bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0470IS GLYCOSURIA A MARKER OF TUBULO-INTERSTITITAL FIBROSIS AND PROGNOSIS 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
Multiple studies have shown that tubular damage is common in glomerular diseases and that it correlates better with chronic kidney disease (CKD) progression than glomerular lesion itself. The link between glomerular and tubular damage is not entirely established. Glycosuria can be found in (proximal) tubular dysfunction and may be used as a marker of tubular lesion and CKD progression. The aim of this study was to evaluate the association between glycosuria (at the diagnosis) and known histological prognostic markers (glomerular sclerosis (%GS) and interstitial fibrosis/tubular atrophy (IFTA)) and CKD progression, in patients with primary glomerulopathies (GP).

Method
We conducted a 36-month retrospective cohort study with 110 patients with primary GP confirmed by renal biopsy in the last 10 years in our centre – 39 (35.5%) IgA Nephropathy, 27 (24.5%) Membranous Nephropathy, 26 (23.6%) Focal Segmental Glomerulosclerosis and 18 (16.4%) Minimal Change Disease. Patients were divided in two groups according to their glycosuric status at the time of the diagnosis. Data was collected from patients’ charts. Exclusion criteria: patients with diabetes or glucose intolerance, use of SGLT2 inhibitors, secondary GP and transplant kidney patients.

Results
The global prevalence of glycosuria was 9.1% (n=10). Glycosuric patients had, at baseline, higher serum creatinine (3.9±5.1 vs 1.7±1.3mg/dL, p=0.001), higher baseline albuminuria (7.1±6.3 vs 3.2±3.4 g/g, p=0.002) and lower serum albumin (2.3±0.7 vs 3.2±1.1 g/dL, p=0.022). Both groups had similar proportion of patients that underwent immunosuppressive therapy. At the end of the follow-up, in glycosuric patients, only albuminuria was higher (3.3±0.6 vs 0.7±0.8 g/g, p&lt;0.0001); the eGFR decline rate (ml/min/year), 3-year eGFR and 3-year CKD stage 5D incidence were not statistically different. Glomerular sclerosis (%GS) and interstitial fibrosis and tubular atrophy (IFTA) were not different between groups. These results were confirmed by multivariate analysis.

Conclusion
Patients with primary GP with glycosuria at diagnosis had higher baseline creatinine and albuminuria. Even though a worse clinical presentation, glycosuria was not associated with well-known prognostic factors (%GS and IFTA) or CKD progression. We can hypothesize that patients with primary GP with glycosuria have severe diseases at diagnosis, but the lesions may have greater reversibility. Prospective and longer studies are needed to confirm these results.
P0483PREGNANCY OUTCOMES IN PREGNANT WOMEN WITH DIFFERENT TYPES OF GLOMERULONEPHRITIS
Authors: Kirsanova Tatiana , Rumiantseva Elizaveta , Ivanova Olga , Vinogradova Maria , Fedorova Tatiana , Balakireva Alina 
Published: June 2020

Abstract

Background and Aims
Connection between active glomerulonephritis (GN) and adverse pregnancy outcomes is well known. The analysis of pregnancy outcomes for patients with different types of GN in remission has not been performed.
Aim: to investigate pregnancy outcomes in women with inactive lupus nephritis (LN), IgA nephropathy and unspecified types of GN.

Method
A retrospective single-center cohort study 11/2014 – 12/2019: 91 patients with GN in remission &gt; 20 weeks of gestation. Proved IgA-nephropathy was observed in 14 patients, proved LN in 26 patients, 4 patients had proved antiphospholipid syndrome secondary to LN and 51 patients had unspecified types of GN (without morphological confirmation). Morphological class of LN was confirmed in 9 patients: 3rd in 5, 4th in 2, 5th in 2 patients. Renal function at conception, during pregnancy and after delivery, pregnancy outcomes, clinical and laboratory parameters were collected. PE was diagnosed by the 2008 WHO criteria. The sFlt-1/PlGF ratio was used as the main differential marker to exclude GN exacerbation in cases of increased proteinuria.

