bluuebunny/crossref_metadata_2025_split huggingface dataset:
MO289IS THERE A ROLE OF INTERSTITIAL CHANGES ON THERAPY OUTCOME AMONG PATIENTS WITH FOCAL-SEGMENTAL GLOMERULAR SCLEROSIS AND MINIMAL CHANGE DISEASE?
Authors: Jankovic Aleksandar , Djuric Petar , Bulatovic Ana , Markovic Dragana , Pešić Snezana , Dimkovic Nada , Naumovic Radomir 
Published: May 2021

Abstract

Background and Aims
It is well-known that interstitial changes in patients with focal-segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are linked with disease chronicity and progression. The aim of this study was to analyze is there any connection between interstitial changes and outcome after “first line” therapy in patients with FSGS/MCD.

Method
From 2014 till 2019, biopsy proven diagnosis of FSGS/MCD was established in 40 patients. Interstitial changes were classified in three groups as following: 0-w/o changes; 1-mild changes; 2-severe changes. Patients with nephrotic syndrome (No=29) were treated with prednisolone (1mg/kg) and after six months we have registered therapy outcome as: CR-complete remission; PR-partial remission; EX-death; NO-no effect.

Results
Among treated patients (age 50.4±15.3 years, 15 men), CR was achieved in 10 patients (34.5%) and 6 out of these 10 (60%) had no interstitial changes. Partial remission was observed in 11 patients (37.9%), in 4 patients (13.8%) therapy did not have any effect, and 4 patients (13.8%) deceased (table 1). All patients in EX and NO group had interstitial changes. There were no significant difference in age, gender, proteinuria, albuminaemia, creatinine and glycaemia levels between groups except for hemoglobin levels that were significantly lower in EX group than in others (∑ 15.144; p=0.002) and urea levels that were significantly higher in EX group (∑ 138.057; p=0.024).

Conclusion
Patients with FSGS/MCD respond well on standard immunosuppressive protocol, particularly in absence of interstitial changes what may increase the chance for achieving complete remission.
MO247NOVEL DIAGNOSTIC AND THERAPEUTIC TECHNIQUES REVEAL CHANGED METABOLOMIC PROFILE IN RECURRENT FOCAL SEGMENTAL GLOMERULOSCLEROSIS*
Authors: Müller-Deile Janina , Sarau George , Kotb Ahmed , Daniel Christoph , Kalkhof Stefan , Christiansen Silke , Schiffer Mario 
Published: May 2021

Abstract

Background and Aims
Idiopathic forms of Focal Segmental Glomerulosclerosis (FSGS) are caused by circulating permeability factors, which can lead early recurrence of FSGS and kidney failure after kidney transplantation. In the past three decades, many research endeavors were undertaken to identify these unknown factors. Even though some potential candidates have been recently discussed in the literature, “the” actual factor remains elusive. Therefore, there is an increased demand in FSGS research for the use of novel technologies that can allow us to study FSGS from a yet unexplored angle.

Method
We used novel treatment options, a personalized in vitro and in vivo assay as well as Raman spectroscopy and mass spectrometry for recurrent FSGS.

Results
Here, we report the successful treatment of recurrent FSGS in a patient after living related kidney transplantation by removal of circulating factors with CytoSorb apheresis (Fig. 1). Interestingly, the classical published circulating factors were all in normal range in this patient but early disease recurrence in the transplant kidney and immediate response to CytoSorb apheresis were still suggestive for pathogenic circulating factors. To proof the functional effects of the patient’s serum on podocytes and the glomerular filtration barrier we used a podocyte cell culture model and a proteinuria model in zebrafish to detect pathogenic effects on the podocyte’s actin cytoskeleton inducing a functional phenotype (Fig. 2, Fig. 3). We then performed Raman spectroscopy in the &lt;50 kD serum fraction (Fig. 4), on cultured podocytes treated with the FSGS serum (Fig. 5), and in kidney biopsies of the same patient at the time of transplantation and at the time of disease recurrence (Fig. 6). The analysis revealed metabolomic changes in podocytes induced by the FSGS serum as well as in focal glomerular and parietal epithelial cell regions in the FSGS biopsy. Several disturbed Raman spectra were identified in the fractionated serum and metabolome analysis by mass spectrometry detected lipid profiles in the FSGS serum, which corresponded to disturbances in the Raman spectra.

