bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0171NON COMPLIANCE AND ACUTE DEHYDRATION ARE MAIN REASON FOR ACUTE KIDNEY FAILURE IN NON-INFANTILE PRIMARY HYPEROXALURIA TYPE I (PH I)
Authors: Hoppe Bernd , Martin Higueras Cristina , Beck Bodo B 
Published: June 2020

Abstract

Background and Aims
Patients with primary hyperoxaluria type 1 have an increased risk of acute or chronic kidney failure (AKF/CKF). There are two risk groups: first the infantile oxalosis patients with an unremarkable family history and second, the adult patients with only an oligosymptomatic course (minor stone events), who get into end-stage renal disease (ESRD) due to oxalate-induced chronic inflammatory processes in the kidney. We were interested in the prevalence of acute kidney failure in the PH1 patients seen in our Hyperoxaluria Center over the last 10 years.

Method
We retrospectively analyzed the database of the German Hyperoxaluria Center for those patients being routinely seen in our outpatient clinic for AKF and its specific reason. AKF was defined as patients with a sudden onset of kidney failure and the necessity of dialysis installment, but a documented stable kidney function (no worse than stage 2-3 CKD) prior to onset of AKF.

Results
Currently, we routinely follow (3-4 times a year) 49 of 117 patients with genetically proven PH1. For the patients not routinely seen, we act as the reference center and primary lab. Out of the 117 PH1 patients, 6 had infantile oxalosis and thus early ESRD; 8 patients are currently on dialysis; and 6 patients have died, 2 patients each either post liver or liver/kidney transplantation (LTx, LKTx), under dialysis or as sudden death, the latter two due to systemic oxalosis. Transplantations were performed in 35 patients with PH1 (LKTx in 28 patients, LTx in 4 patients and 3 had an isolated kidney transplantation). Out of the 49 PH1 patients we routinely follow, AKF was diagnosed in 8 patients aged 11-56 years (one pediatric patient, 3 patients 18-19 years old, and 4 patients 29-56 years old), which has led to CKF and hence, maintenance hemodialyis in 7/8 and even death in one patient. Non-compliance regarding either medication or recommended fluid intake was the reason for AKF in 6 of the 8 patients, the 11 year-old girl, 2 adult patients and the 3 young adults being on the edge of leaving pediatric care. Acute massive diarrhea without adequate fluid substitution led to AKF in 2 middle aged male patients.

Conclusion
AKF is not uncommon in patients with PH1 and is frequently related to either non-compliance or to situations of severe fluid losses with inadequate fluid substitution. In the situations described, AKF is not based on already extremely altered renal function. Therefore, interruption of medication or lack of fluid intake, even for short periods of time, can lead to severe clinical consequences in these patients.
P0187COMPARING OPAL-HK DATA TO REAL-WORLD DATA FROM THE UNITED KINGDOM: HOW EFFECTIVE IS PATIROMER AT ACHIEVING NORMOKALAEMIA?
Authors: Ali Ibrahim , Cornea Georgiana , Intorcia Michele , Kalra Philip A 
Published: June 2020

Abstract

Background and Aims
The first phase of the published OPAL-HK study showed that Patiromer, an oral potassium binder, was effective at reducing serum potassium levels in patients with chronic kidney disease (CKD) stages 3 to 4. This phase was a 4-week, single-group treatment phase. Given the lack of a control arm, the aim of our study was to use real-world data from a large CKD cohort to provide a control comparison to evaluate the efficacy of Patiromer in normalising serum potassium.

