bluuebunny/crossref_metadata_2025_split huggingface dataset:
#3023 KARYOMEGALIC INTERSTITIAL NEPHRITIS SECONDARY TO FAN1 VARIANTS: CHARACTERIZATIONS AND OUTCOMES. RESULTS OF THE FAN1 STUDY GROUP
Authors: Michelle Clince , Elhassan Elhussein , Bleyer Anthony , Mcanallen Susan , Kidd Kendrah , Conlon Peter 
Published: June 2023

Abstract

Background and Aims
Karyomegalic Interstitial Nephritis (KIN) is a rare monogenic disorder caused by biallelic variants in Fanconi Anaemia-associated Nuclease 1 (FAN1), a gene involved in DNA repair. Less than 50 cases have been reported in the literature. Affected individuals suffer from kidney and pulmonary disease and increased susceptibility to cancer. In this international study, we report comprehensive clinical and genetic data on 32 individuals with FAN1-defined KIN.

Method
We performed a comprehensive literature review and an online survey to compile updated data of new and published cases via the FAN1 Study Group. Data collected included genotype, age at diagnosis, initial cause of presentation, kidney progression, transplantation status, and occurrence of cancer and pulmonary disease.

Results
Clinical and genetic data were obtained on 32 individuals, 20 previously unpublished. A total of 27 disease-causing variants in FAN1 were identified, predominantly protein-truncating alleles (see table 1). The median age of initial presentation was 39 years (range 1–69), and 26 (81%) were males. Kidney biopsy was performed in 15 individuals, where characteristic tubular karyomegaly was observed in all biopsies. Extrarenal manifestations were observed in 13/32 (40%) individuals and included liver dysfunction (9%), respiratory disease (22%) and malignancy (9%). At last assessment, 15 (46%) individuals progressed to end-stage kidney disease at an average age of 37.2 years (range 27 to 48 years). Eight (25%) individuals received a kidney transplant at a mean age of 45.1 years (range 34 to 62 years). 25% of individuals with a FAN1 variants developed a malignancy post-transplant. Overall mortality in this group was 12.5% (n = 4). 37.5% (n = 3) of transplant recipients died. One patient died from lung cancer 2.5 years post-transplant, another died from pulmonary aspergillosis 10 years post-transplant and a third died 10 years post-transplant due to interstitial lung disease. Post infectious respiratory failure was the cause of death for the remaining non-transplanted patient.

Conclusion
FAN1 variants result in a high burden of kidney failure and extrarenal comorbidities in this young population group. Incidence of malignancy was almost double that which has been reported in the general transplant population. This result may suggest that careful consideration and counselling should be provided to patients with FAN1 prior to transplantation given their potential for increased risk of malignancy and respiratory failure. In this study, international collaboration has provided new clinical and genetic data for 20 cases of FAN1 not previously published adding to our understanding of the natural history of a very rare disease. Please contact michelleclince@gmail.com if you have a case that you could contribute to our survey.
#3047 A NEW ADJUNCTIVE THERAPY WITH AVACOPAN FOR ANCA-ASSOCIATED VASCULITIS
Authors: Yoshii Ryuichi , Kajiwara Kengo , Matsushita Koki , Nakamura Tomohumi , Tomita Masao , Mukoyama Masashi 
Published: June 2023

Abstract

Background and Aims
Glucocorticoids have been the standard treatment for anti-neutrophil cytoplasm autoantibody-associated vasculitis (AAV). Avacopan, a complement C5a receptor inhibitor, has been approved for the treatment of AAV in Japan, the USA, Germany, and Austria. The use of avacopan is supported by evidence from trials demonstrating disease remission with limited use of glucocorticoids [1–3]. Avacopan is a new, promising adjunctive agent for standard induction therapy for AAV and may potentially reduce steroid use.

Method
We assessed treatment responses of patients with AAV who did not require dialysis between 2018 and 2023. Group 1 consisted of six adult patients who were treated with avacopan (30 mg, twice daily) plus reduced-dose prednisone, and Group 2 consisted of 14 patients who received high-dose prednisone. We compared the mean dose of prednisone, Birmingham Vasculitis Activity Score (BVAS), creatinine, urine protein/creatinine ratio (UPCR), and C-reactive protein (CRP) at weeks 0 and 12 between the two groups.