Results
Rate of preterm birth was significantly higher in patients with LN compared to patients with IgA nephropathy (p = 0.002) and patients with unspecified GN (p &lt; 0.0001), and also in patients with APS plus LN compared to patients with unspecified GN (p &lt; 0.0001) and IgA nephropathy (p = 0.004). PE was observed in 24 patients (25%), type of GN did not affect the incidence of PE. Week of delivery was significantly lower in women with LN compared to IgA-nephropathy (p = 0.013) and unspecified types of GN (p = 0.001) and almost significantly lower in patients with LN plus APS than patients with IgA-nephropathy (p = 0.051) and unspecified types of GN (p = 0.06). Birth weight was significantly higher in babies born to women with unspecified types of GN compared to women with LN (p = 0.018) and LN plus APS (p = 0.047).

Conclusion
Although the rate of PE is similar, the risks of preterm births are high in LN and LN plus APS patients. In this study, laboratory parameters and medical therapy did not significantly affect the pregnancy outcomes.
P0486THROMBOTIC MICROANGIOPATHY IN A PATIENT WITH PERSISTENT CRYOGLOBULINEMIA AFTER HCV ERADICATION AND HETEROZYGOUS DELETION OF CFHR3-CFHR1 REGION: SHOULD WE TREAT WITH RITUXIMAB, ECULIZUMAB OR PLASMAPHERESIS?
Authors: Gallo Pamela , Gallo Pamela , Somma Chiara , Zullo Claudia , Lombardi Marco , Ferro Giuseppe , Dattolo Pietro Claudio 
Published: June 2020

Abstract

Background and Aims
Mixed cryoglobulinemia type II is a systemic vasculitis, caused by deposition of immune complexes in the endothelium of small vessels. Up to 90% are HCV infection related and usually resolve after eradication of the virus.

Method
We report the case of a patient with HCV related type II cryoglobulinemia, persistent despite the eradication of the virus with sofosbuvir and complicated by thrombotic microangiopathy.

Results
Since 2001 patient had cryoglobulinemic vasculitis treated with rituximab in 2002 and in 2011 for a relapse. In 2015 started antiviral therapy with sofosbuvir achieving sustained viral remission.
In December 2018 she admitted to hospital for fever, abdominal pain, diarrhea and oedema.
Blood exams showed acute kidney injury, (scar 3 mg/dl) proteinuria (2,6 g/24h) e microhematuria, haemolytic anaemia and thrombocytopenia, low C4, rheumatoid factor, cryocrit 4% (type II cryoglobulinemia), ANA, ENA e HCV RNA were negatives. On renal biopsy we found a thrombotic microangiopathy (fig.1,2). We rule out a lymphoma through PET total body e immunophenotype.

Conclusion
This report shows persistent cryoglobulinemia with vasculitis relapses, after HCV eradication with direct antiviral therapy (fig. 3), paying attention to complexity of immunological response, causing in this patient a TMA.
Intriguing in our report is the finding of TMA on renal biopsy, without other lesions, contrary to what expected. Cryoglobulinemic vasculitis are usually associated to membranoproliferative glomerulonephritis.
For this reason we search genetic mutation of complement genes, finding a heterozygous deletion on CHFR3-CHFR1 region. A pathogenic role of this deletion, is unknown, but it could mean a different target of therapy.
P0491THE ROUTE TO INDIVIDUALIZED THERAPIES IN PRIMARY MEMBRANOUS NEPHROPATHY: BMI AND KIDNEY OUTCOMES
Authors: Siligato Rossella , Laudani Alfredo , Gembillo Guido , Calabrese Vincenzo , Calderaro Alessandra , Vienna Federica giada , Laurendi Francesco , Cernaro Valeria , Santoro Domenico 
Published: June 2020

Abstract

Background and Aims
A real progress in treating patients more than diseases, would rest on tailored medicine and go beyond the hypothesis that “one size fits all”. Glomerular diseases are not an exception to this consideration, and deserve case-by-case evaluation for choosing both the most suitable therapeutic agent and its appropriate dose, because a standardized treatment may result in worse prognosis.
We analyzed the role of body mass index (BMI) in modifying renal outcomes of patients with nephrotic syndrome due to membranous nephropathy (MN) treated with immunosuppressive drugs.