Method
The initial phase of OPAL-HK comprised 243 patients with CKD G3-4 with a baseline serum potassium of 5.1 to &lt;6.5mmol/L. To acquire a comparative matched cohort, patients were selected from the Salford Kidney Study (SKS), a prospective observational cohort study in the United Kingdom that has been recruiting patients aged ≥18 years old with CKD G3-5 since 2002. A 3-step process was applied to generate a propensity matched cohort. First, patients were chosen if they had an outpatient potassium level at any point after recruitment into SKS between 5.1mmol/L to &lt;6.5mmol/L and whose next outpatient potassium level was checked 24 to 42 days (3.5 to 6 weeks) later (n=755). Second, all key OPAL-HK inclusion and exclusion criteria were applied. This left 162 patients who were then accurately matched in a 1:1 fashion to the 243 OPAL-HK patients using propensity score matching based on 6 baseline variables: age, gender, eGFR, diabetes mellitus, heart failure and potassium level. This produced a cohort of 87 SKS patients matched to their 87 OPAL-HK partner patients. We compared the two patient groups for the following OPAL-HK study endpoints: the mean change in serum potassium from baseline to follow-up at week 4, and the proportion of patients with a serum potassium of 3.8 to &lt;5.1mmol/L at week 4.

Results
The groups were extremely well matched: between SKS vs. OPAL-HK, the mean (±SD) age was 63.9±13.3 vs. 63.7±9.5years (p=0.93); mean eGFR was 30.9±11.6 vs. 31.2±11.7ml/min/1.73m2 (p=0.85); 45 vs. 46 patients were diabetics (p=0.88); 29 vs. 24 had heart failure (p=0.41) and the mean potassium was 5.5±0.3mmol/L in both groups (p=0.68). The follow-up in the SKS cohort was 31±5 days.
At the end of follow-up, the mean potassium level remained 5.5±0.3mmol/L in SKS patients but had reduced to 4.5±0.5mmol/L in the OPAL-HK group (p&lt;0.001), a mean (±SE) change of -1.00±0.06mmol/L. For the secondary endpoint, 74% of patients in OPAL-HK reached the target potassium range of 3.8 to &lt;5.1mmol/L at week 4 compared with 0% from the comparator SKS cohort (see Figure).

Conclusion
Using real-world data as a tightly-matched control arm for the first phase of the OPAL-HK study, Patiromer is shown to be effective at reducing potassium levels in patients with CKD G3-4.
P0193MEASUREMENTS OF SERUM CREATININE AND EGFR IN PATIENTS WITH CHRONIC CARDIORENAL SYNDROME TYPE 2 ARE INSUFFICIENT TO ASSESS RENAL RESPONSES TO RESYNCHRONIZATION THERAPY DURING A TREE-MONTH FOLLOW-UP
Authors: Gala-Błądzińska Agnieszka , Kubrak Tomasz , Czech Joanna , Gargasz Krzysztof , Mazur Danuta , Przybylski Andrzej 
Published: June 2020

Abstract

Background and Aims
Chronic cardiorenal sydrome type 2 (CRS-T2) has a complex pathophysiology. Treatment and evaluation of the clinical effects of CRS-T2 therapy is difficult in clinical practice. The study’s aim was to non-invasively assess renal function using biomarkers associated with nephron injury sites in patients with CRS-T2 following cardiac resynchronization therapy (CRT).

Method
The study included patients with CRS-T2 and heart failure in NYHA classes II-IV, in whom CRT devices had been implanted. Before cardiac resynchronization therapy and after 3 months, patients’ renal function was assessed using biomarkers measured in single urine and blood samples. Test results were correlated with cardiovascular fitness assessments.