Results
The mean total prednisone dose of oral glucocorticoids at both weeks 0 and 12 was much lower in Group 1 than in Group 2 (p&lt;0.05). Mean BVAS, creatinine, UPCR, and CRP in Group 1 was comparable to Group 2. Steroid-related toxicity occurred in three of six (50%) patients in Group 1, and 5 of 14 (36%) patients in Group 2. Liver damage related to avacopan occurred in two of six (33%) patients in Group 1.

Conclusion
Herein, we describe practice observations on the use of avacopan in patients with AAV. While further studies are needed to confirm the efficacy of avacopan because the number of cases treated with avacopan is still lacking, our findings suggest that avacopan was beneficial in AAV patients because of the absence of steroid dependence.
#3062 ROLE OF ONLINE HEMODIAFILTRATION (OL-HDF) IN THE CONTINUOUS MEASUREMENT OF THE DIALYSIS DOSE
Authors: Alman Francisco Alonso , López Yanet Parodis , Ortega Sebastian Hillebrand , Vega Nicanor , Anton-Perez Gloria 
Published: June 2023

Abstract

Background and Aims
OL-HDF provides multiple advantages and benefits to patients compared to conventional high-flow hemodialysis (HFHD). Current monitors have a wide variety of biosensors and devices that provide continuous information on the patient's biological parameter and the efficiency of the dialysis session. Monofrequency bioimpedance (BIVA) provides information regarding the state of hydration (HE) and nutrition (EN) of our patients.

Method
Observational study in 493 patients, distributed in 8 dialysis centers and with a three different model of dialysis monitor. Demographic data, dialysis techniques and regimens, mean values for one month of ultrafiltration rate readings and KT are analyzed. The continuous measurement of DD is estimated by the ion dialysance biosensor. The OL-HDF technique is post-dilutional. The BIVA measurement is performed between 10 and 30 minutes after the end of the session. The Kt/V is estimated from the total TBW (V) by BIVA, using the Watson and Hume-Wiers formulas. Statistical analysis: descriptive, t-Student for statistics of groups and independent samples, Chi square for the association in cross tables.

Results
See Table 1.

Conclusion
Obtaining lower dialysis dose values in OL-HDF than in HFHD in some monitor models, despite longer dialysis times and greater dialyzer surface area, is probably due to dilution due to post- re-infusion and/or to the positioning of the dialysance sensors within the hydraulic circuit. This makes us think about the advisability of maintaining urea Kt/V measurements, by laboratory, to ensure adequate dialysis quality.
#3069 CIRCULATING SCLEROSTIN AFFECTS URINARY CALCIUM EXCRETION AMONG STONE FORMERS
Authors: Rodrigues Fernanda Guedes , Ormanji Milene Subtil , Pietrobom Igor , Matos Ana Cristina , de Borst Martin , Heilberg Ita Pfeferman 
Published: June 2023

Abstract

Background and Aims
Sclerostin plays an important role in bone metabolism and adipose tissue. Experimental studies suggest that sclerostin also influences urinary calcium excretion (Uca), but this relationship has not been addressed in humans. We aimed to study the association of UCa levels with serum sclerostin, bone mineral density (BMD) and body composition among stone-formers (SF).

Method
We retrieved data from patient records, including clinical characteristics, nutritional, biochemistry and hormonal data as well as BMD and body composition determined by dual energy X-ray absorptiometry. Serum sclerostin levels were measured using ELISA kits. Determinants of UCa were studied using multivariate linear regression.

Results
A total of 107 SF (35.8 ± 9.3 years, 54% male) with eGFR of 99.8 ± 14.5 mL/min/1.73 were included. The subjects were divided by sex and clustered into tertiles according to UCa as shown in Table 1. Men in the highest UCa tertile had higher body mass index (BMI) and serum sclerostin levels, were more likely to have hypertension and metabolic syndrome, had lower lean mass, a trend towards higher fat mass (p = 0.06), and no statistical differences in BMD. Women in the highest UCa tertile had higher BMI and a trend towards higher serum sclerostin levels (p = 0.06), were more likely to have hypertension and metabolic syndrome, with no differences in BMD. Sclerostin was positively correlated with fat mass (r = 0.38, p = 0.004) and inversely correlated with lean mass (r = -0.32, p = 0.01) among men, but not among women. To further adjust for the influence of weight load on BMD for both sexes, the latter was also expressed as a ratio per BMI (BMD/BMI). Although lumbar spine BMD/BMI was inversely associated with UCa (β -0.26, p = 0.01) in univariate analysis, it lost significance in the multivariate model (Table 2). Hypertension, metabolic syndrome and serum sclerostin were independent determinants of UCa (Table 2).

