bluuebunny/crossref_metadata_2025_split huggingface dataset:
#5353 THE IDIOPATIC HYPOCRYOGLOBULINEMIA AS AN EMERGING MEMBRANOPROLIPHERATIVE GLOMERULONEPHRITIS
Authors: Longo Roberta , Ceruti Cecilia , Marchisio Martina , Morrone Marco , Caruso Nunziante , Guarino Annalisa , Fenoglio Roberta , Sciascia Savino , Roccatello Dario 
Published: June 2023

Abstract

Background and Aims
A considerable number of patients with high clinical suspicion for cryoglobulinaemic vasculitis either show negative results for the detection of cryoglobulins or show only trace amounts which cannot be characterized for composition. We aimed at establishing whether the failure to detect or the detection of trace amounts of cryoglobulin with conventional methods either identifies a peculiar subset of low level cryoglobulinaemia (from now on hypocryoglobulinaemia) or represents a separate entity.

Method
Using a modified precipitation technique in hypo-ionic medium, we prospectively identified between 2008 and 2021 237 patients (median age 60.8 years [22-97], 137 females) having &lt;0.5% cryocrit and clinical suspicion of autoimmune disorder

Results
54 out of 237 patients (22.7%) had a history of HCV infection. 169 out of 237 patients (71%) had an established underlying disease, while 68 patients (28.6%) (median age 62.9 years [29-93], 35 females) did not show either laboratory markers or clinical symptoms consonant with an underlying aetiology. These 68 cases with only trace amounts of cryoglobulins were defined as having a putatively idiopathic hypocryoglobulinaemia. Nineteen of these 68 patients (27.9%) had a history of HCV infection. Twenty-four patients out of 68 (35.3%) were positive for rheumatoid factor (RF), while 25 (36.7%) patients had signs of complement consumption, and 36 (52.9%) had increased inflammatory indexes. Seven patients only had arthralgia and constitutional symptoms while 61 out of 68 (89.7%) presented with at least one of the three cardinal signs of cryoglobulinaemic vasculitis including skin lesions, peripheral nerve involvement, and glomerulonephritis. Seventy-five percent of the subjects had type III hypocryoglobulins. In patients with hypocryoglobulinaemia the histologic features of glomerulonephritis (also examined by electron microscopy) resembled those of mixed cryoglobulinaemia-associated glomerulonephritis

Conclusion
In conclusion, hypocryoglobulins are often polyclonal and are mainly unrelated to HCV infection. Patients who present high clinical suspicion for vasculitis, especially glomerulonephritis and yet test negative for cryoglobulinaemia detected by standard techniques, could require deeper investigation even in the absence of HCV infection, RF activity or signs of complement consumption.
#5367 CLINICAL SIGNIFICANCE OF NORMO-HYDRATION STATE IN PERITONEAL DIALYSIS PATIENTS
Authors: Hashmi Amna , Szabo Reka P , Varga Istvan , Kardos Laszlo , Balla Jozsef 
Published: June 2023

Abstract

Background and Aims
The assessment of fluid status in dialysis patients is of paramount importance. Managing persistent volume overload and instability during dialysis is a significant challenge, with concomitant symptomatic and asymptomatic intradialytic hypotension (IDH) that results in dialysis-induced myocardial ischemia (cardiac stunning) and long patient recovery times.Our aim was to estimate the status of hydration in our peritoneal dialysis population by body composition monitoring (BCM) device to modify our pharmacological and dialysis policy and to evaluate echocardiographic parameters of the RV functions in patients with end-stage renal disease (ESRD) undergoing PD.

Method
We used a Fresenius Body Composition Monitor (BCM), a whole-body bioimpedance spectroscopy (50 frequencies, 5–1,000 kHz), to assess the body composition of 35 patients on peritoneal dialysis in our centre..We measured body weight, 24 h diuresis and performed a BCM session. Analysed results of echocardiography focused on RV (tricuspid annular plane systolic excursion (TAPSE). As per statistics, comparisons between 2 groups done by students t test, Mann Whitney U test or Wilcoxon test. Correlation between data was done by Pearson or spearman correlation. And lastly categorical variables were assessed by fisher's exact, and Chi squared test.

