiness to stop smoking and clinical condition.
Do Infectious Diseases After Kidney Retransplantation Differ From Those After First Kidney Transplantation?
Authors: Kusejko Katharina , Neofytos Dionysios , van Delden Christian , Hirsch Hans H , Meylan Pascal , Boggian Katia , Hirzel Cedric , Garzoni Christian , Sidler Daniel , Schnyder Aurelia , Schaub Stefan , Golshayan Déla , Haidar Fadi , Bonani Marco , Kouyos Roger D , Mueller Nicolas J , Schreiber Peter W ,   the Swiss Transplant Cohort Study, Amico P , Aubert J-D , Banz V , Beckmann S , Beldi G , Berger C , Berishvili E , Berzigotti A , Binet I , Bochud P-Y , Branca S , Bucher H , Catana E , Cairoli A , Chalandon Y , De Geest S , De Rougemont O , De Seigneux S , Dickenmann M , Dreifuss J L , Duchosal M , Fehr T , Ferrari-Lacraz S , Garzoni C , Golshayan D , Goossens N , Halter F H J , Heim D , Hess C , Hillinger S , Hirsch H H , Hirt P , Hofbauer G , Huynh-Do U , Immer F , Koller M , Laager M , Laesser B , Lamoth F , Lehmann R , Leichtle A , Manuel O , Marti H P , Martinelli M , McLin V , Mellac K , Merçay A , Mettler K , Müller A , Mueller N J , Müller-Arndt U , Müllhaupt B , Nägeli M , Oldani G , Pascual M , Passweg J , Pazeller R , Posfay-Barbe K , Rick J , Rosselet A , Rossi S , Rothlin S , Ruschitzka F , Schachtner T , Schanz U , Schaub S , Scherrer A , Schnyder A , Schuurmans M , Schwab S , Sengstag T , Simonetta F , Stampf S , Steiger J , Stirnimann G , Stürzinger U , Van Delden C , Venetz J-P , Villard J , Vionnet J , Wick M , Wilhelm M , Yerly P 
Published: February 2024

AbstractBackgroundInfectious diseases (IDs) are highly relevant after solid organ transplantation in terms of morbidity and mortality, being among the most common causes of death. Patients undergoing kidney retransplantation (re-K-Tx) have been already receiving immunosuppressive therapy over a prolonged period, potentially facilitating subsequent infections. Comparing ID events after re-K-Tx and first kidney transplantation (f-K-Tx) can delineate patterns and risks of ID events associated with prolonged immunosuppression.MethodsWe included adult patients with records on f-K-Tx and re-K-Tx in the Swiss Transplant Cohort Study. We analyzed ID events after f-K-Tx and re-K-Tx within the same patients and compared infection rates, causative pathogens, and infection sites. Recurrent time-to-event analyses were performed for comparison of infection rates.ResultsA total of 59 patients with a median age of 47 years (range, 18–73) were included. Overall, 312 ID events in 52 patients occurred. In multivariable recurrent event modeling, the rate of ID events was significantly lower after re-K-Tx (hazard ratio, 0.70; P = .02). More bacterial (68.9% vs 60.4%) and fungal (4.0% vs 1.1%) infections were observed after f-K-Tx but fewer viral infections (27.0% vs 38.5%) as compared with re-K-Tx (P = .11). After f-K-Tx, urinary and gastrointestinal tract infections were more frequent; after re-K-Tx, respiratory tract and surgical site infections were more frequent (P &lt; .001).ConclusionsID events were less frequent after re-K-Tx. Affected sites differed significantly after f-K-Tx vs re-K-Tx.
Dalba Got Back? Use of Dalbavancin for the Treatment of Vertebral Osteomyelitis
Authors: Streifel Amber C , Strnad Luke C , Sikka Monica K , Varley Cara D , Makadia Jina , Sukerman Ellie , Douglass Alyse H , Mayer Heather , Young Kathleen , Lewis James S 
Published: February 2024

