bluuebunny/crossref_metadata_2025_split huggingface dataset:
MO306IGA NEPHROPATHY: A 20 YEAR RETROSPECTIVE SINGLE CENTRE EXPERIENCE
Authors: Storrar Josh , Mannan Fahmida , Roy Reuben , Chinnadurai Rajkumar , Sinha Smeeta , Kalra Philip A 
Published: May 2021

Abstract

Background and Aims
IgA nephropathy is the most common glomerulonephritis worldwide. The clinical course is heterogeneous and not always easy to predict. As such, determining which patients to treat with immunosuppression has been the cause of much debate. Over recent years there has been a focus on risk prediction to help with treatment decisions (such as the widely validated International IgA Risk Prediction Tool). Here, we present a 20 year retrospective study from a single centre with the following aims: to describe the epidemiology of our cohort, to assess outcomes (such as progression to ESKD requiring RRT, mortality), and to determine if treatment choices have changed over time.

Method
We collected all cases of IgA nephropathy from our biopsy database between January 2020 and December 2019. This totalled 525 biopsies. Of these, a number were excluded from analysis, including transplant biopsies and repeat biopsies in the same patient. After exclusion, the original 525 biopsies were narrowed down to 452 patients for analysis. We collected demographic data for each patient, along with creatinine and proteinuria values over time, MEST-C scores, progression to ESKD, mortality, use of RAAS blockade and immunosuppressants. Initial analysis was performed using Excel. We plan to perform further multivariate Cox regression analysis to determine if there are associations with progression to ESKD such as degree of proteinuria, MEST-C scores and immunosuppression treatment.

Results
We identified 452 patients with biopsy confirmed IgA nephropathy at our centre between January 2000 and December 2019. 138 (30.5%) were female and 314 (69.4%) were male. The average age at time of biopsy was 45.7 years. Mortality over this period was 19.2% (87 patients). 126 (27.9%) progressed to ESKD requiring RRT, 6 (1.3%) required temporary dialysis whilst 313 (69.2%) did not require RRT. With regards to treatment, 329 (72.8%) were treated with RAAS blockade in comparison to 85 (18.8%) who were not (in 38 patients this was unclear). No immunosuppression was used in 349 (77.2%), whilst a combination of prednisolone; IV cyclophosphamide and prednisolone; and MMF and prednisolone was used in 97 (21.5%).

Conclusion
We present here a large single centre dataset of IgA nephropathy patients over a 20 year period. We show that there remains a significant risk of progression to ESKD over time. It is important to identify those patients most at risk of progression early on in their disease course so that optimal treatment can be initiated. Further analysis of this dataset will allow us to assess whether treatment strategies in recent years has had a beneficial effect on outcomes, and also to assess the correlation between MEST-C scores and treatment decisions.
MO311THE ASSOCIATION BETWEEN NEPHROTIC-RANGE PROTEINURIA AND OXALATE METABOLISM VIOLATION IN PATIENTS WITH PRIMARY GLOMERULONEPHRITIS
Authors: Stepanova Natalia , Snisar Lyudmyla , Lebid Larysa , Savchenko Svitlana 
Published: May 2021

Abstract

Background and Aims
There is a general lack of scientific research on oxalate metabolism in primary glomerulonephritis (PGN) patients. The present study aimed to evaluate plasma oxalic acid (POx) concentration and urinary oxalate (UOx) excretion in PGN patients and determine the role of nephrotic syndrome in oxalate metabolism, which has never before been reported.

Method
A total of 100 participants were enrolled in this cross-sectional single-center study, including 76 PGN patients aged 41 ± 1.83 years and 24 healthy volunteers on a free-choice diet who served as a control reference group to evaluate POx concentration. Among the patients were 53 (69.7 %) patients with nephrotic syndrome (NS) and biopsy-proven PGN and 23 (30.3 %) patients with a clinical diagnosis of PGN. All patients were treated according to KDIGO Clinical Practice Guidelines for Glomerulonephritis.
In addition to routine hematological and biochemical tests, POx concentration and UOx excretion were found in all study participants. POx was measured spectrophotometrically using a commercially available kit (MAK315, Sigma, Spain). Daily UOx excretion was determined using an oxalate oxidase/peroxidase reagent (BioSystems, Spain). Urine protein excretion (UPE) was measured in a 24-h urine collection. The glomerular filtration rate (GFR) was calculated using the CKD-EPI formula.
The data were presented as the median and the interquartile ranges [Me (Q25-Q75)] and compared using the Mann-Whitney test. The Spearman correlation test and the partial correlation coefficient were used to evaluate the association between the examined markers.