Results
CRT was implanted in 56 (n = 17; 30.4% ♀) patients with a mean age of 66 ± 10 years with CRS-T2 in the course of coronary artery disease (n = 38) or dilated cardiomyopathy (n = 18) with eGFRCKD-EPI = 68.55 ± 20.34 ml / min / 1.73m2. The three-month follow-up was continued in 33 (61.11%) patients. Three months after implantation CRT, the responders group cardiovascular endurance improved by at least on NYHA class; showed a significant decrease in NT-pro BNP (n = 24; 72.72%; p = 0.004), a significant (p &lt; 0.05) decrease in serum prostaglandin D2 synthase (sPGD2S) and albuminuria (uACR) concentrations (Figure 1 a), and increases in hematocrit (HCT), erythrocyte count (RBC) and hemoglobin (Hb). The urine samples of the non-responders group following CRT, showed significant (p &lt;0.05) increases in lipocalin concentrations associated with neutrophil gelatinase (uNGAL), and a decrease in cystatin C (uCysC) concentrations (Figure 1 b). There were no significant changes in the concentration of serum creatinine (sCr), eGFRCKD-EPI, serum cystatin C or uromodulin in urine and serum; and urinary kidney injury molecule-1 (uKIM-1) was found in the urine of both responding and nonresponding groups (p&gt; 0.05).

Conclusion
The sCR and eGFR CKD-EPI assessment should not be used to assess renal function in patients with type 2 chronic cardiac renal syndrome who have undergone CRT implantation. Renal function biomarkers that account for the pathophysiology of nephron injury and correlated with myocardial function: sPGD2S, uACR, uCysC, uNGAL. Also, increased HCT, RBC, and Hb are characteristic in responders.
P0196ACUTE TUBULOINTERSTITIAL NEPHRITIS (ATIN) INDUCED BY CHECKPOINT INHIBITORS (ICI) VERSUS CLASSICAL ATIN. ARE THEY THE SAME DISEASE?
Authors: Martinez Valenzuela Laura , Draibe Juliana , Garcia Carro Clara , Agraz Irene , Fulladosa Xavier , Bolufer Monica , Tango Ariel , Torras Joan , Soler María José 
Published: June 2020

Abstract

Background and Aims
The incidence of acute tubulointerstitial nephritis (ATIN) related to drugs has dramatically increased during the past years. A new subtype of ATIN apparently different from classical drug related ATIN has emerged, which has been related to the administration of immune check point inhibitors (ICI). We herein investigated these differences between ICI related ATIN and non-ICI related ATIN, in terms of clinical features, response to treatment with steroids, and the kidney function evolution.

Method
A total of 47 patients diagnosed with acute tubulointerstitial nephritis (ATIN) from two centers were recruited. Of these, 13 patients presented with ATIN during the treatment with ICI, and 34 patients were diagnosed with ATIN attributed to other drugs. The main demographical, clinical and analytical variables such as gender, age, and current medication were recorded. The type of malignancy, oncological treatment, dose of ICI, and presence of extra-renal immune-related adverse-events were also reviewed. Renal biopsy diagnostic, time to drug withdrawal and ATIN specific treatment, as well as laboratory data during the follow-up were also studied.

Results
Patients diagnosed with ICI related ATIN presented with lower creatinine (ICI ATIN 3.8±1.03mg/dl vs. classical ATIN 5.98±4.15, p=0.007) at diagnostic and higher urinary leukocyte count (ICI ATIN 263.2±418.04 vs. classical ATIN 133.55±284.62, p=0.048) as compared to patients with non-ICI related ATIN. Time elapsed from the initiation of the culprit drug to the ATIN diagnostic was longer in ICI ATIN compared to classical ATIN (197.07±184.99 vs 114.4±352.16 days, p=0.006). In addition, during follow-up, the slope of decreasing creatinine over time was lower in ICI related ATIN compared to non-ICI related patients.

Conclusion
In this study we analyzed the differences between ICI ATIN and the classical ATIN. We found that ICI ATIN patients presented a larger latency period after offending drug initiation, milder acute kidney injury, and slower creatinine amelioration as compared to the classical ATIN. These results may be in part ascribed to potential differences in the pathological mechanisms in ATIN development, suggesting that ICI ATIN and the classical ATIN may be different disease with similar renal histology.
P0197CLINICAL FRAILTY SCORING IN PATIENTS WITH END STAGE RENAL DISEASE: A PREDICTOR OF DECLINING HEALTH?
Authors: Hetherington Lucy , Prentice Joanna , Findlay Mark , Collidge Tara 
Published: June 2020

Abstract

Background and Aims
The incidence of frailty increases as GFR decreases. In the end stage renal disease (ESRD) population frailty is associated with early mortality, increased hospitalisations, and significant symptom burden. We examined the use of formal frailty scoring and its role in identifying deteriorating patients with advanced renal disease.

