bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0418LUPUS NEPHRITIS: A MONOCENTRIC STUDY
Authors: Omrane Marwa , Aoudia Raja , Ounissi Mondher , Chargui Soumaya , Jerbi Mouna , Gaied Hanene , Hidri Hafedh , Harzallah Amel , Ben Abdallah Taieb , Goucha Rim 
Published: June 2020

Abstract

Background and Aims
Systemic lupus erythematosus is a multi-visceral autoimmune disease. Renal involvement is one of the most common and serious manifestations of this disease. The histological lesions are highly polymorphic and the renal biopsy remains crucial for the therapeutic management of lupus nephritis (LN). The aim of our investigation was to study the epidemiological, clinical, biological and histological characteristics, outcomes and to evaluate the therapeutic protocols used for lupus nephritis’ treatment and to identify predictive factors of renal prognosis in patients with lupus nephritis.

Method
It was a retrospective study including patients over 16 years old with lupus nephritis proved by kidney biopsy and followed up over a period of 17 years in our department.

Results
We collected 155 women and 19 men with a sex ratio F / H of 8.2. The mean age at the time of the discovery of LN was 32.6 years with a maximum between 15 years and 45 years. The most frequent extra-renal manifestations were articular and dermatological manifestations (79%). Renal symptomatology was dominated by proteinuria noted in all patients, associated to a nephrotic syndrome in 68% of patients. At the time of diagnosis of LN, hematuria was present in 69% of patients and renal failure was present in half of cases. Immunologically, antinuclear antibody were positive in 89.1% of cases, anti DNA positive in 73.4% of cases, anti Sm positive in 79.8% of cases and Antiphospholipids were positive in 50% of cases, associated with an antiphospholipid syndrome in 14.9% of cases. We performed 243 renal biopsies with 174 initial and 69 iterative biopsies. The histological lesions were polymorphic dominated by LN class IV (36.6%) isolated or associated with LN class V (17.7%). All patients received a corticosteroid for induction or maintenance treatment. It was associated with immunosuppressive treatment according to different treatment regimens. The median duration of follow-up was 81.2 months. Renal outcome was marked by complete and sustained remission in 36.7% of cases, incomplete remission with chronic kidney disease in 34.5% of cases, chronic renal failure in 28.7% of cases. At univariate analysis, we identified the young age below 35 years at the time of the discovery of LN, the male sex, increased serum creatinine at the time of biopsy, proliferative forms, the presence of histological signs of chronicity and lesions of thrombotic microangiopathy as predictive factors of poor renal outcomes.

Conclusion
Lupus nephritis is one of the most common and serious manifestations of Systemic lupus erythematosus. The generalization of renal biopsy, the use of early codified therapeutic protocols and regular monitoring and evaluation of disease activity according to the appropriate scores can improve management and survival of patients with renal impairment.
P0429OUTCOMES OF LUPUS NEPHRITIS OVER AN ERA OF EVOLVING IMMUNOSUPPRESSION
Authors: McMinn Jason , Geddes Colin C , McQuarrie Emily 
Published: June 2020

Abstract

Background and Aims
The reported incidence of lupus nephritis (LN) is approximately 6.1 cases per million population per year in Scotland based on Scottish Renal Registry biopsy data. Despite immunosuppressive treatment, approximately 10-30% of patients will progress to established renal failure (ERF) within 15 years. In December 2007, our unit moved from a protocol of Cyclophosphamide/ steroid induction with Azathioprine/ Prednisolone maintenance to Mycophenolate/ steroid induction and maintenance. We undertook this study to compare remission rates before and after this change.

Method
A retrospective electronic patient record analysis was performed for all patients in our centre with a documented native renal biopsy showing a histopathological diagnosis of LN, between 1 July 1993 and 31 December 2017. Repeat biopsies were excluded. Baseline demographics, histopathological class and first and second line induction and maintenance therapies were recorded. Endpoints analysed were; partial and complete response (as defined in KDIGO Clinical Practice Guideline for Glomerulonephritis 2012), time to achieve this response, relapse, progression to ERF and death.