Conclusion
Our novel innovative analysis reveals changed lipid metabolome profiles associated with idiopathic FSGS that might reflect a new subtype of the disease.
MO294COMPARISON OF TIP AND CELLULAR VARIANT OF PRIMARY FOCAL SEGMENTAL GLOMERULOSCLEROSIS
Authors: Zagorec Nikola , Horvatić Ivica , Kasumović Dino , Šenjug Petar , Horaček Matija , Pražetina Marko , Lucijanić Marko , Galešić Ljubanović Danica , Galesic Kresimir 
Published: May 2021

Abstract

Background and Aims
After membranous nephropathy, focal segmental glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome in European population. According to Columbia classification, there are five histological variants of FSGS defined on light microscopy (tip, cellular, perihilar, collapsing and not otherwise specified - NOS) and this classification has a prognostic significance. The aim is to compare features and outcomes of tip and cellular variant of primary FSGS.

Method
All patients with FSGS were identified by a retrospective review of the Registry of kidney biopsies at the Department of Nephrology and Dialysis, Dubrava University Hospital, Zagreb, from 2003 until 2020. Each kidney specimen was analyzed by light, immunofluorescent and electron microscopy and Columbia classification was applied by experienced nephropathologist. Patients with primary FSGS met following criteria: full nephrotic syndrome and diffuse podocyte foot process effacement in absence of secondary causes of FSGS. Laboratory findings were obtained for every patient at the time of biopsy and following outpatient visits. Complete remission was defined as proteinuria &lt; 0.3 g/day with normal kidney function and partial remission as proteinuria 0.3 - 3.5 g/day. Variables are expressed as median ± IQR (interquartile range) and frequencies. Statistical comparison between groups of patients with tip and cellular variant of primary FSGS and disease outcome analysis were done.

Results
Out of 200 patients with FSGS, 59 (29.5 %) had primary form of disease. Tip variant was the most common form of primary FSGS (22 patients, 37 %) followed by NOS (20, 34 %), cellular (13, 22 %), perihilar (2, 3.5 %) and collapsing (2, 3.5 %) variant. Demographic and clinical features with initial laboratory findings are shown in Table 1. There were no significant differences between two groups in all analyzed variables in Figure 1. All patients were treated by anti-RAAS agents and steroids. Median follow-up was 55 months (range 1 – 196 months), and followup data were unavailable for three patients. Figure 2 shows treatment regimens in both patient grouos with treatment outcomes. Remission rate was significantly higher in tip variant (90 % vs. 41 %, p = 0.002). There was no difference in relapse rate between the two groups (p = 0.717).

Conclusion
There were no significant differences in clinical features and laboratory findings at the time of clinical presentation between tip and cellular variant of primary FSGS. Patients with tip variant had significantly higher remission rate than patients with cellular variant.
MO295RESULTS OF 30 YEARS OF RENAL BIOPSIES IN THE GENERAL HOSPITAL OF CIUDAD REAL. WHAT HAS CHANGED?
Authors: Moral Berrio Esperanza , Vozmediano Poyatos Carmen , Arambarri Segura Minerva , González López Lucía , Carreño Parrilla Agustín , Castro Fernández Paz , Ferrer García Guillermo , García Conejo Gloria , Anaya Fernández Sara , Olazo Gutierrez Eliana , Piccone Saponara Luis Guillermo 
Published: May 2021

Abstract

Background and Aims
The renal biopsy (RB) has been performed in the General Hospital of Ciudad Real (HGUCR) since the year 1989. It allows kidney diseases to be diagnosed and treated and pronostics to be made. We will analyse the results of these 30 years as well as the evolution of the various parameters studied.

Method
Descriptive study of the RB carried out in the HGUCR between 1989 and 2019. Age, sex, clinical syndrome (CS) at the time of the RB, number of glomeruli and histological diagnosis will be analysed. The patients are divided into 3 groups according to age: children (&lt;15), adults (15-65) and the elderly (&gt;65). We will establish three periods of 10 years: period A (1989-1998), period B (1999-2008) and period C (2009-2019). The categorical variables are expressed as percentages and the quantitative variables average ± standard deviation. Statistical analysis with SPSS 25.0.

Results
898 RB have been performed, average number of glomeruli 16, 70% of the RB with more than 10 glomeruli. The average age of the patients was 53±19 years old, 58% male. The most frequent CS was acute kidney failure (AKF) (35%), followed by nephrotic syndrome (NS) (30.5%), asymptomatic urinary disorders (19%), chronic kidney disease (11%), nephritic syndrome (3.6%), haematuria (0.7%) and arterial hypertension (0.7%). The most common in children were asymptomatic urinary disorders (50%), in adults NS (34%) and in the elderly AKF (55.5%). The predominant primary glomerulonephritis (GN): IgA nephropathy (IgAN) (15%), followed by membranous nephropathy (MN) (12%) and focal segmental glomerulosclerosis (FSGS) (11%). The most frequent secondary GN: vasculitis (11%) and lupus nephropathy (10%). 164 RB were performed in period A, 370 in period B and 346 in period C. In all three periods the predominant sex was male and the average age increased: 48 years old in A, 51 years old in B and 56 years old in C. Together with the increase in age, the indication of RB changes: NS in the first two periods and AKF in period C. The most frequent pathology in period A: FSGS (17%), IgAN (16%) in period B and IgAN (15%) followed by vasculitis (11%) in period C.