Method
The Rockwood Clinical Frailty Scale (CFS) has high inter-rater reliability and correlates well with objective measures of frailty. We introduced routine recording of the CFS from January 2018 in the renal electronic record for patients on hospital haemodialysis therapy and those undergoing renal replacement therapy (RRT) planning. Based on CFS scoring patients were divided into ‘frail’ (CFS≥6) or ‘robust’ based (CFS&lt;6) and patient demographics are described. The association of being ‘frail’ or a decline in score with mortality at seven months were described using adjusted logistic regression analyses.

Results
A total of 1663 scores were recorded in 800 patients. 57.3% of patients were male. The median age at entry date was 66 (IQR 55,75) years. The median CFS score was 4 (IQR 3,5). At follow-up 74 (9.3%) had died. The median score prior to death was 5.5. 182 (22.8%) were ‘frail’. During the study period 469 patients had more than one score documented. Death at follow-up was more common in those who were ‘frail’, 20.9 vs 5.8%, p&lt;0.001. Patients who were deceased at follow-up were more likely to have had a deterioration in frailty score, 51.9% vs 24.4%, p=0.002. Being ‘frail’ or having a deteriorating frailty score was associated with death at seven-month follow-up independent of age, sex or diabetic nephropathy status.

Conclusion
The presence of ‘frailty’ as measured by CFS, or deterioration in CFS is associated with death at follow-up, independent of age, sex or diabetic nephropathy. Routine monitoring of frailty using the CFS provides a simple method to identify patients who are deteriorating and at risk of death. High or deteriorating CFS score should trigger clinical review and anticipatory care planning where appropriate.
P0200KEEP ON (KEEPING RAAS INHIBITION TREATMENT WITH OPTIMAL POTASSIUM CONTROL). DESIGN OF A CLINICAL TRIAL OF USING SODIUM ZIRCONIUM CYCLOSILICATE FOR KEEPING RAAS INHIBITION AND/OR MRAS IN PATIENTS WITH CKD AND CONGESTIVE HEART FAILURE HISTORY
Authors: Puchades Montesa Maria Jesãºs , Panizo Gonzalez Nayara , D'Marco Luis , Gonzalez-Rico Miguel , Tomas Patricia , Sanchis Irina , Martinez-Diaz Ana Isabel , Pérez Ys Aurora , Gimenez-Civera Elena , Perez-Bernat Elisa , Gorriz Jose Luis 
Published: June 2020

Abstract

Background and Aims
Hyperkalemia (HK) is a potentially life-threatening condition, in patients with chronic kidney disease (CKD) and congestive heart failure (CHF).
The majority of patients affected with CKD or CHF, must be treated with inhibitors of renin angiotensin aldosterone system (RAASi) and mineralocorticoid receptor antagonists (MRAs). However, the treatments previously mentioned, increase the risk of HK episodes, which is the main cause of RAASi and MRAs downtitration or discontinuation, representing an undesirable clinical scenario, given that the patients are at high risk of be deprived of their nephroprotective effect and cardio-renal benefits
The aim of the study is: to analyze if, in patients with HK, CKD and CHF treated with RAASi and/or MRA, serum potassium (sK) reduction by Sodium zirconium cyclosilicate (SZC) treatment is non-inferior to RAASi and/or MRAs discontinuation or downtitration.

