bluuebunny/crossref_metadata_2025_split huggingface dataset:
#4031 VALIDATION OF THE GRAMS MODEL AS A PROGNOSTIC TOOL IN ADVANCED CHRONIC KIDNEY DISEASE (ACKD)
Authors: Collado Alba Temprado , Blandino Marisa Vidal , Cosano Gema Montilla , Gómez Cristina Andrades , Prieto Francisco Javier Toro 
Published: June 2023

Abstract

Background and Aims
Elderly patients with Advanced Chronic Kidney Disease (ACKD) present high cardiovascular comorbidity and increased mortality risk. The CKD-G4+ risk calculator was developed by Grams specifically for the population with CKD G4 and analyzes the risk of requiring kidney replacement therapy (KRT), cardiovascular events and death. Ours aims was validation of the Grams score as a prognostic tool in patients with CKD G4 aged over 65 years old in our hospital area.

Method
Descriptive and retrospective non-randomized study of a cohort of patients older than 65 years with CKD G4 attending for the first time for consultation during 2016 and 2017. Follow-up for 4 years or first clinical event defined as need for KRT, eGFR &lt; = 8 ml/min/1.72 m2 or death. Demographic and clinical data were collected for Grams model risk calculation (age, sex, CKD-EPI eGFR, urine albumin/creatinine ratio (CAC), systolic blood pressure (SBP), diabetes, smoking, cardiovascular history, non-fatal cardiovascular event (CHF, AMI, stroke) and death). SPSS statistical analysis.

Results
Of 663 patients who started follow-up in ACKD consultations in our hospital area between 2016 and 2017, 303 patients who met inclusion criteria were analyzed, 31 were excluded due to loss to follow-up. The clinical characteristics of the studied population are collected in Table 1. The results are collected in Table 2, they include predicted and observed incidences of death, KRT onset and cardiovascular events in 2 and 4 years from the first visit. Predictors used in this study included Grams calculator risk scores at 2 and 4 years (Grams-2 and Grams-4,respectively) for any KRT, cardiovascular events, and death.

Conclusion
In our cohort of patients with G4 ACKD older than 65 years old, the Grams score provides good discrimination to significantly predict the onset of KRT and cardiovascular events. The risk of death was moderately accurate and should be taken into account for care planning. Further external validation studies are required.
#4037 DEVELOPMENT OF AN EXPERT MODEL FOR THE DIAGNOSIS OF INHERITED KIDNEY DISEASES
Authors: de Arizón Leonor Fayos , Ramírez Elizabeth Romelia Viera , Leon Rafael , Teran Melissa Pilco , Furlano Monica , Vallverdu Montserrat , Guirado Lluis , Perera Alexandre , Torra Roser 
Published: June 2023

Abstract

Background and Aims
Inherited kidney diseases (IKDs) account for 10-20% of cases of Chronic Kidney Disease (CKD). Mutations in over 400 genes are known as drivers of more than 150 monogenic kidney disorders. Clinical diagnosis is often challenging as these disorders are characterized by numerous clinical features often shared by many diseases as well as very variable expression. Over the years, rare-disease databases such as OMIM and Orphanet have complied information to facilitate diagnosis of rare diseases. Based on these, The Human Phenotype Ontology (HPO) developed a standardized directory for phenotypes with phenotype-disease associations. New phenotype-driven gene-priorization tools such as Phenomizer have been developed in the recent years using these resources, however structural inaccuracies prevent a proper uptake of these tools. To date, no specific renal tool has been developed. The aim of the study was to develop an expert model for the diagnosis of IKDs based on phenotype semantic similarity distances such over Human Phenotype Annotations.

Method
Using MONDO ontology which encompasses HPO phenotypes, OMIM and Orphanet, HPO annotations corresponding to 196 IKDs were exported an analyzed. Based on literature, original HPO annotations for each IKD were manually curated (inaccurate terms were eliminated, new known HPOs were added and new annotations were generated in case they were missing). Frequencies were added to each annotation. Specific kidney data such as CKD stage, age at need of kidney replacement therapy and degree of proteinuria were included. Age of onset, prevalence, and specific gender sub analysis in case of diseases with X-linked inheritance was also integrated. All curated phenotype-disease associations were reviewed by European experts. Using MONDO annotations for each IKD as synthetic patients a statistical model with a friendly user web-based interface was developed in Java.

Results
Analysis of current ontologies by experts in the filed revealed that HPO terms associated to IKDs were inaccurate and non-specific. A new phenotype-driven tool fed with new curated terms and specific kidney data was found to be superior to current tools. Future contributions to ontologies such as HPO, MONDO and open data-sources with curated terms and updated classifications concerning IKDs will help to improving the diagnosis and characterization of IKDs.