Conclusion
In the HGUCR the most common biopsied kidney pathology is IgAN, followed by MN. There has been an increase in the age of the patients as well as an increase in AKF and vasculitis. The KB constitutes a highly useful diagnostic test that allows us to establish prognostics and appropriate treatments.
MO296MEAN PLATELET VOLUME IN FAMILIAL MEDITERRANEAN FEVER RELATED AMYLOIDOSIS AND COMPARISON WITH COMMON PRIMARY GLOMERULAR DISEASES
Authors: Yildirim Tolga , Kutahya Berranur , Is Fatma , Erdevir Mehmet , Uzerk Kibar Muge , Koc Neriman Sila , Saglam Arzu , Yilmaz Seref Rahmi , Erdem Yunus 
Published: May 2021

Abstract

Background and Aims
Compared to healthy controls, mean platelet volume (MPV) is frequently higher in patients with Familial Mediterranean fever (FMF) but lower in amyloidosis patients. The cause of differing MPV levels in FMF patients with and without amyloidosis is not clear. We hypothesized that severe proteinuria and renal dysfunction in amyloidosis could be responsible from low MPV in contrast to FMF patients without amyloidosis. We aimed to compare MPV levels of FMF patients with amyloidosis and MPV values of patients with different glomerular diseases to understand if low MPV is unique to amyloidosis or MPV is similar in all glomerular diseases indicating that it is a consequence of proteinuria and/or renal dysfunction.

Method
We compared pre-biopsy MPV levels of patients with amyloidosis secondary to FMF, to MPV levels of patients with membranous glomerulonephritis, focal segmental glomerulosclerosis (FSGS) and IgA nephropathy that all present with proteinuria and renal dysfunction. Factors affecting MPV were also determined.

Results
703 patients (411 male, 292 female) were included in the study. Mean age was 42.6±14.3 years. There were 124 patients with amyloidosis, 224 patients with IgA nephropathy, 188 patients with membranous glomerulonephritis and 167 patients with FSGS. Patients with amyloidosis had lower MPV compared to patients without amyloidosis (7.9±1.2 fL vs. 8.2±0.9 fL respectively, p=0.008). Patients with amyloidosis had also significantly lower MPV compared to patients with each of the other diagnoses. MPV was negatively correlated with platelet count (r = - 0.351, p &lt;0.001). There was no significant correlation of MPV with serum creatinine and proteinuria.

Conclusion
This study is the largest study of MPV in patients with biopsy proven amyloidosis and confirms previous studies reporting low MPV in amyloidosis. This study indicates that low MPV in amyloidosis cannot be explained with severe proteinuria and renal dysfunction.
MO299RELATIONSHIP BETWEEN LOW-TITER PHOSPHOLIPASE A2 RECEPTOR ANTIBODY AND TREATMENT RESPONSE OF IDIOPATHIC MEMBRANOUS NEPHROPATHY
Authors: Cui Haoyuan , Li Chao , Li Hang , Wen Yu-bing , Chen Limeng , Li Xuemei 
Published: May 2021

Abstract

Background and Aims
The circulating anti-Phospholipase A2 receptor (PLA2R) antibody has been widely detected since its discovery in 2009. It was suggested that the cut-off of serum PLA2R antibody (PLA2RsAb) should be reset at below 20 RU/mL to prove diagnostic efficacy. The correlation between low titer PLA2RsAb and treatment response has not been discussed. In this study, we focused on the low titer PLA2RsAb during follow-up of PLA2R-related IMN.