Method

Results
The study will demonstrate results on serum electrolytes, renal function, albuminuria, KDQoL questionnaire and changes in relative overhydration (multifrequency bioimpedance -BCM Fresenius-)

Conclusion
The KEEP ON study will define the ability of SZC to facilitate the use of RAAS-I and / or MRA in patients with HK and cardiorenal syndrome allowing the maintenance of the medications recommended by international guidelines for the treatment of CHF at different degrees of CKD while maintaining the potential cardio-renal and nephroprotective benefit.
P0201ASSOCIATION OF INTRARENAL VEIN FLOW PATTERNS WITH CARDIAC FUNCTION IN OUTPATIENTS WITH CHRONIC KIDNEY DISEASE AND HEART FAILURE
Authors: Droulias Ioannis , Stavroulopoulos Aristeidis , Troganis Efstratios , Katsaounos Helias , Chatzigiannakos Dimitrios 
Published: June 2020

Abstract

Background and Aims
In patients with chronic kidney disease (CKD) and heart failure (HF), renal venous congestion plays a key role in determining renal dysfunction and a worse prognosis. Αim of this ongoing study is to identify Doppler intrarenal venous flow patterns reflecting renal congestion in patients with CKD and HF and to detect possible associations with cardiac function parameters. Prognostic implications will be evaluated at a next stge.

Method
We prospectively enroll outpatients affected by CKD stages 3-4 and HF, in stable clinical conditions and in conventional therapy. All patients undergo clinical evaluation, routine biochemistry, transthoracic echocardiogram and renal echo-Doppler. Pulsed Doppler flow recording is performed at the level of the right interlobular renal veins at the end-expiratory phase. The intrarenal venous flow patterns are divided into five types according to the waveforms of the flow. Type A and B are characterized by a continuous flow and considered normal waveforms. Type C is characterized by a short interruption and/or reversal flow during the end-diastolic or protosystolic phase. Type D and E are characterized by a wide interruption and/or reversal flow, respectively. Types C, D and E are considered abnormal and reflect increasing venous pressure within the kidneys.

Results
Until now, 36 patients (27males / 9 females), 13 (36%) diabetics, aged 69.4±10.6 years old, have been included, and baseline characteristics are presented. Twenty patients (55%) have CKD stage 3, and 16 patients CKD stage 4 (45%). Fifteen (40%) of these patients have HF New York Heart Association (ΝΥΗA) class II, 18 (50%) ΝΥΗΑ class ΙΙΙ and 3 (10%) ΝΥΗΑ class IV. Mean ejection fraction (EF) is 34.5±7.8%, moderately reduced in 9 (25%) and severely reduced in 27 (75%) of these patients. By analyzing the waveforms, we noticed that 27 patients (75%) had venous patterns of continuous flow (type A 18% and type B 57%) and 9 (25%) had venous patterns with not continuous flows (21% type C and 4% type D). The patients with renal venous patterns A and B had higher EF than those with renal venous patterns C and D (36±8% vs. 30±5%, p=0.045). We have also observed that worsening HF according to NYHA classification was significantly associated to more pathological renal venous patterns (rho=0.402, p=0.034)

Conclusion
In patients with CKD and HF we may observe abnormal intrarenal vein flow patterns, in the context of renal congestion, related to the functional state of the heart. Further studies will indicate the clinical and prognostic significance of these measurements to better characterize patients with cardio-renal syndrome.
P0202FIRST REAL-LIFE DATA OF PATIENTS TREATED FOR THROMBOTIC THROMBOCYTOPENIC PURPURA FROM THE FRENCH THROMBOTIC MICROANGIOPATHIES NETWORK
Authors: Maanaoui Mehdi , Maury Eric , Jean François AUGUSTO , Halimi Jean Michel , Barbet Christelle , Malot Sandrine , Haeck Marie , Coppo Paul 
Published: June 2020

Abstract

Background and Aims
Caplacizumab, a bivalent Nanobody, targets the A1 domain of von Willebrand Factor, inhibiting the interaction between ultra-large vWF and platelets in the treament of Acquired Thrombotic Thrombocytopenic Purpura (aTTP). Results of the Phase 3 HERCULES study confirm that treatment with caplacizumab reduces the time to platelet count response, resulting in faster resolution of aTTP. Treatment with caplacizumab also resulted in a highly clinically meaningful reduction in aTTP-related death, recurrence of aTTP, or a major thromboembolic event during study drug treatment. Data from clinical practice confirming the clinical trial data is still scarce. We report here the first real world evidence collected in the French thrombotic microangiopathies network.