Conclusion
Current phenotype-based gene prioritization tools and rare disease databases have contributed significantly to improving the clinical diagnosis of rare diseases. However, these tools are nonspecific and imprecise when focusing on a specific field such as IKDs. Curation of current annotations by experts in the field will greatly improve the accuracy of these tools and facilitate clinical diagnosis of IKDs.
#4046 ADVANCED CHRONIC KIDNEY DISEASE, PREOPERATIVE ISOMETRIC EXERCISE AND VASCULAR ACCESS: LET´S CLEAR THE DOUBTS
Authors: González Irati Tapia , Simó Vicent Esteve , Pallares Sara Ibañez , Guzmán Fátima Moreno , Oleas Diana , Julia Jorge Mario Alfonzo , Gallego Verónica Duarte , Potau Mónica Pou , Solé Anna Saurina , Serna Manel Ramírez de Arellano 
Published: June 2023

Abstract

Background and Aims
Arteriovenous fistula (AVF) is the best vascular access for hemodialysis. However, the role of preoperative isometric exercises in patients with advanced cronic kidney disease (CKD) who are candidates for AVF is unclear and not well reported in the literature. The main objetive of this study was to evaluate the effect of preoperative isometric exercise on vascular territory in advanced CKD prior to AVF creation.

Method
An 8 months prospective single-center study. We performed an isometric preoperative exercise program for 4 weeks on the non dominant arm (exercise arm) and was compared with the dominant arm (control arm) performing usual activity in our advanced CKD patients. Demographic data, upper limb muscle strength (ULMS), Doppler ultrasound (DUS) measurements (foream and distal cefalic vein (FCV, DCV) diameter, basilic vein (BV) diameter and depth, peak systolic velocities (PSVs) and diameters in the radial artery (RA) and braquial artery (BA), as well as percentage of patients candidates to AVF in both arm (exercise and control arm) according to Spanish Clinical Guidelines of Vascular Access (VA) and possible medical complications were analyzed.

Results
27 patients. 67,7%men. Mean age 70,3±10,4years. Main cardiovascular risk factors: HBP (93,5%) and DM (64,5%). Exercise arm was left side in 87,1%. A significant increase was observed in ULMS only in exercise arm at the end of study (23,8±8,7Kg vs 27, 6±9,5Kg, p = 0,001). Related to DUS measurements, there were not diffences between groups on vein caliber. However, a significant increase was observed in RA PSV (57,7±15,3 vs 62,6±17,4cm/seg, p = 0,044), BA diameter (4,6±0,6 vs 4,9±0,9mm, p = 0,029) and percentage of patients who could be candidates to AVF (70,4 vs 92,6%, p = 0,034) in exercise arm at the end of the study. We did not observe any related complications (pain, hypotension or muscle injury).

Conclusion
Preoperative isometric exercises would increase the percentage of performing a native AVF in those advanced CKD patients candidates for HD. Similarly, our results suggest that preoperative isometric exercise could be a useful tool to improve the vascular territory, mainly arterial, in our patients. However, more studies are required to confirm our results.
#4052 IMMUNOSUPPRESSIVE TREATMENT RESULTS IN PATIENTS WITH PRIMARY IGA NEPHROPATHY IN TURKIYE: A NATIONWIDE STUDY
Authors: Oruc Aysegul , Sumnu Abdullah , Turkmen Aydin , Basturk Taner , Cebeci Egemen , Turgutalp Kenan , Çetinkaya Hakkı , Kibar Muge Uzerk , Seyahi Nurhan , Tatar Erhan , Ergül Metin , Derİcİ Ülver , Ayli Deniz , Pınar Musa , Bakar Betul , Kazancioglu Rumeyza , Yildiz Abdulmecit , Dirim Ahmet Burak , Yilmaz Zulfikar , Turkmen Kultigin , Tunca Onur , Koc Mehmet , Kutlay Sim , Micozkadioglu Hasan , Azak Alper , Boztepe Burcu , Üstündağ Sedat , Ozturk Seda Safak , Unsal Abdülkadir , Karadağ Serhat , Sahin Gulizar Manga , Yenigun Ezgi Coskun , Eren Necmi , Gullulu Mustafa 
Published: June 2023

Abstract

Background and Aims
IgA nephropathy (IgAN) is the most common cause of primary glomerulonephritis in Turkey, as well as all over the world. Along with the frequent occurrence, deleterious renal outcome odds make treatment approaches important. Additionally, for high-risk individuals immunosuppressive treatment (IST) is recommended. However, studies to date revealed conflicting results regarding IST. Therefore, we aimed to investigate IST results among IgAN patients which is the leading primary glomerulonephritis in Turkiye.