Results
120 patients who underwent a biopsy during the 24.5-year period received a diagnosis of lupus nephritis. 82.5% of patients were Caucasian. Median duration of follow-up was 72 months. 15% of patients died and 5% developed ERF within the follow-up period, representing one death per 56 patient years and one incident case of ERF per 168 patient years.
There were 40 patients in the pre-December 2007 group and 80 in the post-December 2007 group. Those in the earlier group were younger, with a mean age of 35.4 years versus 44.8 years in the later group (p=0.002). 23% had class V in the earlier group compared to 15% in the later group (p=0.31). Proportions of female patients were 80% and 75% in the early and late groups respectively (p=0.54). Median creatinine was 106µmol/L in the pre-December 2007 group and 89.5 µmol/L in the post-December 2007 group (p=0.96).
Patients in the pre- and post- December 2007 groups had comparable rates of complete response, at 72% and 71% respectively. However, those diagnosed before December 2007 were slower to respond, with a median time to achieve complete response of 10.5 months, compared to 6 months in those diagnosed after 1 December 2007 (p=0.007).

Conclusion
Following a change in our immunosuppressive induction regimen from Cyclophosphamide/ steroids to Mycophenolate/ steroids, our response rates have remained similar, however speed of attaining remission has improved.
P0431TRENDS OF PRIMARY GLOMERULAR DISEASE IN TURKEY FROM 2009 TO 2017: A REGIONAL REGISTRY REPORT FROM TSN-GOLD WORKING GROUP
Authors: Gul Cuma Bulent , Kucuk Mehmet , Ozturk Savas , Demir Erol , Eren Necmi , Sumnu Abdullah , Seyahi Nurhan , Gullulu Mustafa , Dede Fatih , Derici Ülver , Koc Yener , SAHIN Garip , Oymak Oktay , Manga Sahin Gulizar , Tatar Erhan , Dursun Belda , Dheir Hamad , Apaydin Süheyla , Suleymanlar Gultekin , Ulu Memnune Sena , Altunoren Orcun , Kutlay Sim , Mese Meral , Sahin İdris , Ustundag Sedat , Turkmen Kultigin , Yilmaz Mehmet Emin , Turan Kazancioglu Rumeyza , Uzun Ozcan , Candan Ferhan , Aydin Zeki , Oygar Deren , Aktas Nimet , Erdem Yunus , Paydas Saime , Guven Taymez Dilek , Can Basak , Kıykım Ahmet , Koc Leyla , Sezer Siren , Duranay Murat , Bardak Simge , Altintepe Luftullah , Kaya Burcu , Azak Alper , Alisir Ecder Sabahat , Cavdar Caner , Selcuk Nedim Yılmaz 
Published: June 2020

Abstract

Background and Aims
Several glomerular disease registries in Europe, and they shared their data; however, there was a lack of current data on trends of primary glomerulopathy in Turkey. Glomerular disease patterns can change geographical areas and populations decade by decade. Our aim is to present Turkey's primary glomerular disease pattern from 2009 to 2017.

Method
3878 native kidney biopsy records were assessed in the Turkish Society of Nephrology Glomerulopathy (TSN-GOLD) Working Group Registry. Secondary disease (lupus, etc.) and transplant biopsies do not enroll in the registry. These records divided into four periods, before 2009, 2009 to 2013, 2013 to 2017, and 2017 to current.

Results
A total of 3858 patients (M = 2173, K = 1685) were examined. There was no difference in the distribution of number of patients according to periods (Q1 = 968, Q2 = 960, Q3 = 968, Q4 = 962). Nephrotic syndrome was the most common biopsy indication in all quarters (58.6%, 53%, 44.1%, 51.6%, respectively). Glomerulopathy types; Membranous GN (29.9%, n = 290) was the first in Q1 and IgA nephropathy (17.7%, n = 172) was the second. IgA nephropathy (28.5%, n = 274) was the most common glomerulopathy in Q2, while Membranous GN (25.5%, n = 245) was the second most common. IgA nephropathy (29.9%, n = 285) was the most common glomerulopathy in Q3, followed by FSGS (22.9%, n = 222) and Membranous GN. In Q4, FSGS (26.8, n = 258) was the most common glomerulopathy, followed by IgA nephropathy (26.7%, n = 257) and Membranous GN (24.5%, n = 236) (Figure 1).