Conclusion
Present findings disclosed that serum sclerostin, hypertension and metabolic syndrome were independent determinants of urinary calcium in stone formers. These data suggest that in addition to the hormones traditionally thought to alter calcium reabsorption in the kidney, sclerostin may play a significant additional role, warranting further intervention studies in order to test potential medication strategies to reduce calciuria in this population.
#3091 A STUDY FOR HOSPITALIZED PATIENTS WITH COVID-19 WITH/WITHOUT IMMUNOSUPPRESSION AND RENAL FAILURE: DOES THE SURVIVAL RATE CHANGE?
Authors: Vakiani Styliani , Triantafyllidou Maria , Katsanos Dimitris , Lakoniti Klaoudia , Vakianis Panteleimon 
Published: June 2023

Abstract

Background and Aims
Introduction The purpose of our research topic was to study the survival of immunocompromised or non-immunosuppressed patients as well as patients with Chronic Kidney Disease who were hospitalized due to covid-19 infection during a two-year period (2020-2022).

Method
Material and Methods A total of 1270 patients (of whom 570 were women) with a mean age of 63 years (+/- 24 years) were studied. From these, 1000 patients were without immunosuppression and 270 were theoretically and practically immunosuppressed, including 30 patients with Chronic Kidney Disease and of these 10 were with end-stage Chronic Kidney Disease. It is important to mention that 87 patients had to be hospitalized in the ICU, of which 74 died. The rest were hospitalized in the Biocontainment Unit, where 92 deaths were observed. 100 patients without immunosuppression died and 66 with immunosuppression. From the patients with End-Stage Chronic Kidney Disease, 3 died, theoretically falling into the immunocompromised category. The treatment protocol included all the therapeutic measures recommended by CCD.

Results
There was no statistically significant difference betweem the rate of death in immunocompromised patients and those who belonged in non immunocompromised group. However, there was a simple difference in deaths (66 out of 270 immunosuppresed patients) and 100 in non-immunocompromised patients, while there was also a difference in their need for hospitalization in the ICU and the Unit for Special Infections, but not statistically significant.

Conclusion
In conclusion, it appears that the immunocompromised are more prone to severe diseases having poor outcome. Furthermore, it was observed that patients with End Stage Chronic Kidney Disease had a fairly low mortality rate even though they are considered immunocompromised.
#3103 OMENTIN-1 AND SUBCLINICAL ATHEROSCLEROSIS IN ESKD PATIENTS UNDERGOING HEMODIALYSIS AND IN KIDNEY TRANSPLANT RECIPIENTS
Authors: Zicarelli Mariateresa , Greco Marta , Arcidiacono Valentina , Presta Pierangela , Caglioti Alfredo , Andreucci Michele , Dragone Francesco , Foti Daniela , Coppolino Giuseppe , Bolignano Davide 
Published: June 2023

Abstract

Background and Aims
In end-stage kidney disease (ESKD) patients, atherosclerosis is a key-player for cardiovascular risk, being influenced by endothelial dysfunction, inflammation and mineral dysmetabolism. Omentin-1 is an adipokine involved in various pathological conditions (diabetes, metabolic syndrome, obesity), and has recently been studied as a prognostic factor for atherosclerosis progression and mortality in the general population. In this study, we evaluated the possible relationships between Omentin-1 and incipient carotid atherosclerosis in the clinical setting of ESKD.

Method
Circulating blood Omentin-1 levels were measured in a cohort of 77 asymptomatic ESKD patients (40 kidney transplant recipients, Ktx and 37 chronic hemodialysis patients, HD) and in 30 healthy controls. Carotid intima-media thickness (cIMT) was measured before a single HD session or before a Ktx outpatient visit. Pathological cIMT was defined for mean values &gt; 0.9 mm and/or an unilateral cIMT above the 75th percentile.