Results
Average age was 47 years old (SD 9,45 t-test p = 0,1154:). Gender distribution was 54,3% of our patients were female. 8,6% of our patients were anuric, 91,4% had residual urine (more than 1 litre). TAPSE value were average 23,17 mm (±4,5).We measured over-hydration in 8 cases by BCM that followed dialysis policy modification. In 8 PD patients we measured more than 2 L volume overload (VO) with BCM followed by dialysis policy modification (fluid intake restriction, diuretic dose increasing, frequent solution exchange).

Conclusion
Our data raised the importance of regular echocardiography to identify it. More attention should be focused on improving BP and volume control and identifying treatment strategies that effectively lower further deterioration in PD patients. One way of doing this is monitoring with BCM. With this clinician can manage persistent overload in CAPD patients, to better modify the dialysis regime, restrict fluid intake and use diuretics, to all in all preserve LV and RV function, and to prevent deterioration of the peritoneal membrane, and most importantly improve their survival.
#5386 ONCO-NEPHROLOGY: THE ROLE OF KIDNEY BIOPSY IN THE MANAGEMENT OF SIDE EFFECTS OF TARGETED THERAPIES
Authors: Caruso Nunziante , Lalloni Stefania , Marchisio Martina , Morrone Marco , Rabajoli Gianluca , Careddu Andrea , Geraci Giovanni , Fenoglio Roberta , Sciascia Savino , Roccatello Dario 
Published: June 2023

Abstract

Background and Aims
The introduction of innovative therapies, resulting from revisiting cancer as a disease of the immune system, has changed the scenario of complications. These new classes of drugs, such as targeted therapies and immune checkpoint inhibitors, assure substantial advantages in cancer therapy, despite some side affecting various organs, including the kidney. Histological evaluations of kidney disorders induced by targeted/immunotherapy are limited.

Method
In this study we examined the histological features of patients treated with new cancer agents who underwent kidney biopsy for new onset kidney failure and/or urinary abnormalities.

Results
The cohort included 30 adult patients. The most frequently administered therapies were immunotherapy (30%), targeted therapy (26.7%), immunotherapy plus targeted therapy (13.3%), immunotherapy plus chemotherapy (13.3%), targeted therapy plus chemotherapy (16.7%). The most common histological finding was tubular interstitial nephritis (30%) that was associated with acute tubular necrosis in 4 cases, and thrombotic microangiopathy (23.3%). After kidney biopsy, 16 of the 30 patients were treated according to the histological diagnosis. Fourteen patients were treated with steroids. One patient with membranous nephropathy was treated with a single dose of rituximab. A patient with severe thrombotic microangiopathy requiring dialysis received a treatment with eculizumab for 3 months. Overall some renal response was obtained in all patients treated with glucocorticoids, while complete kidney response was achieved in the patient treated with rituximab. Cancer treatment was resumed without change in 21 out of 30 patients.

Conclusion
Kidney biopsy is critical for the management of kidney toxicities and should be strongly encouraged for patients showing adverse kidney effects of novel cancer agents.
#5394 PHYSICAL ACTIVITY AND THE RISK OF DEMENTIA IN END-STAGE RENAL DISEASE PATIENTS WITH HEMODIALYSIS: A NATIONWIDE POPULATION-BASED STUDY
Authors: Choi Hong Sang , Kim Minah , Oh Tae Ryon , Suh Sang Heon , Kim Chang Seong , Bae Eun Hui , MA Seong Kwon , Kim Soo Wan 
Published: June 2023

Abstract

Background and Aims
Several studies have reported that dementia has a high prevalence in patients with end-stage renal disease (ESRD) patients. However, the relationship between physical activity and the risk of developing dementia has not been elucidated in ESRD patients with hemodialysis.

Method
A total 19,877 hemodialysis patients aged ≥ 40 years who underwent health check-ups were identified from the Korean National Health Insurance Service database and were analysed in this study. Individuals with physical activities were defined as meeting following criteria: (i) three or more days/week of vigorous activity of at least 20 min per day or (ii) five or more days/week of moderate-intensity activity of at least 30 min per day. The occurrence of dementia was monitored through the end of 2018 by the ICD-10 codes.

Results
During the 4.6-year follow-up, 1,755 patients with hemodialysis developed dementia. Physical activity was associated with the lower risk of any dementia (hazard ratio [HR], 0.779, 95% confidence interval (CI), 0.7-0.867) even after adjusting for several confounding factors. Risk of developing dementia was still lower in hemodialysis patients with physical activity when we analyse the risk of Alzheimer's dementia and vascular dementia separately (HR 0.817 and 0.685, respectively). In subgroup analyses, the risk of dementia tended to lower in hemodialysis patients with physical activity than without physical activity in all subgroups.