Conclusion
While biopsy indications did not change over the years, the incidence of FSGS and IgA nephropathy gradually increased.
P0434COST-EFFECTIVENESS OF MAINTANCE THERAPY WITH AZATHIOPRIME VERSUS RITUXIMAB (TAILORED OR FIXED-SCHEDULE) IN ADULTS WITH GENERALIZED ANCA VASCULITIS IN COLOMBIA
Authors: Contreras Kateir Mariel , Orozco Ortiz Viviana , Puche Eduardo José , Garcia Paola Karina , Gonzalez Camilo Alberto , Rodriguez Martha Patricia , Rosselli Diego 
Published: June 2020

Abstract

Background and Aims
Azathioprine has been for decades the drug of choice for maintenance therapy in patients with generalized ANCA vasculitis in remission. However, recent studies show that rituximab, a high-cost biological agent, which can be administrated in two different schedules, might be more effective, so it is necessary to know the cost- effectiveness.
Our goal was to compare the cost-effectiveness of the 3 maintenance schemes: standard therapy with azathioprine; fixed-dose rituximab and rituximab tailored according to CD19 lymphocyte level and ANCA titres, from the perspective of the Colombian healthcare system.

Method
We designed a 5-year annual cycle Markov model with the following stages: remission, minor relapse, mayor relapse and death. Transition probabilities were obtained from a systematic review of the literature (Scopus and Pubmed). Following national guidelines for economic studies, costs (in 2018, 1 euro = 3489 Colombian pesos) were estimated based on national drug registries, and official tariff manuals for procedures and other resources. Main outcome was quality-adjusted life years (QALY), using lupus nephropathy as a proxy; values were obtained from Tufts CEA Registry and validated by local expert panel through a modified Delphi technique. Cost-effectiveness threshold was three-times per capita GDP (16.872 euros). Discount rate was 5%. Univariate and probabilistic sensitivity analyses were performed.

Results
Overall discounted 5-years costs were € 1149 for azathioprine; € 4025 for tailored rituximab and € 5221 for fixed rituximab. QALY gains were 2.94, 3.63 and 3.64, respectively. Both tailored and fixed rituximab were cost-effective (cost per QALY gained: € 4168 and € 5817 respectively), but tailored dosing was preferable due to its lower cost. Sensitivity analyses did not modify these results significantly.

Conclusion
To our knowledge this is the first economic evaluation that compare azathioprine with tailored and fixed rituximab regimens as a vasculitis maintenance treatment in adults with ANCA generalized. Due to its lower effectiveness azathioprine should not be the first line of treatment. Tailored rituximab should be a better option than fixed schedule due to its lower cost with similar effectiveness.
P0435PREDICTORS OF RENAL SURVIVAL IN ELDERLY NEPHROTIC SYNDROME
Authors: Ben ayed Tasnim , Gorsane Imen , Trabelsi Raja , Ounissi Mondher , Ben Abdallah Taieb 
Published: June 2020

Abstract

Background and Aims
With increasing longevity, the numbers of elderly patients presenting with renal diseases including glomerular disease are increasing. Nephrotic syndrome (NS) is a common presentation of glomerular disease in the elderly. We performed this study to assess predictors of renal survival in this population.

Method
A retrospective study including one hundred and six patients aged 65 years or more hospitalized for NS in the Internal Medicine department A of Charles Nicolle hospital at Tunis, between January the 1st, 1975 and December the 31st, 2016.
A multivariate study was carried out, the dependent variable being the evolution towards end-stage renal disease (ESRD).