Results
Omentin-1 levels were higher in the entire ESKD cohort than in healthy controls (324 [90.3-770] vs. 110 [35.4-240.9] pg/ml; p = 0.03). Ktx patients had lower levels than HD patients (p = 0.01) while higher Omentin-1 levels were found in HD patients (474.9 [197.2-1432.1] ng/ml) compared to both healthy subjects (p = 0.009) and Ktx (p = 0.01). Mean cIMT in all ESKD was 0.78 ± 0.32 mm. 36 patients (46.7%) with pathological cIMT values displayed lower Omentin-1 levels as compared with others (168.7 [51.1-457.8] vs 474.9 [197.2-1432.1] and p = 0.004). Multivariate correlations analyses indicated Omentin-1 as the stronger independent predictor of carotid atherosclerosis (β-0.687; p = 0.03) in the whole cohort, even more than age, total cholesterol and diastolic BP.

Conclusion
In ESKD patients, Omentin-1 reflects the severity of carotid atherosclerosis independently from traditional confounders and may serve as an additive biomarker for risk prediction and risk stratification. Future studies are awaited to confirm these preliminary findings in larger cohorts.
#3108 ASSOCIATION BETWEEN INCIDENT DEPRESSION AND CLINICAL OUTCOMES IN PATIENTS WITH CHRONIC KIDNEY DISEASE
Authors: Zhu Nanbo , Virtanen Suvi , Xu Hong , Carrero Juan Jesus , Chang Zheng 
Published: June 2023

Abstract

Background and Aims
Depression is highly prevalent and related to increased morbidity and mortality in patients on dialysis, but less is known among patients with earlier stages of CKD. This study investigated the associations between depression and clinical outcomes in patients with CKD not receiving dialysis.

Method
We identified 157 398 adults with CKD stages 3–5 not previously diagnosed with depression from the Stockholm CREAtinine Measurements (SCREAM) project. The primary outcomes included hospitalization, CKD progression (&gt;40% decline in eGFR, initiation of kidney replacement therapy, or death due to CKD), major adverse cardiovascular events (MACE; myocardial infarction, stroke, or cardiovascular death), and all-cause mortality. Survival analyses were used to estimate the associations between incident depression and adverse health outcomes, adjusting for socio-demographics, kidney disease severity, healthcare utilization, comorbidities, and concurrent use of medications.

Results
During a median follow-up of 5.1 (interquartile range: 2.3–8.5) years, 12 712 (8.1%) patients received an incident diagnosis of depression. A total of 634 471 hospitalizations (4,600 935 hospitalized days), 42 866 MACEs, and 66,635 deaths were recorded, and 9 795 individuals met the criteria for CKD progression. In the multivariable-adjusted analyses, incident depression was associated with an elevated rate of hospitalized days (rate ratio: 1.77, 95% confidence interval [CI]: 1.71–1.83), as well as an increased rate of CKD progression (hazard ratio [HR]: 1.38, 95% CI: 1.28–1.48), MACE (HR: 1.22, 95% CI: 1.18–1.27), and all-cause mortality (HR: 1.41 95% CI: 1.37–1.45). The association with CKD progression was more evident after one year of depression diagnosis (HR: 1.47, 95% CI: 1.36–1.59). Results were consistent across a range of sensitivity analyses.

Conclusion
Among patients with non-dialysis-dependent CKD stages 3–5, incident depression is associated with poor prognosis, including hospitalization, CKD progression, MACE, and all-cause mortality.
#3115 THE ASSOCIATION OF FIBROBLAST GROWTH FACTOR-23 AND CARDIORENAL SYNDROME IN PATIENTS WITH DIABETES MELLITUS
Authors: Vasilkova Volha , Pchelin Ivan , Mokhort Tatiana 
Published: June 2023

Abstract

Background and Aims
Previous studies suggest that interactions between the heart and the kidney can contribute to the progressive dysfunction of both organs. Recently, there has been an increase in the prevalence of cardiovascular disease (CVD) and chronic kidney disease (CKD) due to increasing diabetes mellitus rate. It is known that fibroblast growth factor-23 (FGF-23) has been found to be related to kidney metabolism especially as a biomarker and a key factor to be used in the kidney field. The aim of our study was to assess potential therapeutic approaches of the use of FGF-23 in clinical settings as a potential circulating biomarker for CVD and CKD.

Method
A total of 155 patients with DT2, aged 34 to 84 years (60 [60;72]), were examined. Control group included 94 healthy people the same age. All patients underwent standard clinical and laboratory examination, with an assessment of the levels of FGF-23 in baseline plasma samples. Renal function was assessed based on the levels of serum creatinine, cystatin C, eGFR, which was calculated according to the CKD-EPI formula, and albuminuria, which was assessed as albumin/creatinine ratio (A/C). An echocardiographic examination was conducted according to the standard protocol with the calculation of dimensional, volume and speed characteristics.