Results
POx concentration was significantly higher in the patients with PGN compared with the healthy volunteers: 29.9 (14.9-51.7) vs 18.9 (16.2-23.8) µmol/L, p = 0.01. Although the patients with NS demonstrated a statistically higher GFR level compared with the patients with mild proteinuria [70.5 (47-87) vs 50 (22-76.2) mL/min/1.73 m2, p = 0.01], these patients also had the highest POx level (Fig. 1). Moreover, POx concentration was significantly associated with GFR (r = -0.27, p = 0.005), serum phosphate (r = 0.26, p = 0.007) and UPE (Fig. 2) levels. No significant differences were found in UOx excretions between the groups. However, the higher level of UPE was, the higher level of UOx was observed in the PGN patients with NS (Fig. 3). The partial correlation analysis confirmed a strong association between UPE and POx concentration independently of the patients’ age, gender, GFR and serum phosphate levels (r = 0.22, p = 0.04).

Conclusion
Nephrotic-range proteinuria was significantly associated with the elevation of POx concentration and UOx excretion in the PGN patients. More research with a larger cohort is needed to confirm this preliminary evidence and validate NS as a risk factor for oxalate metabolism violation in PGN patients.
MO314ANA POSITIVITY IN IGA NEPHROPATHY: IS SYSTEMIC LUPUS ERYTHEMATOSUS COMING UP?
Authors: Catapano Fausta , Fabbrizio Benedetta , Mancini Elena 
Published: May 2021

Abstract

Background and Aim.
IgA nephropathy (IgAN) is the most common chronic primary glomerulonephritis leading to progressive renal failure in 1/3 of patients. Although it is a limited non-systemic renal disease, many systemic diseases, such as Systemic Lupus Erythematosus (SLE), are sporadically associated with mesangial IgA deposits. We investigate frequency and meaning of ANA positivity in IgAN.

Methods.
All biopsy-proven IgAN patients followed in our Unit with ANA positivity were selected. Data are compared by non parametric test (Wilcoxon signed-rank test).

Results.
15/68 (22%) IgAN patients resulted ANA+ during a 9-year median follow-up. 10 were females and five were male. Mean age was low. Renal function was normal in 9/15. 24 h Proteinuria was non nephrotic in 15/16. In 10 patients there was low C3. Patient characteristics are summarized in Table 1. At follow-up, renal function did not change significantly (p=NS), however mean 24h-Uprot reduced (p=0.075) Table 2. One male developed SLE, one female a lupus-like Mixed Connective Tissue Disease.

Conclusions.
Our data suggest that 1) in 22% of IgAN patients ANA positivity could be detected; 2) ANA+ IgAN patients are mostly women with normal renal function and non nephrotic proteinuria; 3) after a median 9-year follow-up, ANA+ IgAN patients, on therapy, presented a stable renal function and a reduced proteinuria; 4) ANA+ IgAN patients could develop SLE. More clinical observations and studies are needed for supporting the hypothesis that IgAN+ANA positivity could be part of a new clinical entity of SLE patients.
MO317DETERMINANTS OF LENGHT OF HOSPITAL STAY (LOS) IN NEPHROTIC SYNDROME PATIENTS
Authors: Accarino Simone , Colucci Marco , Pasquinucci Ettore , Sileno Giuseppe , Esposito Vittoria , ARAZZI MARTA , Cristini Eleonora , Esposito Ciro 
Published: May 2021

Abstract

Background and Aims
Generalized edema, non responsive to oral diuretics, is one of the main causes of hospital admission for nephrotic syndrome patients. Although hospital length of stay (LOS) may vary widely, in 2017 the average LOS in acute-care hospitals was lower than 8 days in OECD countries. The aim of the present study was to determine the factors commonly associated with a longer LOS in patients admitted for edema due to nephrotic syndrome in the Nephrology Unit of ICS Maugeri, Pavia, Italy

Method
In this retrospective study we reviewed the medical records of all patients admitted for nephrotic syndrome between 2012-2020 in the Nephrology Unit of ICS Maugeri. Inclusion criteria were the following: age between 18-85 years of age; severe edema non responsive to oral, low dose diuretics; patients with heart failure, serum creatinine &gt; 3.5 g/dl or on dialysis treatment were excluded from the study. Patients were divided into two groups according to the length of stay: ≤ 7 days or ≥ 8 days. Age, gender, serum protein concentration, creatinine, and hemoglobin; serum cholesterol and tryglicerides, urinary protein excretion rate; types of glomerular disease, weight loss were recorded. Student T tests and one-way Anova were performed to evaluate the differences between the means.