Results
We studied 106 patients with an average age of 70 ± 4.5years [65-83 years] with a sex ratio (M/F) of 1.7. Twenty-three percent of patients were diabetic. The median proteinuria was 4.6 [3-19.5 g/l], the mean albumin level was 20 ± 5.6g/l and the mean protidemia was 50 ± 6.9 g/l. Nephrotic syndrome was impure in 89.6 % of patients with high blood pressure in 70.5 % of cases, hematuria ≥2 + in 34.7% of cases and renal failure in 88.4 % of cases. The renal biopsy was performed in 41 patients. The most common glomerular lesions were Membranous nephropathy (29 %) followed by amyloidosis (27 %). NS was secondary in 63.2 % of cases mainly to amyloidosis (35.8 %) and diabetes (19.8 %). Idiopathic nephropaty was dominated by membranous nephropathy (11.3 %) and primitive membranoproliferative glomerulonephritis (MPGN) (6.6 %). At the end of follow-up, 35 % of patients achieved complete or partial remission and 58.5 % progressed to ESRD. The multivariate study found as independent risk factors of progression to ESRD uremia ≥ 17 mmol/l (ORa =33.2 [1.3 - 837.7]; p&lt;0.05), phosphoremia ≥ 1.6 mmol/l (ORa=22.1 [1.8-266.5]; p&lt;0.05), potassium concentration ≥ 4.3mmol/l (ORa=24.7 [2.4,251.5]; p&lt;0.01), extra-renal signs (ORa=38,9 [2.4 - 634.3]; p: 0.01), secondary nephropathy (ORa=74 [3.1 - 1788.2]; p&lt;0.01) and MPGN (ORa=48[1.4 - 1675.5]; p&lt;0.05). The protective factors were hemoglobinemia ≥ 9.3g/dl (ORa=0.007 [0 - 0.2]; p&lt;0.01), kidneys well differentiated on ultrasound (ORa=0.032 [0.003 - 0.4]; p&lt;0.01) and treatment with two diuretics (ORa=0.03 [0.003 - 0.4]; p&lt;0.01).

Conclusion
Elderly NS was characterized by a poor prognosis, in particular secondary to delayed and non-uniform treatment strategies, hence the need for rising physician awarness about this decease and consultation on a standardized treatment strategies.
P0445PREVALENCE AND PREDICTORS OF GLYCOSURIA IN PRIMARY GLOMERULOPATHIES
Authors: Ormonde Carolina , Laranjinha Ivo , Gaspar Augusta , Gonçalves Margarida , Gil Célia , Dickson Jorge , Machado Domingos 
Published: June 2020

Abstract

Background and Aims
Albuminuria is a marker of glomerular lesion, however some studies have suggested that proximal tubule (endocytosis of albumin) is also essential to determine the amount of albumin excreted in the urine. It has been hypothesized that patients with glomerulopathies and concomitant tubular damage might present higher levels of albuminuria. Glycosuria is a marker of proximal tubular dysfunction and may be used as an easy and useful marker of tubular lesion in glomerular diseases. The aim of this transversal study was to evaluate the prevalence of glycosuria in primary glomerulopathies (GP) and identify its predictors.

Method
We included all patients diagnosed in the last 10 years in our centre with primary GP confirmed by renal biopsy. Clinical, laboratory and biopsy results were collected from patients’ charts. Patients were divided in two groups according to their glycosuric status at the diagnosis. Exclusion criteria: patients with diabetes and glucose intolerance, use of SGLT2 inhibitors, transplant kidney patients and secondary GP.