Method
We performed a retrospective analysis of 29 MN patients related to our center (M 19, F 10), checking their anti PLA2R status at kidney biopsy time and any change in proteinuria, serum creatinine (SCr) and body mass index (BMI) between diagnosis and at last follow-up visit, in search of a correlation with their response to immunosuppressive treatment. Data with non normal distribution are expressed as median and interquartile range [IQR].

Results
The median follow-up of our patients lasted 49 months [IQR 14-80]. At biopsy time, almost half of them was anti-PLA2R positive (n=14 subjects) while the other resulted to be negative and were thus screened for secondary causes of MN (n=15). Irrespective either of anti-PLA2R status or the administration of Ponticelli regimen or Rituximab, complete remission was achieved in 31% of patients, while 34.5% experienced a partial remission and 34.5% resulted non responder to at least a therapeutic protocol. Median proteinuria reduction was of 3.732g/24h [IQR 7.636-1.77 g/24h]. Median rise in SCr was 0.140 mg/dl [IQR -0.11 – 0.4 mg/dl]. Direct association between the proteinuria/BMI ratio at diagnosis and remission of proteinuria at last follow up was statistically significant (r=-0.7506; P&lt;0.0001) (Fig.1).

Conclusion
Albeit remission is not directly correlated with basal BMI, our results appear to confirm that an appropriate drug posology, not only according to patients’ renal or hepatic function but also to their body mass, may result in more effective individualized therapies in MN; nevertheless, this recorded association must be confirmed by large scale randomized clinical trials.
P0496THE IMPORTANCE OF COMPLEMENT LEVELS AND CLINICAL CHARACTERISTICS OF PRIMARY MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS IN TURKEY
Authors: Tatar Erhan , Oygar Deren , Seyahi Nurhan , Eren Necmi , Cantürk Yağmur , Güngör Özkan , Derici Ülver , Sipahioğlu Murat , Yıldız Abdülmecit , Karadağ Serhat , Sumnu Abdullah , Odabas Ali Riza , Turgut Didem , Cavdar Caner , SAHIN Garip , Dursun Belda , Guven Taymez Dilek , Yılmaz Aydın Fatma , Ulu Memnune Sena , Basturk Taner , Yadigar Serap , Bozaci Ilter , Behlül Ahmet , Sipahi Savaş , Bicik Bahçebaşı Zerrin 
Published: June 2020

Abstract

Background and Aims
Membranoproliferative glomerulonephritis (MPGN) may be caused by disturbances in the complement system. Hypocomplementemia is a characteristic of MPGN. Among these patients frequency of hypocomplementemia and their relation with clinical and histopathological findings are still not clearly known. The aim of this study is to evaluate the hypocomplementemia frequency among the primary MPGN patients in Turkey and its relation with histopathological subtypes, findings and demographic characteristics.

Method
The data was obtained from national multicentered (47 centers) records of primary glomerular diseases in Glomerulonephritis Study Group of the Turkish Society of Nephrology database from May 2009 to June 2019. Primary MPGN cases were evaluated and divided into 2 groups with and without hypocomplementemia.