Results
60 patients (42 male, 18 female) with a total number of hospital accesses of 93 were enrolled in the study. Mean age was 66.8 ± 13.07 years. Average LOS was 9.02 ± 7.4 days. Protein excretion rate was 6.7 ± 3.6 g/24 hours at the admission and was not statistically changed at discharge. Mean total serum protein and creatinine concentration at the admission were 4.7 ± 0.8 g/dl and 1.8 ± 1.1 mg/dl respectively. Patients with LOS &lt; 7 days were younger (64 ± 11.9 vs 69 ± 13.6 years, p &lt;0.05), had a lower serum creatinine (1.55 ± 0.92 vs 2.08 ± 1.2 mg/dl, p&gt;0.001) and a significantly higher total serum protein concentration (5.02 ± 0.77 vs 4.65 ± 0.76 g/dl, p&lt; 0.001) and haemoglobin (12.6 ± 1.8 vs 11.4 ± 1.8 g/dl, p&lt; 0.05) compared to patients with longer LOS. Proteinuria was not significantly different between the two groups (6.27 ± 3.36 vs 7.1 ± 3.9 g/24 hours, p= NS). While serum cholesterol and tryglicerides were higher in the group of patients with longer LOS, weight loss was similar in the two groups at discharge. Although the difference was not significant, the group with longer hospitalization had a greater number of patients with a diagnosis of focal segmental glomerulosclerosis (FSGS)

Conclusion
Our results demonstrate that age, total serum protein concentration, serum creatinine, higher lipids and probably the diagnosis of FSGS may affect the hospital length of stay of patients with nephrotic syndrome admitted for severe edema. A more aggressive diuretic treatment may be needed in elderly nephrotic syndrome patients with lower GFR and total serum protein concentration.
MO318END-STAGE RENAL DISEASE RELATED FACTORS IN ANCA-ASSOCIATED VASCULITIS
Authors: Ferrer García Guillermo , Moral Berrio Esperanza , Castro Fernández Maria Paz , Piccone Saponara Luis Guillermo , Carreño Parrilla Agustín , Martínez Calero Alberto , Arambarri Segura Minerva , Sidel Tambo Diego , Sanchez Escudero Patricia , Vozmediano Poyatos Carmen 
Published: May 2021

Abstract

Background and Aims
Management of ANCA-Associated Vasculitis (AAV) is in constant update. The aim of the study is to describe our experience as a territorial reference center with this systemic disease and to analyze which factors have a significant influence on the development of end-stage renal disease (ESRD).

Method
Retrospective observational study. All the patients who developed AAV in our center between 2010 and 2019 were included. Demographic variables (age, sex), renal function, other vasculitis related symptoms, induction and maintenance therapy, response degree and follow-up were collected. Categorical variables are expressed as percentages and compared using Chi2 test. Quantitative variables are expressed as mean ± standard deviation and compared using Mann-Whitney U test. Cox regression was performed to determine independent predictors of ESRD. Kaplan-Meier was used to estimate ESRD-free survival. Statistical significance for a value of p&lt; 0,05. Statistical analysis was performed with SPSS 25.0.

Results
45 patients were analyzed, with an average age of 70 ± 11 years. 62.2% were men. Mean time of follow-up 36 ± 31.6 months. 37.8% presented c-ANCA autoantibodies and 57.8% p-ANCA. Mean baseline serum creatinine level was 5.51 ± 3.65 mg/dl and proteinuria 2.82 ± 2.48 g/24h. 77.8% received cyclophosphamide as induction immunosuppressive treatment whereas 13.3% rituximab. 50% received azathioprine, 36.1% mycophenolate and 13.9% rituximab as maintenance treatment. 37.8% patients underwent plasma exchange therapy and 44.4% hemodialysis. Complete remission was achieved by 13.3% of patients, while 57.8% partial remission. 28.9% had absence of remission. 28.9% achieved ESRD. ESRD was associated with undergoing hemodialysis (69.2% vs 30.8% p=0.033), to the type of response (complete 7.7% vs partial 23.1% vs no response 69.2%), baseline creatinine level (8.36 ± 5.44 vs 4.35 ± 1.64 mg/dl p=0.011), creatinine 6 months after induction treatment (4.3 ± 2.05 vs 2.04 ± 0.77 mg/dl p=0.001) and at the end of follow-up (6.33 ± 2.47 mg/dl vs 2.2 ± 1.29 mg/dl p=0.001) and also to baseline proteinuria (4.21 ± 3.12 vs 2.25 ± 1.96 p=0.003), proteinuria 6 months after induction treatment (1.4 ± 1.46 vs 0.58 ± 0.73 g/24h p=0.014) and at the end of follow-up (2.48 ± 1.9 vs 1.12 ± 1.64 p=0.001). Logistic regression only showed end of follow up serum creatinine level as an independent risk factor of ESRD (OR3.74 IC 95% 1.01-13.75 p=0.047). ESRD-free survival chance after 5 of follow-up was 67%.