Results
We studied 110 patients – 39 (35.5%) IgA Nephropathy (IgAN), 27 (24.5%) Membranous Nephropathy (MN), 26 (23.6%) Focal Segmental Glomerulosclerosis (FSGS) and 18 (16.4%) Minimal Change Disease (MCD). Demographic data were not different between patients with and without glycosuria. Global prevalence of glycosuria was 9.1% (n=10) – patients with MN had higher prevalence of glycosuria (n=4, 17.4%), followed by FSGS (n=3, 15.5%), MCD (n=1, 5.9%) and IgAN (n=2, 5.4%). We found that patients with glycosuria had higher serum creatinine (3.9±5.1 vs 1.7±1.3 mg/dL, p=0.001), higher albuminuria (7.1±6.3 vs 3.2±3.4 g/g, p=0.002), lower serum albumin (2.3±0.7 vs 3.2±1.1 g/dL, p=0.022) and lower hemoglobin (12.0±2.5 vs 13.4±2.0 g/dL, p=0.050). Nevertheless, we did not find differences between glycosuric and non-glycosuric patients in percentage of glomerular sclerosis (%GS) or interstitial fibrosis and tubular atrophy (IFTA). In a multivariate analysis, glycosuria was positively associated with MN diagnosis, serum creatinine and albuminuria, but not with hematuria, %GS and IFTA.

Conclusion
Glycosuria is not frequent in primary GP. MN is the primary GP that most frequently presents glycosuria. Glycosuria is associated with higher albuminuria and lower serum albumin levels, corroborating the hypothesis that albuminuria as an offender to the tubules will affect the amount of albumin excreted in the urine. We also found that patients with glycosuria presented a more severe renal dysfunction (higher creatinine) at the GP diagnosis.
P0459ADULT MINIMAL CHANGE DISEASE IN TURKEY: THE RESULTS OF TURKISH SOCIETY OF NEPHROLOGY GLOMERULAR DISEASES WORKING GROUP
Authors: Yılmaz Mürvet , Sipahioğlu Murat , Dervişoğlu Erkan , Aydemir Nihal , Uzun Sami , Istemihan Zulal , Unsal Oktay , Tatar Erhan , Berktas Hacı Bayram , Ozdemir Kayalar Arzu , Sumnu Abdullah , Kumru Şahin Gizem , Çetinkaya Hakkı , Kazan Sinan , Kocyigit Ismail , Gokalp Cenk , Hasbal Nuri Baris , Ucar Ayse Serra , Ozelsancak Ruya , Guven Taymez Dilek , Yadigar Serap , Alagoz Selma , Aslan Bilal Burçak , Yaylaci Selcuk , Aydin Zeki , Jabrayilov Jabrayil , Turgutalp Kenan , Dursun Belda , SAHIN Garip 
Published: June 2020

Abstract

Background and Aims
Minimal change disease (MCD) is the most common cause of the nephrotic syndrome (NS) in children. Although there are many data regarding the course, response to treatment, and outcomes in pediatric patients, only a few series have examined these issues in adults. The aim of the study was to delineate the demographic and clinical properties of MCD of adult population in our country.

Method
All over the country, a total of 47 centers entered data between May 2009 and May 2019 to the database created by of Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD) Working Group. Demographic and clinical characteristics and biopsy findings recorded to the database were analyzed.

Results
In total, 258 patients were compatible with the pathological diagnosis of MCD. Mean age was 36.16±15.44 years and 53.88% were male. At diagnosis, 66.7% patients presented with pretibial edema,15.9% with hypertension. Microscopic hematuria occured in 18.2% patients. Only 24 patients (9.3%) presented with a decrease in renal function (serum creatinine greater than 1.3 mg/dl). The mean daily proteinuria was 6.3 g, and serum albumin was 2.6 mg/dl. The most frequent indication for biopsy was NS (86.4%). On kidney biopsy, the mean number of glomeruli was 18.08±11.11. Mezangial proliferation and mezangial IgM were present in 27.5% and 22.5%, respectively. Only in 3.5% of the included patients, electron microscopic studies were performed.

Conclusion
This study describes the characteristics of a cohort of adult patients with MCD. MCD in adults may sometimes present concurrently with hematuria, hypertension, and acute kidney injury.
P0465NEPHROTIC SYNDROME IN ELDERLY PATIENTS
Authors: Ben ayed Tasnim , Gorsane Imen , Trabelsi Raja , Ounissi Mondher , Ben Abdallah Taieb 
Published: June 2020

Abstract

Background and Aims
Nephrotic syndrome (NS) is one of the manifestations of acute or chronic glomerular nephropathy in the elderly. Our study objective was to determine the particularities of NS in the elderly.