Conclusion
Physical activity is associated the lower risk of developing dementia in ESRD patients with hemodialysis.
#5441 EVIDENCE THAT CHAPERONE 4-PBA TREATMENT ALLEVIATES THE RENAL PHENOTYPE IN ALPORT SYNDROME MOUSE MODELS
Authors: Ioannou Pavlos , Odiatis Christoforos , Antoniadou Kyriaki , Pieri Myrtani , Papagregoriou Gregory , Malatras Apostolos , Samiotaki Martina , Horaček Matija , Ljubanovic Danica Galesic , Stylianou Kostas , Deltas Constantinos 
Published: June 2023

Abstract

Background and Aims
Alport Syndrome (AS) is a severe inherited glomerulopathy caused by mutations in the genes encoding the α-chains of type IV collagen, the most abundant component of the glomerular basement membrane (GBM). Alport patients lack effective therapies beyond blockade of the renin-angiotensin system). This work describes the repurposing of two FDA-approved chemical chaperones (4-PBA and TUDCA) to rescue two AS mouse models: a knock-in and a compound heterozygous model bearing the Col4a3-p.Gly1332Glu mutation, recapitulating the most common mutation found in Cypriot patients.

Method
In either a short-term or long-term treatment, AS and wild type (WT) mice received chaperones or vehicle daily. To examine the biochemical and histological effects of chaperones on treated mice, kidney, blood, and urine samples were collected after therapy.

Results
Electron microscopy studies showed that the GBM of the 4-PBA treated AS mice after the long-term treatment has a considerable improvement in morphology, compared with vehicle-treated or TUDCA-treated AS mice. Importantly, EM measurements displayed a significant (p-value&lt;0.0001) reduction of lesions and a decline of the lesions-severity in the GBM of 4-PBA treated AS mice. No adverse effects were noted in the GBM of the chaperone-treated wild-type mice. Also, the interstitial fibrosis, global and segmental glomerulosclerosis were ameliorated in the 4-PBA treated AS mice. Additionally, the treatment with 4-PBA maintained proteinuria and hematuria at low levels in AS mice. Importantly the de novo 35 kDa Col4a3 fragment which was previously detected in non-treated AS mice, was reduced after treatment with 4-PBA.

Conclusion
Together, these results suggest a therapeutic potential for the 4-PBA agent in combating renal dysfunction in AS.
#5443 ASSOCIATION OF SERUM CALCIUM-TO-MAGNESIUM RATIO WITH CARDIOVASCULAR AND CEREBRAL VASCULAR DISEASES IN DIALYSIS PATIENTS
Authors: Lee Sua , Lee Soyoung , Kim Kyeong Min , Hong Yu Ah , Park Hoon Suk , Shin Jong Ho , Choi Bum Soon , Kim Byung Soo , Ban Tae Hyun 
Published: June 2023

Abstract

Background and Aims
Cardiovascular disease (CVD) and cerebral vascular accident (CVA) are the important complications and the leading causes of death in dialysis patients. High serum calcium (Ca) level promotes the clotting cascades and vascular calcification, which leads to CVD and CVA. Magnesium (Mg) is a well-known Ca blocker, inhibiting the formation of thrombin. An imbalance between serum Ca and Mg levels has been reported as an associated factor with CVD and mortality. This study investigated the associations of serum Ca-Mg ratio with CVD and CVA in dialysis patients.

Method
We conducted a cross-sectional analysis using baseline data from a multicenter prospective cohort for dialysis patients in South Korea. A total of 860 patients were divided into tertile groups according to serum Ca-Mg ratio levels. Patients with serum Ca-Mg ratio less than 3.28 were classified as the low group, and patients with serum Ca-Mg ratio more than 3.87 were classified as the high group, and the rest were classified as the middle group. Serum Ca level was calculated and applied as corrected Ca level considering serum albumin level. The associations of serum Ca-Mg ratio with prevalence of acute coronary syndrome (ACS), congestive heart failure, aortic artery calcification, and CVA were assessed.