Conclusion
Only serum creatinine level at the end of follow-up could be found as an associated factor with ESRD. Greater number of patients would be needed in order to obtain other factors leading to ESRD in patients with AAV.
MO320LUPUS FLARE MIMICKING COVID-19 INFECTION: A CASE REPORT
Authors: Sabah Meryem , Jabrane Fatimzahra , El oury Hiba , Khalfaoui Mohamed Amine , Chehab Nasreddine , Medkouri Ghizlaine 
Published: May 2021

Abstract

Background and Aims
The world is in midst of the coronavirus disease 2019 (COVID-19) pandemic.

Method
Studies of the COVID-19 pathophysiology show that its defining character is an overt inflammatory response, similar to cytokine release syndrome, causing a dysregulated immune response. Systemic lupus erythematosus (SLE) is also a disease of immune dysregulation contributing to multisystem compromise. There is very little literature to suggest that COVID-19 could potentially mimick SLE presentation. We describe the case of a female patient who presented with typical Covid19 clinical features, later diagnosed as a new-onset SLE.

Results
A 51-year-old female presented with fever, dyspnea, cough and desaturation at the emergency room. Chest computed tomography scan showed bilateral areas of ground-glass opacities in a peripheral distribution. She was admitted in a Covid-19 ICU. She then progressed to severe acute respiratory distress syndrome, and worsening renal function with proteinuria and hematuria. Further investigations showed bilateral pleural effusions, ascites, leukopenia and thrombocytopenia, positive antinuclear and anti-double-stranded DNA antibodies, and low levels of C3 and C4. SARS-Cov-2 PCR was negative twice. After the establishment of the diagnosis, the patient was transferred to the internal medicine department where she received decongestive therapy, intravenous (IV) pulses of methylprednisolone, along with hydroxychloroquine. She improved sustainably and was discharged after two weeks.

Conclusion
A patient with new-onset lupus presented with clinical features typically seen in COVID-19 patients. The adequate management of the patient was delayed due to the misguided diagnosis.
MO321THE USE OF INTRAVENOUS CYCLOPHOSPHAMIDE AND ORAL STEROIDS IN PRIMARY MEMBRANOUS NEPHROPATHY WITH ADVANCED KIDNEY DISEASE
Authors: Ragy Omar , Hamilton Patrick , Kanigicherla Durga 
Published: May 2021

Abstract

Background and Aims
International guidelines do not recommend specific use of immunosuppression treatment in membranous nephropathy patients presenting with an estimated glomerular filtration rate of &lt;30ml/min/1.73m2. This is due to the scant published evidence of effectiveness and uncertainty around toxic effects at this stage. In this study, we sought to examine the safety and effectiveness of combined cyclophosphamide and steroids in primary membranous nephropathy with advanced kidney disease.

Method
This is a retrospective study of 18 patients with a biopsy confirmed membranous nephropathy who received combination therapy between 2004 and 2019. The mean age was 66.5 with a 5:1 male: female ratio. The immunosuppression regime included monthly intravenous pulse cyclophosphamide and daily oral steroids. All patients had an initial eGFR of &lt; or = 30 ml/min/1.73 m2 at time of treatment. Clinical parameters are serially monitored as part of clinical care and anti-PLA2R antibody levels were measured at the time of and 1-year post immunosuppression treatment where available.
The primary outcome was the achievement of partial remission as per standard criteria. Secondary outcomes were immunological response defined by following the anti-PLA2R antibody levels at the time of presentation and 1-year post, need for renal replacement therapy, outcomes including hospital admission, infections, malignancy, and death.

Results
Partial Remission was achieved in 16 out of 18 (88%) patients.7 patients (39%) reached complete remission, with none of them requiring dialysis over an average follow-up of 5 years. The average increase in eGFR was 12.5 ml/min/1.73m2 from the time of immunosuppression till the latest follow up.
2 out of 18 (11%) patients were refractory to treatment and required initiation of dialysis 1 and 8 years post immunosuppression. Results for the anti-PLA2R antibody test were available for 12 (66.6%) patients at presentation all shown to be positive. Of these, 7 (58%) achieved both immunological and clinical remission. 4 patients developed 4 different cancers (Bladder, mesothelioma, skin SCC and sigmoid) during follow-up; 2 patients required hospital admission for episodes of infections. Both were managed with antibiotics, and patients were discharged home safely. 5 patients died at the end of the follow-up period (27.7%).