Method
This is a retrospective study, carried out in the Internal Medicine department A of Charles Nicolle hospital at Tunis, between January the 1st, 1975 and December the 31st, 2016. This study included subjects aged 65 years old or over hospitalized for NS.

Results
We studied 115 patients with an average age of 71 ± 5 years [65-83 years] with a sex ratio (M/F) of 1.7. Twenty-three percent of patients were diabetic. The median proteinuria was 4.7 g/l [3-19.5 g/l], the mean albumin level was 20 ± 6g/l and the mean protidemia was 50.6 ± 6.9 g/l. Nephrotic syndrome was impure in 89.5 % of patients with high blood pressure in 54 % of cases, hematuria ≥2 + in 30% of cases and renal failure in 82.7 % of cases. Renal biopsy was performed in 45 patients. The most common glomerular lesions were Membranous nephropathy (29 %) followed by amyloidosis (24.5 %). NS was secondary in 65.2 % of cases mainly to amyloidosis (35.6 %) and diabetes (19 %). Idiopathic nephropaty was dominated by membranous nephropathy (9.5 %) and primitive primitive (MPGN) (4.3 %). The treatment was symptomatic for 84.4% of patients. Corticosteroids and/or immunosuppressive treatment have been used for 15.6% of patients. At the end of follow-up, 35.3 % of patients achieved complete or partial remission and 56.6 % progressed to ESRD.

Conclusion
Elderly NS was characterized by a poor prognosis due to delayed cosultation and non-uniform treatment strategies. Multicentric study in order to identify different action axes could improve the prognosis of this disease. Multicentric study in order to identify different action axes could improve the prognosis of this disease.
P0467THE DIRECT USE OF ORAL ANTI-COAGULANTS IN MEMBRANOUS NEPHROPATHY
Authors: Wills Maximilian , Gupta Sanjana , Gage Alice , Ashman Neil , Forbes Suzanne 
Published: June 2020

Abstract

Background and Aims
Barts Health has a dedicated membranous nephropathy (MN) service with 15-20 incident cases per year. Nephrotic syndrome secondary to MN in particular is associated with arterial and venous thrombosis. There is little evidence to guide optimum pharmacological prophylaxis. In those considered high risk, options include warfarin, heparin or aspirin. The advent of direct oral anti-coagulants (DOACs) offers an alternative, though evidence of efficacy is isolated to case studies.
All patients with an albumin &lt;25g/L are offered anticoagulation. We describe our use of DOACs in a large single centre MN cohort.

Method
Retrospective data MN patients between 2015-2019 was analysed. This included: demographics; frequency, type and timing of thromboembolic and bleeding events; biochemical data at initiation and cessation of agent.

Results
Total number of patients: 26
DOAC treatment courses: 30
Exposure to drug (patient days): 9899
Malignancy was excluded in all cases, with the exception of 1 case of MN secondary to cancer.
There were 3 thromboembolic events in 3 patients; all events occurred on rivaroxaban and in PLA2Rab positive MN.
Thrombotic events were all arterial, with no venous events, 2 cerebral artery infarcts and 1 lower limb arterial thrombosis. These patient’s mean initial presentation PLA2Rab titres were 164 Kunits/L (75-248) and all were nephrotic at the time of the thromboembolic event. Events were 29, 128 and 340 days post DOAC initiation. 2 of these patients had a venous thrombosis prior to DOAC initiation. Event rate: 0.11 per patient year
Safety data also demonstrated 3 bleeds in this patient cohort - all were minor as per ISTH criteria. 2 epistaxes and 1 associated with rectal prolapse, all without a haemoglobin drop.

Conclusion
Our experience is that DOACs are safe and effective in patients with MN and offer a viable anti-coagulant alternative.