Abstract
Data evaluating dalbavancin use for vertebral osteomyelitis remain limited. In our retrospective cohort, 29 of 34 (85.3%) patients completed their dalbavancin course. Adverse reactions occurred for 6 (17.6%) and infection recurrence in 3 (8.8%) within 90 days. Dalbavancin appears to be safe and well-tolerated for vertebral osteomyelitis.
Burnout, Compassion Fatigue, and the Long Haul of Caring for Long COVID
Authors: Ramers Christian B , Scott John D , Struminger Bruce B 
Published: February 2024

Abstract
The current landscape of clinician burnout is prompting the need for our health care system to revise its approach toward complex conditions such as long coronavirus disease (COVID), myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS), and other postinfectious fatiguing illnesses (PIFIs). We discuss our efforts here at Family Health Center of San Diego (FHCSD) to help share insight and glean perspective from clinicians who have participated in our Centers for Disease Control and Prevention (CDC)–funded 3-year continuing professional development initiative. The Long COVID and Fatiguing Illness Recovery Program uses multidisciplinary team-based case consultation and peer-to-peer sharing of emerging best and promising practices (ie, teleECHO [Extension for Community Healthcare Outcomes]) to support the management of complex cases associated with long COVID, ME/CFS, and other PIFIs. We believe that this perspective captures a key moment in the trajectory of postpandemic clinician burnout and prompts further reflection and action from the health care system to improve clinician- and patient-level outcomes related to the care of patients with postinfectious fatiguing illnesses.
Modeling Invasive Aspergillosis Risk for the Application of Prophylaxis Strategies
Authors: Young Jo-Anne H , Andes David R , Ardura Monica I , Arrieta Antonio , Bow Eric J , Chandrasekar Pranatharthi H , Chen Sharon C A , Hammond Sarah P , Husain Shahid , Koo Sophia , Lavergne Valéry , Nguyen M Hong , Patterson Thomas F , So Miranda , Thompson George R , Morrissey C Orla , Schuster Mindy G 
Published: February 2024

Abstract
The epidemiology of invasive aspergillosis (IA) is evolving. To define the patient groups who will most likely benefit from primary or secondary Aspergillus prophylaxis, particularly those whose medical conditions and IA risk change over time, it is helpful to depict patient populations and their risk periods in a temporal visual model. The Sankey approach provides a dynamic figure to understand the risk of IA for various patient populations. While the figure depicted within this article is static, an internet-based version could provide pop-up highlights of any given flow's origin and destination nodes. A future version could highlight links to publications that support the color-coded incidence rates or other actionable items, such as bundles of applicable pharmacologic or non-pharmacologic interventions. The figure, as part of the upcoming Infectious Diseases Society of America's aspergillosis clinical practice guidelines, can guide decision-making in clinical settings.
The characteristic time scale of cultural evolution
Authors: Wand Tobias , Hoyer Daniel 
Published: February 2024

Abstract
Numerous researchers from various disciplines have explored commonalities and divergences in the evolution of complex social formations. Here, we explore whether there is a “characteristic” time course for the evolution of social complexity in a handful of different geographic areas. Data from the Seshat: Global History Databank is shifted so that the overlapping time series can be fitted to a single logistic regression model for all 23 geographic areas under consideration. The resulting regression shows convincing out-of-sample predictions, and its period of extensive growth in social complexity can be identified via bootstrapping as a time interval of roughly 2,500 years. To analyze the endogenous growth of social complexity, each time series is restricted to a central time interval without major disruptions in cultural or institutional continuity, and both approaches result in a similar logistic regression curve. Our results suggest that these different areas have indeed experienced a similar course in the their evolution of social complexity, but that this is a lengthy process involving both internal developments and external influences.
Male spondyloarthritis patients and those with longer disease duration have less severe disc degeneration: propensity score-matched comparison
Authors: Cheung Samuel Tin Yan , Tsang Helen Hoi Lun , Cheung Prudence Wing Hang , Cheung Jason Pui Yin 
Published: February 2024

Abstract

Objective
Using whole spine sagittal T2 MRI, we aimed to compare the severity and prevalence of disc degeneration (DD) in axial SpA patients vs the general population and to determine any association between spinal inflammation, structural changes, mobility and DD among SpA patients.