Conclusion
Here we show that the combination of intravenous pulse cyclophosphamide and steroid is well tolerated and effective in achieving remission in primary membranous nephropathy with advanced renal disease. Both immunological and clinical responses could be achieved in this cohort. Longer-term risk in such patients includes malignancy and infections. This study provides reassurance that this cohort of patients can be considered for immunosuppressive treatment, although prospective studies are required to provide further robust evidence.
MO323MEDICATION INDUCED RELAPSE OF FGSG
Authors: Anvari Evamaria , Barnes Julie 
Published: May 2021

Abstract

Background and Aims
31-year-old female with past medical history of Multiple Sclerosis diagnosed with Minimal Change Disease (MCD) in 2011. At diagnosis she had proteinuria that exceeded 7 g per day. She was treated with steroids and went into complete remission. In 2017 presented again with nephrotic syndrome and on repeat biopsy she was diagnosed with Focal Segmental Glomerulosclerosis (FSGS). She was restarted on steroids and had some improvement but despite 20 weeks of high dose steroids did not achieve remission. After every attempt of tapering, she would relapse.

Method
Other treatments tried without success were tacrolimus, cyclophosphamide and rituximab. Mild improvement with tacrolimus but stopped after a grand mal seizure. The lowest achievable steroid dose was 20mg daily. She was started on combination therapy tacrolimus and mycophenolate mofetil and was able to come off steroids and went into remission.

Results
After a few months of remission, she began to relapse. It was noted after extensive investigation that she had been placed on cabergolin for hyperprolactinemia by her endocrinologist at the time she began to relapse. Cabergolin was stopped and she went into remission again. We present the first case of FSGS relapse due to cabergolin.

Conclusion
Cabergoline is metabolized by hydrolysis and has limited cytochrome P450 (CYP) metabolism. Despite limited CYP metabolism, cabergoline does have an interaction with clarithromycin, a known inhibitor of CYP and p-glycoprotein. One rat study suggest that mycophenolate is a substrate for p-glycoprotein, so it is possible that there is some competitive inhibition. This would explain why the patient relapsed while on cabergoline and in remission after stopping it.
MO326CORTICOSTEROIDS FOR THE TREATMENT OF AUTOIMMUNE DISEASE: A SYSTEMATIC REVIEW AND META-ANALYSIS OF REPORTED ADVERSE EVENTS IN RANDOMISED CONTROLLED TRIALS
Authors: Major Rupert , Grant Robert , Nockels Keith , Das Mrinal , Floege Jürgen , Barratt Jonathan 
Published: May 2021

Abstract

Background and Aims
Systemic corticosteroids are commonly used to treat autoimmune diseases such as immunoglobulin A (IgA) nephropathy. Corticosteroids are associated with increased risk of adverse events such as weight gain, hyperglycaemia, hypertension, infection and bone fracture occur frequently and affect safe long-term use. Adverse events of corticosteroids in clinical trials may have been historically under-reported. We aimed to perform a systematic review and meta-analysis of reported adverse events of corticosteroids in autoimmune diseases, with a particular focus on kidney pathologies.

Method
Pre-registered protocol systematic review (Prospero ID: CRD42020206650). The following databases were searched from 1980 to 2020:
Placebo-controlled trials in adults with any form of autoimmune disease receiving intravenous or oral corticosteroids were included in the systematic review. Trial protocol major adverse events and all-cause mortality were included as the current study’s main outcome.

Results
After exclusion of duplicates between databases, 4490 OVID MEDLINE and 4987 Cochrane abstracts were reviewed. In total, 110 published clinical trials were identified for inclusion in the study: 14 clinical trials were in kidney autoimmune diseases, including 8 in IgA nephropathy. Results of their published adverse events, including where appropriate meta-analysis, will be presented. Specific results of those studies of kidney pathologies such as IgA nephropathy will be presented.

Conclusion
This systematic review with a pre-registered protocol identified 110 clinical trials examining systemic corticosteroid use versus placebo in autoimmune diseases. These results will help to understand whether the risks of systemic corticosteroids for autoimmune disease has been historically under-reported in the medical literature and whether a clear risk and benefit profile of corticosteroids in autoimmune disease can be assessed.