Results
The average age of all patients was 59.1±12.1 years, and the average duration of dialysis was 7.9±5.9 years, and the duration of the low group was shorter than those of other groups. The high group had higher prevalence of peripheral vascular disease (P = 0.004). Serum albumin, phosphorus and Mg levels in the high group were lower than other groups (P&lt;0.001). The prevalence of ACS showed higher in the high group than in other groups (P = 0.017). The prevalence of cerebral infarction showed higher in the high group than the low group (P = 0.016). In multivariable logistic regression analysis, higher Ca-Mg ratio was independently associated with the prevalence of ACS (Odds ratio 1.321, 95% confidential interval 1.009-1.728, P = 0.043) and cerebral infarction (Odds ratio 1.392, 95% confidential interval 1.002-1.896, P = 0.036).

Conclusion
A high Ca-Mg ratio was associated with the development of ACS and cerebral infarction. If the optimal cut-off of Ca-Mg ratio is determined, it can be used as a predictive marker for ACS and cerebral infarction.
#5448 INCIDENCE OF ACUTE PULMONARY EDEMA AFTER THE SYSTEMATIC USE OF ULTRASOUND B-LINES
Authors: Urso Alessandra , Mallamaci Francesca , Tripepi Rocco , Panuccio Vincenzo Antonio 
Published: June 2023

Abstract

Background and Aims
Acute pulmonary edema (APE) due to fluid overload is considered the most feared complication in hemodialysis patients. Various diagnostic tests have been proposed to assess fluid status in patients with end stage kidney failure (ESKF) and among these lung ultrasound (by measuring the number of B-Lines) is emerging as a promising tool to identify pulmonary congestion in this patient-population.

Method
In the setting of a retrospective study, we pragmatically compared the incidence rate of APE in our center before and after the implementation of lung ultrasound as a routine practice in our unit. Two periods were considered. A pre-implementation period [from 01/01/2007 to 31/12/2008, total person-time: 1913 months/patient, n = 98 patients (Group 1), 85 prevalent and 13 incident patients] and a post-implementation period [from 01/01/2017 to 31/12/2018, total person-time: 2061 months/patient, n = 108 patients (Group 2), 81 prevalent and 27 incident patients]. By accurately reviewing the electronic medical records, all episodes of APE were counted, i.e. all episodes characterized by sudden or worsening dyspnea associated with signs of salt and water overload, confirmed by chest auscultation or chest X-Ray, which required an additional dialysis session and excluding events due to infectious or irritating problems.

Results
The two groups (Group 1 vs Group 2) were quite similar between them as for age (64±15 vs 67±14 years, p = 0.06), proportion of males (65% vs 62%, p = 0.37) and median dialysis vintage [40 months (interquartile Range, IQR: 16–93 months) vs 46 months (IQR: 12–92), p = 0.76]. The two groups did not differ as for diabetes (21% vs 25%, P = 0.33), hypertension (75% vs 71%, p = 0.36), and smoking habit (29% vs 28%, p = 0.51). Of note, the proportion of patients with background cardiovascular comorbidities was significantly higher in patients of Group 2 (enrolled in the post-implementation period) than in those of Group 1 (31% vs 19%, p = 0.04]. A total of 37 APE episodes in 18 patients (from 1 to 4 episodes per patient) were identified in patients of Group 1 vs 7 APE episodes in 5 patients (from 1 to 2 episodes per patient) in those of Group 2. The incidence rate of APE was 82% lower in patients during the post-implementation period (4 episodes per 100 patients/year, 95% CI: 1–8) than in those during the post implementation period (23 episodes per 100 patient/year, 95% CI: 17–32) (incidence rate ratio: 0.18, 95% CI: 0.10-0.29, p&lt;0.001).

Conclusion
The systematic use of lung ultrasound (simple, easy to learn, rapid and non-invasive method, easily performed at the patient's bed) in every day clinical practice drastically reduced (-82%) the episodes of APE in hemodialysis patients. Further observational and interventional studies are needed to confirm these results.
#5478 REAL-WORLD SIGNS AND SYMPTOMS AT DIAGNOSIS IN PATIENTS WITH C3 GLOMERULOPATHY - INTERIM RESULTS FROM A MULTI-COUNTRY STUDY
Authors: Proudfoot Clare , Pannagl Katharina , Nguyen Jennifer , King Andrea , Murphy Kathleen , Decourcy Jonathan , Lafayette Richard 
Published: June 2023

Abstract

Background and Aims
C3 glomerulopathy (C3G) is a rare form of glomerulonephritis, with an estimated incidence of 1 – 2 per million per year. C3G is associated with a high risk of disease progression with approximately 50% of patients reaching kidney failure within 10 years of diagnosis. Common signs and symptoms include proteinuria, hematuria, edema and hypertension. The aim of this analysis was to better understand the clinical characteristics of C3G patients from the US, Europe and Asia, at the time of diagnosis.