Method
We retrospectively enrolled 43 Chinese patients with biopsy-proved PLA2R-related IMN from March 2017 to October 2019. Baseline data were collected including age, gender, serum creatinine (Scr), albumin (Alb), 24h urine protein (24hUP), and PLA2RsAb at biopsy. PLA2RsAb was detected at one month, three months, six months and one year. The patients were stratified into three groups by the lowest PLA2RsAb titer during follow up. Low titer group was defined as anti-PLA2R antibody ＜2RU/mL (53.5%, n=23). Median titer group was defined as anti-PLA2R antibody &gt;2 but ＜20RU/mL (32.6%, n=14). High titer group was defined as anti-PLA2R antibody &gt;20RU/mL (13.9%, n=6). Complete remission (CR) was defined as 24hUP ＜0.3g/d. Partial remission (PR) was defined as 24hUP at the range of 0.3-3.5g/d and a reduction &gt;50% of the baseline proteinuria. No remission (NR) was defined as absence of CR or PR at one-year.

Results
In the entire cohort, patients were 49 ± 16 years old with 28/15 male/female ratio. Baseline data of low, mid and high titer group were as follows: the median PLA2RsAb were 34.3(22.1, 314), 103(55.8, 361), 73.6(53.0, 215)RU/mL, P = 0.305; the median 24hUP were 6.8(3.5, 12.4), 6.4(5.6, 8.9), 4.9(3.4, 7.2)g/d, P = 0.493; the average Scr were 100.4 ± 60.0, 92.3 ± 29.2, 82.0 ± 21.9 umol/L, P = 0.681; the median Alb were 27.0 ± 5.7, 23.8 ± 5.8, 30.2 ± 3.3 g/L, P = 0.054. The rate of CR, PR, NR were 34.8% (8/23), 34.8% (8/23), 30.4% (7/23) in low titer group, 7.1% (1/14), 71.4% (10/14), 21.4% (3/14) in mid titer group, 0% (0/6), 66.7% (4/6), 33.3% (2/6) in high titer group, P = 0.119

Conclusion
The correlation between low titer PLA2RsAb and treatment response showed no statistical significance.
MO301RITUXIMAB FOR RELAPSING MEMBRANOUS NEPHROPATHY: A SPANISH HOSPITAL EXPERIENCE
Authors: Fernandez Lorente Loreto , Manrique Joaquin , Visus Maria Teresa , Izquierdo Diana , Castaño Itziar , Slon Roblero Maria Fernanda , Gomez Nerea , Romero Gregorio , Purroy Iruzun Carolina 
Published: May 2021

Abstract

Background and Aims
B cell targeting agent Rituximab has been proven to be effective and safe for the treatment of idiopathic membranous nephropathy (iMN) in previous studies with nearly 75% achievement of partial or complete remission of nephrotic syndrome. The aim of this study was to evaluate this treatment in a cohort of patients at Complejo Hospitalario de Navarra with relapsing disease.

Method
This is a retrospective, cross sectional study including 12 patients with membranous nephropathy diagnosed by means of a kidney biopsy. All of them were treated before with different immunosuppressive regimens and had a relapse at the time of the inclusion. All of them were treated with two iv infusions of Rituximab. In this study we report patient clinical and immunological baseline characteristics and treatment response at 12 months of follow up.

Results
Between 2015-20 a total of 12 patients (41,6% women and 58,3% men) were treated with two iv infusions of Rituximab with a total mean dose of 2gr. All of them diagnosed previously of iMN, and 10/12 were PLA2r positive. Baseline laboratory data showed serum creatinine levels 1,3±0,9 mg/dl, serum albumin 29±9 g/L, 24 hour- urinary protein excretion of 6,8±3,2 and serum PLA2R levels of 50,54±63,2 RU/ml. Either complete or partial response (CR and PR) were achieved in 83,3% of the cases, however only 3/12 (25%) patients had a complete response at 12 months follow up. All patients who responded had also a significative decrease PLA2r antibodies. Three of the patients who did a PR were treated with an incomplete dose of iv Rituximab (two infusions of 500mg).

Conclusion
Rituximab was effective in our cohort of patients with iMN with achievement of 83,3% response at 12 months of follow up, however only 25% of patients had complete response maybe due to incomplete dosing. Immunological response was seen in all patients. Still, a longer follow up of these patients is needed in order to evaluate Rituximab response.
MO303URINARY ALBUMIN-TO-CREATININE RATIO INDICATES NECROTIZING AND CRESCENTIC GLOMERULONEPHRITIS IN ANCA-ASSOCIATED VASCULITIS
Authors: Hakroush Samy , Tampe Björn 
Published: May 2021

Abstract

Background and Aims
Renal involvement is a common and severe complication of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) as it can cause acute kidney injury (AKI), end-stage renal disease (ESRD) or death. We have previously reported that elevated urinary albumin-to-creatinine ratio (uACR) correlates with rapid deterioration of kidney function in ANCA GN. Therefore, we here aimed to describe the association between proteinuric findings and histopathological diagnosis of necrotizing and crescentic ANCA GN in 50 urinary samples at admission and corresponding renal biopsies of patients with AAV.