Method
The data of 1656 IgAN patients in the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases Study Group (TSN-GOLD) were analyzed. A total of 506 primary IgAN patients (63.4% male, mean age 38.9±12.5 years) were included and divided into two groups according to treatment protocols as isolated corticosteroid (69.6%) and combined IST (30.4%) groups. The median follow-up duration was 24 (3-218) months.

Results
Remission (66.6% partial remission, 33.4% complete remission) was achieved in 70.6% of patients. Systolic and diastolic blood pressures, urea, creatinine, and proteinuria levels were lower, and eGFR levels were higher in responsive patients (Table 1). There was no difference between the treatment groups in terms of remission rates (p = 0.147) and remission types’ rates (p = 0.279). Remission rates were different between treatment subgroups. However, there was no difference between the treatment subgroups according to the remission types (p = 0.132) (Table 2). Complete remission was lower in the S1 and T1 categories (p = 0.003 and 0.039, respectively). The serious infection was higher in the combined IST group (17.1% vs 2.9%). The outcome data of 229 individuals was evaluated, 40 of 229 (17.5%) developed ESRD and 8 were dead. In the multivariate analysis, eGFR (OR 1.007, 95%CI 1.001-1.013, p = 0.020), proteinuria (OR 1.000, p = 0.009), MEST-C S1 (OR 1.912, 95%CI 1.216-3.005, p = 0.005), MEST-C T2 (OR 0.226, 95%CI 0.102-0.501, p = &lt; 0.001) were found to be significant regarding remission.

Conclusion
IST provides remission in high-risk IgAN patients but was associated with serious adverse events. The fact that the remission rates were similar between the treatment groups and that the complete remission rate was low in chronic changes supports the necessity of determining the treatment choice according to patient characteristics.
#4087 THE EFFECTS OF SOCIOECONOMIC STATUS ON MAJOR ADVERSE CARDIOVASCULAR EVENTS: A NATIONWIDE POPULATION-BASED COHORT STUDY
Authors: Bae Eun Hui , Lim Sang Yoeb , Kim Su In , Cho Da Hae , MA Seong Kwon , Kim Soo Wan 
Published: June 2023

Abstract

Background and Aims
Although multiple factors influence the risk of major adverse cardiovascular events (MACE), the effects of socioeconomic status on MACE in the presence and absence of renal dysfunction (RD) have not been comprehensively explored in South Korea.

Method
We examined the effects of socioeconomic status on MACE in individuals with and without RD. The data of 44,473 Koreans from 2008 to 2017 were obtained from the health care big data platform. Their socioeconomic status was assessed using a socioeconomic score (SES) based on their marital status, education, household income, and occupation. The incidence of myocardial infarction (MI), stroke, and death was compared according to five SES levels (0–4). Multiple linear regression analysis was used to evaluate the hazard ratios and 95% confidence intervals (CIs) for outcomes based on the participants’ SES.

Results
MI risk was only affected by education level. The participants’ income, education, and SES affected their stroke risk, whereas death was associated with all four socioeconomic factors. The incidence of stroke and death increased as SES worsened (from 0 to 4). SES was positively related to the risk of stroke and death in participants without RD. SES did not affect MI, stroke, or death in the participants with RD.

Conclusion
A low socioeconomic status is associated with the risk of stroke and death, especially in individuals without RD.
#4107 THE ROLE OF CYTOKINE ADSORBER IN REDUCTION INTERLEUKIN-6 LEVELS: A MATCHED CASE-CONTROL SINGLE CENTER ANALYSIS
Authors: Jerman Alexander , Gubensek Jakob , Berden Jernej , Peršič Vanja 
Published: June 2023

Abstract

Background and Aims
Extracorporeal cytokine adsorption is used with the aim to reduce pathogenic levels of cytokines in critically ill patients. Although technically and theoretically plausible, little convincing data exist to support wide use of cytokine adsorber in clinical practice. We aimed to retrospectively compare the time course of interleukin-6 (IL-6) in patients treated with or without the adsorber (CytoSorb®).

Method
In this retrospective, case-control study all patients treated between Jan 2017 and Dec 2021 in a medical ICU were screened and included, if at least two IL-6 measurements were available. Patients were divided to adsorber group and standard of care group based on the treatment received. Unmatched and matched groups were compared regarding IL-6, lactate, CRP, procalcitonin, and vasopressor demand at predefined time points. Mortality rates were also compared.