Method
An analysis was conducted using interim data from the Adelphi C3G Disease Specific Programme (DSP), a cross-sectional survey of C3G-treating nephrologists in US, EU5 (France, Germany, Italy, Spain, UK), China and Japan (study ongoing since August 2022; interim analysis based on data until November). Nephrologists completed structured forms administered via online links for consecutive patients presenting with C3G. The forms included demographic and clinical information including signs, symptoms, and lab values amongst others.

Results
In this interim analysis, 88 nephrologists had completed records for 277 patients in this survey, including 95 in US, 120 in EU5, 39 in China and 23 in Japan. Median patient age at diagnosis was 40.9, and 60% were male. 80% had C3 glomerulonephritis (C3GN) and 19% had dense deposit disease (DDD). Median proteinuria at diagnosis was 2.9 g/day, and was ≥1 g/day in 82% of patients. Median eGFR at diagnosis was 50 ml/min/1.73m2 (Table 1). In addition to proteinuria, the main clinical signs and symptoms at diagnosis were hematuria, edema, hypertension and fatigue. Pain, appetite loss and sleep problems were also reported. Physicians described disease severity at diagnosis as moderate in 53% and severe in 31% of patients.

Conclusion
This study allows evaluation of a rare disease across geographies. C3G patients experience substantial symptomatic and clinical burden at diagnosis. This symptom burden, high proteinuria, and relatively low eGFR is consistent with physician assessment that the disease is moderate or severe by the time of diagnosis. Facilitating early diagnosis of C3G and rapid initiation of treatment could be beneficial for patients in slowing disease progression.
#5479 EXPLORING CANADIAN PATIENT EXPERIENCES OF LIVING WITH LUPUS NEPHRITIS
Authors: Cardwell Francesca , George Sydney , Boucher Adrian , Barber Megan , Cheema Kim , Elliott Susan , Clarke Ann 
Published: June 2023

Abstract

Background and Aims
Lupus nephritis (LN) is one of the most severe manifestations of SLE; however, the Canadian patient experience remains understudied. This research investigated patient experiences and perspectives of 1) LN diagnosis; 2) living with LN; and 3) LN healthcare and treatment.

Method
Patients aged ≥18 years with biopsy-proven pure or mixed ISN/RPS Class III, IV, or V LN and fulfilling the ACR 1997 or SLICC 2012 Classification Criteria for SLE were purposefully recruited from a Canadian lupus cohort to participate in semi-structured in-depth interviews. These were conducted virtually and transcribed verbatim for subsequent thematic analysis using NVivo Qualitative Data Analysis Software.

Results
Thirty patients with LN were interviewed; 86.7% were female, mean (SD) age was 42.1 (16.4) years, mean (SD) age at SLE diagnosis was 29.8 (15.3) years (Table 1). Patients reported challenges seeking, receiving and adjusting to the LN diagnosis, and all described emotional impacts associated with diagnosis. Most patients had limited knowledge of LN prior to diagnosis, and this lack of understanding made it difficult to contextualise their illness (Figure 1). Patients reported both emotional wellbeing and physical health impacts associated with living with LN. While most have continued in paid employment, patients identified altered career aspirations, role changes, and the need for accommodations in the workplace. Patients also described modified leisure and social activities (Figure 1). While many identified supportive friends and family, a lack of nuanced understanding of their experiences by others was reported. LN was described as a factor in family planning considerations amongst those of childbearing age. Specifically, fear of changing LN medications and their reproductive side effects, fear of experiencing a flare while pregnant/when parenting, and concerns about passing on LN/other autoimmune conditions to their children emerged (Figure 1). Numerous aspects of LN management present challenges for patients, including visiting a range of healthcare providers, taking medication, monitoring diet, stress management and ensuring adequate rest. While many reported successful LN management with medication, others expressed concern with cost and side effects. The challenges associated with a lack of LN-specific information and resources were identified (Figure 1).

Conclusion
A lack of individual and public understanding of LN coupled with the uncertainties of diagnosis/living with LN create a substantial psychosocial burden as patients negotiate acceptable risk in the face of uncertainty (e.g., in family planning, treatment, paid employment, leisure). Results emphasize the need for wider LN awareness and will inform the development of LN-specific patient resources to increase understanding of LN and better support decision-making.