Method
A total number of 50 urinary samples at admission and corresponding renal biopsies with confirmed renal involvement of AAV were retrospectively included between 2015 till 2020 in a single-center observational study.

Results
Renal involvement of AAV revealed variable proteinuria ranging from low-range to nephrotic syndromes, however most patients presented with subnephrotic proteinuria predominated by albumin (uACR). Severely increased uACR levels &gt;300 mg/g correlated with reduction of normal glomeruli (P&lt;0.001), attributed to increased glomerular crescents (P&lt;0.001) and necrosis (P=0.008). By contrast, no such association was observed for global sclerotic glomeruli (P=0.58), revealing that uACR reflects necrotizing and crescentic ANCA GN rather than adaptive glomerular hyperfilitration in chronic sclerosing stage. These findings were additionnaly bolstered by histopathological subgrouping and ARRS: patients with uACR levels &gt;300 mg/g were classified either into Berden’s crescentic class (P=0.002) or ANCA renal risk score (ARRS) high/intermediate risk (P=0.003). No association between uACR levels and extrarenal manifestation of AAV disease could be observered, suggesting that uACR levels reflected specific renal involvement with ANCA GN and further confirmed by survival analysis for cumulative incidence of RRT during the short-term course of disease. In summary, uACR measurements at admission were associated with renal biopsy findings thereafter. Levels of uACR &gt;300 mg/g were more frequently observed in necrotizing and crescentic ANCA GN with classification either into Berden’s crescentic class or ARRS high/intermediate risk and specific for renal involvement in AAV.

Conclusion
Early identification of patients who mostly benefit from aggressive immunosuppressive therapy is of clinical importance. Our observation that uACR levels at disease onset predict necrotizing and crescentic ANCA GN requires further investigation for therapeutic decision especially in patients with severe deterioration of kidney function.
MO304COMPLEMENT INHIBITION FOR REFRACTORY GLOMERULONEPHRITIS IN SYSTEMIC VASCULITIS
Authors: Uriol Miguel , Obrador Mulet Aina , Arrufat Gemma , Escriva Ana , Lado Fuentes Mario , Garcia José Luis , Pallares Lucio 
Published: May 2021

Abstract

Background and Aims
Systemic vasculitis(SV) is a life-threatening disease and, in some cases, refractory to intensive multi-immunosuppressant drugs. Complement hyperactivation has gained interest in the pathogenesis of the SV. We report the efficacy of the short course of C5-inhibitor (eculizumab) in refractory cases of lupus nephritis (r-LN) and refractory ANCA-associated glomerulonephritis (r-AGN).

Method
In this retrospective study, eight consecutive patients were assessed (r-LN:n=3 and r-AGN:n=5). All patients were previously treated with three or more drugs included: corticosteroids (n=8), mycophenolate (n=8), rituximab (n=7), immunoglobulins (n=5), therapeutic plasma exchange (n=4), cyclophosphamide (n=1), and belimumab (n=1). Eculizumab was considered for use in off-label indication in patients with progressive renal deterioration (worsening creatinine, protein-to-creatinine ratio) or developing a high-risk lethal complication after the induction immunosuppressive therapy. The histologic lesson observed were: a) r-LN: Type VI (n=1), Type V(n=1), and type IV(n=1), and b) r-AGN: sclerotic (n=3), and malignant hypertension+/-thrombotic microangiopathy (n=1), in one patient who developed pulmonary hemorrhage no renal biopsy was performed.

Results
Mean age (SD): 51(17)years. Median (p25-p75) of follow-up: 19(12-28) months. Overall, 2(25%) of the patients needed chronic renal replacement therapy (one r-AGN patient who required urgent haemodialysis at presentation and one r-LN within 12 months after the onset eculizumab). During the follow-up the eGFR(mean(95%CI) changed from 28(16-40) to 30(20-41)mil/min/1.73m2(P = 0.51) and the median(p25-p75) protein-to-creatinine ratio decreased from 2.3(1.5-6.4) to 0.6(0.3-1.7) mg/mg(P= 0.028). The mean eculizumab cycle dose number was 5(95%CI:3-6), four patients required two cycles. Overall, the mean eculizumab dosage required was 7162 mg (95%CI: 2969-11355). No major side effects were recorded.

Conclusion
The coadyuvant complement inhibition with eculizumab stabilized renal function and decreased proteinuria in six out of the eight previously refractory patients and represents a promising tool in treating lupus and ANCA vasculitis nephritis.