Results
In total, 193 cases were included in the study. The indications for renal biopsy were isolated nephrotic syndrome (55.4%), nephritic syndrome (21.2%), nephrotic syndrome with a nephritic component (6.2%) and asymptomatic urinary findings (17.2%). 34.2% of the cases were reported as immune complex type, another 34.2% of the cases were reported as C3 glomerulopathy. 31.6% of patients were not specified. 82 (42.4%) of the cases presented hypocomplementemia. Hypocomplementemic patients were younger (34±14 vs 41±15, p=0.002) and most frequently female (56% vs 41%, p=0.03). At same time, serum albumin and hemoglobin levels were lower and anemic patient rates were higher (p&lt;0.001). In this group, the nephrotic syndrome rates were higher (72% vs 54%, p=0,01). Hypocomplementemia rate in the C3 glomerulopathy was 52% (in DDD 40%, in C3GN 55% ) whereas in immune complex type 41% and in non specified type 34%. In the pathological evaluation; among the hypocomplementemic group the endocapillary proliferation and glomerular exudative differentiation rates presented significant increase. However the chronicity index were lower in the kidney biopsy (Table 1).

Conclusion
This multicenter study provided important data about the epidemiology of MPGN with importance of hypocomplementemia in Turkey. Hypocomplementemia is associated with both subtypes of MPGN patern, anemia and nephrotic syndrome. At the same time, hypocomplementemia is a valuable parameter for active MPGN pattern in the histopathological evaluation. This may be important in determining the treatment.
P0499SPONTANEOUS REMISSION IN PRIMARY MEMBRANOUS NEPHROPATY WITH POSITIVE ANTIPLA2R ANTIBODIES
Authors: ORTEGO SOFIA , Villacorta Javier , Fernandez Lucas Milagros , Carrasco Angela , RODRIGUEZ MENDIOLA NURIA , CASILLAS ESTHER , Lopes Vanessa , DIAZ MARTA , Rita Claudia , GAÑAN ISRAEL 
Published: June 2020

Abstract

Background and Aims
Patients with primary membranous nephropaty experience a spontaneous remission rate between 30-40%. AntiPLA2R antibodies are involved in the pathogenesis of the disease. Some studies demonstrated that levels of circulating anti-PLA2R in the beginning of the disease revealed a strong correlation with clinical disease activity, and it could help discriminate against those who will make spontaneous remission

Method
The principal objetive was to analize the spontaneous remission rate in patients with membranous nephropaty with positive antiPLA2R antiboidies and study clinical and serological variables associated. A retrospective analysis studied the assocaition between analitis, clinical and serological variables of patients with with spontaneous remission and membranous nephropaty who were diagnosed in between 2015 and 2018 in Ramon y Cajal Hospital.

Results
We included 13 patients with primary positive antiPLA2R antibodies membranous nephropaty. Patients ha a mean age of 61,6 ± 20 and a 64.3% were males. All of them had nephrotic syndrome in the onset disease. Patients were aged 6,6 ± 3.8 gr/24horas, in serum albumine 2.3 ± 0.5 g/L and the serum Creatinine (Cr) was 1 ± 0.5 mg/dl. Medium AntiPLA2R tittle was ±. All the patients were treated at the diagnosis time with ARB/ACE inhibitors . 7 of the 13 (53.8%) patients experienced a spontaneous remission of the nephrotic syndrome. The mean time to spontaneous remission was 4.8 ± 2.4 months. Patients who presented spontaneous remission debuted with lower serum Cr at debut. No significative differences were found in between remisión rate in association with total proteinuria neither PLA2R titles in the beginning.