Method
Patients with an acute episode of aTTP were included in an observational prospective multicentric study. The diagnosis of aTTP was done according to the French score (platelet count &lt; 30 g/l and serum creatinine&lt; 200µmol/l) or ADAMTS13 activity if available. Adult patients were proposed to be treated with daily plasma exchange (PE) in addition to corticosteroid and frontline rituximab plus early initiation of caplacizumab. PE was stopped after 2 days of platelet count normalization.

Results
Fifty patients (36 females, median age of 46 years old) were treated for aTTP. 30 patients (60%) had cerebral involvement and 13 (26%) had cardiac involvement. Cardiac troponin I was above upper normal value for 61% of patients. Before treatment, median platelet count was 16 g/l and median serum creatinine was 86 µmol/l. Three initial clinical suspicions of aTTP were finally diagnosed as HUS (2 patients) or vitamin deficiency (1 patient) resulting in the stop of treatment. A median number of 6 plasma exchanges was administered (min:3 - max:16). 375mg/m rituximab injections (3 to 4 for 35/43 patients with reported data) were mostly initiated within 3 days after the first plasma exchange. Caplacizumab treatment was initiated early (37/42 patients with reported data within 2 days after 1st plasma exchange) with a total median duration of treatment of 33 days (min:18 - max:35). The median platelet count normalization was 5 days (min:4 - max:6). 6/41 (14,6%) experienced exacerbations. No deaths and no patients refractory to therapy were reported. Eight adverse events related to caplacizumab therapy were reported, all with good outcomes.

Conclusion
Concomitant start of additional treatments allow to target severe ADAMTS13 deficiency and platelet aggregation which causes early mortality. The clinical French score seems useful in clinical practices.
P0203EARLY DETECTION OF BREAST ARTERIAL CALCIFICATION IN DIFFERENT STAGES OF CHRONIC KIDNEY DISEASE PATIENTS
Authors: Sultan Basma , Omar Hamdy , Ahmed Housseini , Elprince Mahmoud , Anter adly Osama , Tawfik Gamal 
Published: June 2020

Abstract

Background and Aims
Vascular calcification (VC) plays a major role in cardiovascular disease (CVD), which is one of the main causes of mortality in patients with chronic kidney disease (CKD).
The study aims at early detection of breast arterial calcification (BAC) in different stages of CKD (stage 2, 3& 4) patients as an indicator of systemic VC.

Method
A case control study was conducted targeting CKD women, aged 18- 60 years old. The sample was divided into 3 groups; A,B,C (representing stage 2, 3 & 4 of CKD) from women who attended nephrology and Internal medicine clinics and admitted in inpatient ward in Suez Canal University Hospital. A 4th group (D) was formed as a control group and included women with normal kidney functions (each group (A, B, C, D) include 22 women). The selected participants were subjected to history taking, mammogram to detect BAC and biochemical assessment of lipid profile, Serum creatinine (Cr), Mg, P, Ca, PTH and FGF23.

Results
Our study detected presence of BAC in about 81.8% of hypertensive stage 4 CKD patients compared with 50% in stage 3 CKD, also in the majority of stage 4 CKD patients who had abnormal lipid profile parameters and electrolyte disturbance.
Most of the variables had statistical significance regarding the presence of BAC.

Conclusion
Although it is difficult to determine the definite stage at which the risk of VC begins but in our study, it began late in stage 2 CKD, gradually increased prevalence through stage 3 and became significantly higher in stage 4. These results suggest that preventive strategies may need to begin as early as stage 2 CKD.