Methods
Two prospectively collected cohorts of SpA patients (n = 411) and the general population (n = 2007) were recruited. Eventually, 967 participants from the populational cohort and 304 participants from the SpA cohort were analysed. Two hundred and nineteen matched pairs were generated by propensity score matching. Imaging parameters, including Pfirrmann grading, disc herniation, high-intensity zone, Schmorl’s node, Modic change and anterior marrow change were studied and compared from C2/3 to L5/S1. DD was defined as Pfirrmann grade 4 or 5. Demographic factors, including age, sex and BMI, were collected. Multivariable linear regression was used to determine the association between spinal inflammation [Spondyloarthritis Research Consortium of Canada (SPARCC) spine MRI index], structural changes [modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS)] and mobility (BASMI) with lumbar Pfirrmann score.

Results
SpA patients had lower prevalence of DD (P &lt; 0.001). The disease stage-stratified regression model showed that SPARCC spinal MRI index was associated with higher lumbar Pfirrmann scores in early disease (β = 0.196, P = 0.044), whereas mSASSS was associated with lower lumbar Pfirrmann scores in later disease (β = −0.138, P = 0.038). Males had higher mSASSS (P &lt; 0.001) and lower odds of whole spine DD (odds ratio = 0.622, P = 0.028).

Conclusion
SpA patients had lower DD severity than the general population. Males had higher mSASSSs, and increased mSASSS at later disease was associated with less severe DD.
Enhancing cartilage regeneration and repair through bioactive and biomechanical modification of 3D acellular dermal matrix
Authors: Gao Wei , Cheng Tan , Tang Zhengya , Zhang Wenqiang , Xu Yong , Han Min , Zhou Guangdong , Tao Chunsheng , Xu Ning , Xia Huitang , Sun Weijie 
Published: February 2024

Abstract
Acellular dermal matrix (ADM) shows promise for cartilage regeneration and repair. However, an effective decellularization technique that removes cellular components while preserving the extracellular matrix, the transformation of 2D-ADM into a suitable 3D scaffold with porosity and the enhancement of bioactive and biomechanical properties in the 3D-ADM scaffold are yet to be fully addressed. In this study, we present an innovative decellularization method involving 0.125% trypsin and 0.5% SDS and a 1% Triton X-100 solution for preparing ADM and converting 2D-ADM into 3D-ADM scaffolds. These scaffolds exhibit favorable physicochemical properties, exceptional biocompatibility and significant potential for driving cartilage regeneration in vitro and in vivo. To further enhance the cartilage regeneration potential of 3D-ADM scaffolds, we incorporated porcine-derived small intestinal submucosa (SIS) for bioactivity and calcium sulfate hemihydrate (CSH) for biomechanical reinforcement. The resulting 3D-ADM+SIS scaffolds displayed heightened biological activity, while the 3D-ADM+CSH scaffolds notably bolstered biomechanical strength. Both scaffold types showed promise for cartilage regeneration and repair in vitro and in vivo, with considerable improvements observed in repairing cartilage defects within a rabbit articular cartilage model. In summary, this research introduces a versatile 3D-ADM scaffold with customizable bioactive and biomechanical properties, poised to revolutionize the field of cartilage regeneration.
Revisiting Event-Study Designs: Robust and Efficient Estimation
Authors: Borusyak Kirill , Jaravel Xavier , Spiess Jann 
Published: February 2024

Abstract
We develop a framework for difference-in-differences designs with staggered treatment adoption and heterogeneous causal effects. We show that conventional regression-based estimators fail to provide unbiased estimates of relevant estimands absent strong restrictions on treatment-effect homogeneity. We then derive the efficient estimator addressing this challenge, which takes an intuitive “imputation” form when treatment-effect heterogeneity is unrestricted. We characterize the asymptotic behaviour of the estimator, propose tools for inference, and develop tests for identifying assumptions. Our method applies with time-varying controls, in triple-difference designs, and with certain non-binary treatments. We show the practical relevance of our results in a simulation study and an application. Studying the consumption response to tax rebates in the U.S., we find that the notional marginal propensity to consume is between 8 and 11% in the first quarter—about half as large as benchmark estimates used to calibrate macroeconomic models—and predominantly occurs in the first month after the rebate.