Results
We screened 3865 patients and included 52 patients in the adsorber group and 94 patients in the standard of care group. After matching, there were 21 patients in each group. Patients had similar age (53 (41, 68) vs. 61 (56, 67) years), ECMO was used in 24% (both groups) and renal replacement therapy in 100% of patients (both groups), baseline noradrenaline requirement (0.04 vs. 0.03 mg/kg/h), serum lactate (6.8 vs. 5.4 mmol/l), pH (7.27 vs. 7.21), CRP (182 vs 167 mg/l), and IL-6 (2441 vs. 2552 ng/l) were comparable between adsorber group and SOC group. There were no significant differences in the time course of IL-6, lactate, CRP, procalcitonin, and noradrenaline requirement between the groups. Two-day (33% vs. 28%) and ICU (57% vs. 62%) mortality and Kaplan-Meier survival curves (log-rank p = 0.9) were comparable.

Conclusion
In our matched case-control study, no difference in IL-6 or inflammatory parameters reduction, noradrenaline demand, or mortality was observed between patients, treated with adsorber (CytoSorb) or standard of care.
#4124 SEX AND CARDIOVASCULAR DISEASE IN STAGE G2-5 CKD PATIENTS
Authors: Mallamaci Francesca , Provenzano Pasquale Fabio , Parlongo Giovanna , Leonardis Daniela , Caridi Graziella , Marino Francesco , D'arrigo Graziella , Tripepi Giovanni Luigi , Zoccali Carmine 
Published: June 2023

Abstract

Background and Aims
Male sex is considered a major risk factor for cardiovascular (CV) disease in the general population, but the role of this factor in the high risk for CV disease in the pre-dialysis CKD population is still debated.

Methods
We tested the relationship between sex and fatal and non-fatal major CV events (myocardial infarction, heart failure, arrhythmia, angina, stroke, transient ischemic attack, peripheral vascular disease, major arterial or venous thrombotic episodes and sudden death) in a cohort including 759 stage 2-5 CKD consecutively recruited from 22 Nephrology units in southern Italy between October 2005 and September 2008. After the initial assessment, patients were followed up for a median time of 36 months (range 0.3–48 months).

Results
Four hundred fifty-five patients were males (60%). The proportion of smokers was about 4 times higher in males (71.4%) than in females (17.4%). Males and females differed in the prevalence of diabetes (38.5% versus 29.6%) and the frequency of background CV comorbidities (35.6% versus 19.7%, P&lt;0.001). Waist circumference (100.9±12.4 versus 96±14.1 cm), eGFR (37.5±13.4 versus 33±12.7 ml/min/1.73 m2), 24-hour urinary protein excretion (median: 0.7 g/24h, IQR: 0.2-1.6 g/24h versus 0.5, IQR: 0.2-1.2 g/24 h), and haemoglobin (13.4±1.9 versus 12.0±1.4 g/dL) were higher in males than in females. Serum phosphate (3.6±0.75 versus 3.9±0.75 mg/dL), hs-CRP (median; 2.2 mg/dl, IQR: 1-4.7mg/dl versus 2.8 mg/dl, IQR: 1.2-6.4 mg/dl) and total cholesterol (178.3±42.1 versus 198.8±45.6 mg/dl) were lower in males than in females. During follow-up, 42 patients died, and 118 had fatal and non-fatal CV events. On univariate Cox regression analyses, male gender failed to be associated with all-cause mortality but was strongly related to the incidence rate of fatal and non-fatal major CV events [HR 1.75, 95% CI: 1.18-2.60, P=0.006]. Data adjustment for a series of major potential confounders did not materially affect the strength of this relationship [HR:1.78, 95% CI: 1.03-3.09]. Further analysis testing the effect of age on major CV outcomes by gender showed an effect modification by this risk factor on the same outcome (P=0.037) because the hazard ratio of male versus female CV events increased progressively with ageing (Figure 1).