Results
The levels of FGF-23 were significantly higher in DT2 patients with CKD 5 in comparison with stages 1–4. There were positive strong significant association between FGF-23 and creatinine (r = 0.71, p&lt;0.001). In both unadjusted and adjusted analyses, probability of decreased eGFR was associated strongly with FGF-23 (COR; 95% CI 1.890; 1.362-2.622, p&lt;0,001). When evaluating the dependence of the probability of decreased eGFR on the FGF-23 using the ROC analysis, the cut-off value of FGF-23 was 0,9 pmol/l. The sensitivity and specificity of the method were 75.3% and 74.5%, respectively (AUC 0.832±0.035 with 95% CI: 0.764-0.901, p&lt;0.001).
DT2 patients with left ventricular hypertrophy (LVH) were characterized by higher levels of FGF-23 (2,45 [0,65;7,43] vs 0,57 [0,19;2,19] pmol/l in control group). DT2 patients with LVH and natriuretic peptides levels ≥ 35 pg/ml for BNP and/or ≥125 ng/ml for NT-proBNP had significantly high concentrations of FGF-23 than patients with normal levels of natriuretic peptides (3,22 [1,09;9,55] vs. 0,52 [0,27;1,08] pmol/l, p&lt;0,0001). In order to assess the diagnostic significance of the FGF-23 for predicting left ventricular mass index (LVMI) thickening and increasing the proBNP concentration, a ROC analysis was performed. Thus, at the level of FGF-23 (AUC-0.702) = 0.9 pmol/l, the sensitivity and specificity for LVMI thickening were 66.9% and 67.3%.

Conclusion
Our study identifies FGF-23 as a promising target of novel therapeutic interventions in cardiorenal syndrome, which should be investigated in future clinical studies.
#3125 EXTERNAL VALIDATION OF A NOVEL MULTIMARKER GFR ESTIMATING EQUATION
Authors: Tio Maria Clarissa , Zhu Xiaoqian , Lirette Seth , Rule Andrew , Butler Kenneth , Hall Michael , Dossabhoy Neville , Mosely Thomas , Shafi Tariq 
Published: June 2023

Abstract

Background and Aims
The use of multiple markers may improve the accuracy in glomerular filtration rate (GFR) estimation especially when the interpretation of creatinine and cystatin C is limited. We sought to externally validate a recently developed multi-marker nuclear magnetic resonance (NMR)-based estimated GFR equation (eGFR-NMR) using the biracial Genetic Epidemiology Network of Arteriopathy cohort.

Method
We included 224 sex, race/ethnicity, and mGFR-category-matched participants. GFR was measured using urinary clearance of iothalamate (mGFR). We calculated eGFR-NMR using serum creatinine, valine, myo-inositol, and cystatin C, age, and sex. We compared the reliability of eGFR-NMR with current eGFR equations (2021 Chronic Kidney Disease Epidemiology Collaboration equations for creatinine [eGFR-Cr] and creatinine with cystatin C [eGFR-Cr-CysC]) using median bias, precision, and accuracy metrics. In particular, we evaluated its performance in age, sex, and race subgroups.

Results
In the overall cohort, mean age was 63 (±8) years, 54% were females, 49% were Black individuals, and mean mGFR was 78.7 (±24.3) ml/min/1.73 m2. eGFR-NMR overestimated mGFR by 2 mL/min/1.73 m2 (95% CI, 4 to 0.7) while eGFR-Cr-CysC underestimated mGFR by −5 mL/min/1.73 m2 (95% CI, −2 to −7). All equations had acceptable accuracy metrics. When stratified by age, sex, and race, eGFR-NMR performed the best among Black males age &lt;65 years compared to current equations (Figure 1). In this subgroup, eGFR-NMR was unbiased (bias, 2mL/min/1.73 m2 [95% CI, -3 to 10]) compared to substantial biases of eGFR-Cr (bias, 17 mL/min/1.73 m2 [95% CI, 9 to 24]) and eGFR-Cr-CysC (bias, 15 mL/min/1.73 m2 [95% CI, 6 to 20]). In other subgroups, measures of accuracy for eGFR-NMR, eGFR-Cr, eGFR-Cr-CysC were generally similar.

Conclusion
eGFR-NMR can be used to estimate mGFR and was more accurate than CKD-EPI equations among Black males age &lt;65 years.