Conclusion
In our experience, approximately a 50% of patients with membranous nephropaty experienced an spontaneous remission. There was no significative association in between AntiPLA2R titles and spontaneous remission rate in our study. Patients with better renal function in the diagnosis were more frequently associated with spontaneous remission.
P0500RESULTS OF A PHASE 1 TRIAL TO INVESTIGATE THE SAFETY, TOLERABILITY, PHARMACOKINETICS, AND PHARMACODYNAMICS OF BION-1301 IN HEALTHY VOLUNTEERS
Authors: Lo Jeannette , Van Zandvoort Peter , Van de Crommert Jos , Endsley Aaron , Müller Thomas , Honarmand Somayeh , Mittan Angelique , Barratt Jonathan , Walling Jackie , Essayan David 
Published: June 2020

Abstract

Background and Aims
IgA nephropathy (IgAN), the leading cause of primary glomerulonephritis, is an autoimmune disease with no approved treatments. Slow progression to end-stage-renal disease occurs in up to 50% of patients with IgAN over approximately 20-25 years, resulting in the need for dialysis or kidney transplant. A critical step in IgAN pathogenesis is the production of galactose-deficient IgA1 (Gd-IgA1) leading to the generation of anti-Gd-IgA autoantibodies and the formation of immune complexes that result in kidney inflammation and damage. Patients with IgAN have elevated levels of A proliferation-inducing ligand (APRIL), which may contribute to IgAN pathogenesis by regulating differentiation and proliferation of B cells. In a study of patients with IgAN, those with high plasma APRIL levels had higher levels of Gd-IgA1 and proteinuria and lower estimated glomerular filtration rates compared to those with lower plasma APRIL levels. BION-1301 is a first-in-class humanized antagonistic antibody targeting APRIL. BION-1301 reduced serum levels of IgA, IgM, and IgG with no drug-related toxicity in nonhuman primates and was well-tolerated with no dose-limiting toxicities in a Phase 1/2 first-in-human clinical study in patients with late-stage multiple myeloma. The Phase 1 trial described here will characterize the safety, pharmacokinetic (PK), and pharmacodynamic (PD) profiles of BION-1301 in healthy volunteers (HV) and patients with IgAN.

Methods
The protocol for this study (NCT03945318) is comprised of 3 parts, of which only Parts 1 and 2 are complete. Parts 1 and 2 assess BION-1301 in HV, and Part 3 will assess BION-1301 in patients with IgAN. Parts 1 and 2 are double-blind, randomized, placebo-controlled single and multiple ascending dose designs, respectively. Part 3 will assess a multiple dose regimen in IgAN patients. Part 1 enrolled 36 HV in 5 dose cohorts, randomized in a 3:1 ratio to receive a single dose of BION-1301 or placebo delivered by IV infusion. Part 2 enrolled 27 HV in 3 dose cohorts, randomized in a 2:1 ratio to receive 3 doses of BION-1301 or placebo delivered by IV infusion once every two weeks over a one month period. Dose escalation decisions were made by a Safety Review Team based on safety, tolerability, and PK-related stopping criteria.

Results
To-date, BION-1301 continues to be well tolerated. Its biological activity in the study is characterized by free APRIL, IgA, IgG, and IgM levels; unblinded safety, PK, and PD results for Parts 1 and 2 in Healthy Volunteers will be presented.

Conclusion
BION-1301, a humanized anti-APRIL antibody, may provide a novel avenue for the potential treatment of IgAN that offers potential advantages compared to currently used antihypertensive or immunosuppressive therapies.
P0509CLINICAL ANALYSIS FOR PROGNOSTIC FACTORS BASED ON RESULTS OF VERY SHORT-TERM TREATMENT IN MEMBRANOUS NRPHROPATHY
Authors: Iwashita Takatsugu , Takayanagi Kaori , Yamasaki Maiko , Aoyagi Mai , Shimizu Taisuke , Hasegawa Hajime 
Published: June 2020

Abstract

Background and Aims
Membranous nephropathy (MN) is known to affect frequently in elderly patients.
Since the duration of MN treatment is likely to be prolonged, it is desirable that the treatment duration would be shortened by the concomitant use of immunosuppressant and prednisolone (PSL). Therefore, we evaluated prognostic factors using the results of treatment in a very short period of time in patients with membrane nephropathy at our hospital.