Conclusion
The excess risk for CV mortality by the male gender in the general population holds in stage G2-5 CKD patients. Age is a modifier for the excess risk for CV events in CKD patients because the risk excess of the male gender increases linearly across a wide age spectrum in CKD patients.
#4150 INTERNATIONAL COMPARISON AND TIME TRENDS OF FIRST KIDNEY TRANSPLANT RECIPIENT CHARACTERISTICS ACROSS EUROPE: A STUDY FROM THE ERA REGISTRY
Authors: Boenink Rianne , Kramer Anneke , Stel Vianda , Jager Kitty J , Ortiz Alberto 
Published: June 2023

Abstract

Background and Aims
Large international differences exist in the kidney transplantation (KT) rate across Europe. Countries might transplant a different group of end-stage kidney disease (ESKD) patients, but up to now an overview of recipient characteristics in first kidney transplant recipients in the different European countries is lacking. Therefore, the aim of this study was to give an overview as well as time trends in age, sex and primary renal disease (PRD) distribution of first kidney transplant recipients in Europe.

Method
The European Renal Association (ERA) Registry database was used to obtain data on adult patients receiving their first KT between 2010 and 2019 from 12 European countries. The numbers and percentages of recipients in each age, sex and PRD group were calculated by country, donor type and year. The first KT rate was calculated by dividing the number of first KTs by the adult general population counts and multiplied by one million.

Results
In total, 99,543 patients received a first KT between 2010 and 2019 for all countries combined. The percentage of recipients aged 65 years and older varied greatly between countries, with Bosnia and Herzegovina having the lowest percentage (1%), and the Netherlands, Spain and Norway having the highest percentages (&gt;25%). Similar results were found for deceased donor first KT, but for living donor first KT Spain was no longer among the countries with the highest percentage of recipients aged 65 years and older. The percentage of female recipients ranged between 33% in Austria and 38% in the Netherlands for total first KT, between 33% in Austria and 38% in Denmark for deceased donor first KT and between 30% in Austria and 39% in the United Kingdom for living donor first KT. When comparing the distribution of PRDs, Bosnia and Herzegovina had a relative high percentage of recipients with glomerulonephritis (35%), while Finland had a high percentage of recipients with diabetes mellitus (29%) and the Netherlands and Norway a high percentage of recipients with hypertension / renal vascular diseases (17% and 22% respectively). This distribution was similar for deceased and living donor first KT recipients. Over time, the most prominent change regarding the recipient characteristics was observed for the age of the recipient, with an increasing proportion of recipients aged 65 years and older from 18% in 2010 to 28% in 2019 for all countries combined, and with a similar trend observed in most countries.

Conclusion
We observed large differences in the recipient characteristics at first KT between European countries and over time, especially for the recipient age and PRD. These new insights in the distribution of recipient characteristics in the first KT population could elucidate which ESKD patients receive a KT.
#4151 POLYCYSTINS ARE REQUIRED FOR RENAL TUBULOINTERSTITIAL FIBROSIS
Authors: Xing Yufeng , Wu Ming , Ye Chaoyang 
Published: June 2023

Abstract

Background and Aims
Renal fibrosis is the common pathway of various chronic kidney diseases progressing to end stage renal failure. Polycystin-1 (encoded by PKD1 gene) and polycystin-2 (encoded by PKD2 gene) form a transmembrane complex and function as a stress sensor, which is located in the primary cilia. Polycystins are involved in the disease condition of different organs. Mutation of PKD genes causes autosomal dominant polycystic kidney disease and deletion of polycystin attenuates heart injury induced cardiac fibrosis. The role of polycystins in renal tubulointerstitial fibrosis is currently unclear.

Method
Unilateral ureteral obstruction (UUO), unilateral ischemia-reperfusion injury (UIRI), aristolochic acid or folic acid induced mouse models of renal fibrosis were established for this study.

Results
Here we showed that polycystin-2 is up-regulated in these three mouse models of renal fibrosis and tightly correlated with the expression of collagen-I in a time dependent manner. Treatment with triptolide inhibited the expression of polycystin-2 and pro-fibrotic markers in UUO and UIRI models. Moreover, triptolide or PKD2 siRNA inhibited the expression of polycystin-2 and pro-fibrotic markers in vitro. Using Pkd2 conditional knockout mice, we showed that genetic deletion of Pkd2 reduced the expression of pro-fibrotic markers in UUO kidneys. Polycystin-1 was also up-regulated in renal fibrotic models and conditional deletion of Pkd1 reduced the expression of pro-fibrotic markers in UUO or folic acid induced fibrotic kidneys. Furthermore, the expression of the methyltransferase EZH2 is positively correlated with the expression of polycystins in fibrotic kidneys. Conditional knockout of EZH2 attenuated the anti-fibrotic responses induced by Pkd1 deletion in UUO kidneys.

Conclusion
In conclusion, polycystins are up-regulated in fibrotic kidneys and promote renal tubulointerstitial fibrosis through up-regulation of EZH2, suggesting that primary cilia are required for renal tubulointerstitial fibrosis.