Method
Biopsy-proven 66 cases with MN, hospitalized between April 2009 and December 2017, were enrolled in this study.
All cases were divided between two groups, high responder and low responder, based on the 50% proteinuria-reduction ratio at a month after the beginning of therapy including sole administration of PSL and concomitant use of immunosuppressants such as Cyclosporin or Mizoribine.
High responder and low responder were comparatively studied.
All biopsy specimens were stained by anti-phospholipase A2 receptor (PLA2R) and thrombospondin type 1 domain containing 7A (THSD7A) antibodies by standard protocol.

Results
Estimated glomerular filtration rate (eGFR) was 70.5 vs 60.4 ml/min (p = 0.087), showing no difference.
Baseline urine protein-to-Cr ratio (PCR) and degree of hematuria in high responder were significantly higher than those in low responder; baseline PCR: (6.95 vs 3.86, p = 0.003), degree of hematuria: (1 vs 0, p = 0.036)
There was no difference in intensity of immunofluorescent staining of IgG, A, M, C3 between high responder and low responder. We also studied the difference in immunofluorescent intensity of PLA2R, THSD7A and IgG subclass. Intensity of IgG3 and ratio of PLA2R/IgG4 intensity in low responder were significantly higher than those in high responder; IgG3 intensity: (0 vs 0.5, p = 0.049), PLA2R/IgG4 ratio: (0.58 vs 1.00, p = 0.029)

Conclusion
Obtained results showed that higher baseline PCR and degree of hematuria might be related to the rapid therapeutical response.
On the other hand, higher staining intensity of IgG3 and PLA2R/IgG4 intensity ratio might reduce the response of treatment.
P0510INFLAMMATORY BOWEL DISEASE IN KIDNEY TRANSPLANTATION:CASE REPORT
Authors: Gana Abir 
Published: June 2020

Abstract

Introduction
Kidney transplantation has become the treatment of choice for chronic end-stage renal disease because it improves not only the quality of life of patients but also their survival rate. However, several complications can be observed in transplant patients such as immune rejection, infections and recurrence of initial nephropathy etc.

Case summary
A 21-year-old man diagnosed with chronic end-stage renal failure 5 years ago with a renal biopsy showing an aspect of proliferative glomerulonephritis.
The study of the alternate complementary path show a C3 low level, C4 and CH50 normal level, H factor and I factor was normal too. The patient presented purpuric skin lesions with a biopsy of granular deposits of C3. We started extra renal purification by peritoneal dialysis and the patient had, one year later, a kidney transplant from his mother. Ten month later, we have notice a progressive renal function decline with an aspect of proliferative membranous glomerulonephritis with deposits predominant of c3 in graft biopsy. The diagnosis of the recurrence of the initial nephropathy was made but the treatment was delayed by the onset of cutaneous leishmaniosis. Few months later the patient was presented with diarrhea lasting more than a month, which led us to do a colonoscopy, which objectified an aspect of chronic inflammatory bowel disease evoking earlier a Crohn's disease.
Therefore, we stopped tacrolimus and put the patient on full dose corticosteroid therapy and azathioprine without having a remission, and appearance of skin lesions at the elbow and pelvis evoking pyoderma gangrenosum in skin biopsy. In addition, the patient has presented an ischemic retinal damage probably related to a deregulation of the alternate complement pathway.

Discussion
The complement system is not only a host defense system against microbes, but also a surveillance system that contributes to maintain tissue homeostasis and tissue repair. However, uncontrolled complement activation can induce tissue damage, and inappropriate activation is involved in the pathogenesis of various conditions like autoimmune diseases, sepsis and transplant and extensive inflammation and tissue damage. The impairment of alternative path can explain the extra-renal manifestation of this patient especially the appearance of inflammatory bowel disease witch can be explained by impaired local immune defense in the gut.

Conclusion
Immune system abnormalities can occur after kidney transplantation this leads to the appearance of several inflammatory abnormalities such as chronic inflammatory bowel disease which may in our case be due to an abnormality of the alternate pathway of the complement.