bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0477CLINICOPATHOLOGICAL STUDY OF MIXED CRYOGLOBULINEMIC GLOMERULONEPHRITIS SECONDARY TO HEPATITIS B VIRUS INFECTION
Authors: Li Chao , Li Hang , Su Wei , Wen Yu-bing , Ye Wei , Ye Wen-ling , Qin Xu-zhen , Cai Jian-fang , Li Xuemei , Li Xuewang 
Published: June 2020

Abstract

Background and Aims
To describe the clinical features, renal pathology findings and prognosis in patients with mixed cryoglobulinemic glomerulonephritis (Gn) caused by hepatitis B virus (HBV) infection.

Method
This was a retrospective study including seven Chinese patients with HBV infection associated mixed cryoglobulinemic Gn in a tertiary referral hospital from April, 2016 to March, 2019. The demographic, clinical, pathological characteristics, treatment and follow-up data were collected and analyzed.

Results
Age at renal biopsy was 47±12 years, including three females and four males. 24hUP was 5.6(3.0, 6.6)g/d and five cases presented with nephrotic syndrome. The median baseline eGFR(CKD-EPI) was 23.5(20.2, 46.3) ml/min per 1.73m2. The extrarenal manifestations were: purpura (n=6), arthralgia (n=1), peripheral neuropathy (n=1), and cardiomyopathy (n=1). Six cases had type II cryoglobulinemia with IgMκ, the other one had type III. The median cryocrit was 4.0 (1.0, 15.0) %, rheumatoid factor was 368(117, 733) IU/ml, C3 was 0.48(0.41, 0.57) g/L, C4 was 0.013(0.003, 0.118) g/L. Renal pathologic findings on light microscopy: endocapillary proliferative Gn (n=3), membranoproliferative Gn (n=3), and mesangial proliferative glomerulonephritis(n=1). Hyaline thrombi were seen in four cases, while crescents were found in two cases. On immunofluorescence microscopy, the predominant types of immunoglobulin deposits were: IgM(n=5), IgA(n=1), and codominance of IgG and IgA(n=1). HBsAg and HBcAg deposits were found in only one case. Ultrastructural studies showed granular subendothelial and mesangial electron-dense deposits in all patients and organized microtubules was seen in only one case. All patients received antiviral medication (entecavir, n=6; lamivudine, n=1). They were given corticosteroid alone(n=2) or combined with cyclophosphamide(n=4) or mycophenolate mofetil(n=1). Two patients received plasmapheresis. The median follow-up time were 18 (6, 37) months. One patient died from pneumonia, and one progressed to end stage of renal disease (ESRD). At endpoint of follow-up, 24hUP was 2.1 (0.8-5.2) g/d, and eGFR (CKD-EPI) was 55.3 (20.7, 111.8) ml/min per 1.73m2.

Conclusion
Mixed cryoglobulinemic Gn should be screened for HBV etiologies, especially in HBV-endemic country. Endocapillary proliferative Gn was the common pathologic type, as well as membranoproliferative Gn. Diagnosis and treatment in early stage benefit patients’ renal outcome. Long-term prognosis should be investigated in further studies.
P0484CLINICAL FEATURES AND OUTCOME OF LUPUS NEPHRITIS IN MOROCCO: A SINGLE CENTER STUDY OF 207 CASES
Authors: Naouaoui Salma , Chettati Meriem , Fadili Wafaa , Laouad Inass 
Published: June 2020

Abstract

Background and Aims
renal involvement is a common and potentially severe complication of systemic lupus erythematosus (SLE). Few data for Lupus nephritis (LN) exist on North Africans, especially those from Morocco. The aim of our study was to report the long-term renal outcome of a cohort of Moroccan patients with LN.

Methods
the records of 207 patients (191 females, 16 males) with LN (American College of Rheumatology criteria) admitted to the departments of nephrology and internal medicine of Marrakesh University Hospital were reviewed and included in a retrospective analysis.

Results
the mean age at the time of renal biopsy was 31,7 ±  15,9 years. Time between diagnosis of SLE and the onset of renal disease was 29 ± 10 months. Renal involvement was observed concomitantly with the onset of SLE in 39 patients (18,8%). At presentation, 65 patients (31,4%) had hypertension. Hematuria was seen in 158 patients (76,3%), nephrotic syndrome in 82 patients ( 39,6%) and renal failure in 76 patients (36,7%). The most frequent extarenal manifestations were skin and joint manifestations in 89,3% and 83,6% respectively. The most common histological finding was type IV LN in 96 patients, followed by type III LN in 51 patients. 191patients (92,3%) received corticosteroids as initial therapy followed by immunosuppressive therapy in 127 patients (61,3%). During the follow-up period, partial or total remission was obtained in 56,5% of cases while 61 patients (29,4%) progressed to end stage renal disease (ESRD) and 29 patients (14%) died. Logistic regression detected that hypertension, high serum creatinine at admission and proliferative histological classes were the main risk factors for poor renal outcome.

Conclusion
Our results showed that LN was particularly severe in our population compared with other published data with predominance of proliferative forms.
P0489URINARY PROTEOMIC MARKERS OF MEMBRANOUS NEPHROPATHY
Authors: Moszczuk Barbara , Krata Natalia , Cysewski Dominik , Perkowska-Ptasinska Agnieszka , Pączek Leszek , Foroncewicz Bartosz , Zagożdżon Radosław , Mucha Krzysztof 
Published: June 2020

Abstract

Background and Aims
Membranous nephropathy (MN) is considered one of the major conditions causing nephrotic-range proteinuria. Last years brought a huge progress in the field of non-invasive diagnostics, mainly due to the PLA2R antibody use in diagnostics and follow-up of primary MN. However, our understanding of MN is far from complete, especially that PLA2R-antibodies are found only in approximately 60-70% of all cases. Urinary proteomics is a promising tool that can help us to improve diagnostics, monitoring and understanding of MN. Our previous studies (Patent No. WO/2017/212463) suggested that specific urinary proteins can be used in the diagnostics of IgA nephropathy, lupus nephritis and ADPKD. The aim of this study was to evaluate with Mass Spectrometry (MS) patients diagnosed with MN in comparison to healthy controls.

Method
This study included patients with biopsy-proven MN (25) and healthy controls (7). Urine samples were obtained from a midstream of the second- or third-morning (SPOT) sample. The samples were processed up to 2 h after collection and stored at -80°C for further measurements with MS. The results were related to demographic data, standard laboratory tests and GFR estimated with use of Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation.

Results
The signal intensity of urine protein A, B and C (patent pending and the name of proteins disclosed until the date of ERA-EDTA Congress) was found to be significantly higher (p &lt; 0.05) in MN patients compared to healthy controls (Fig. 1). Mass spectrometry, according to the specific amino-acids fragments of each tested protein, confirmed the differences between tested and control group. Additionally, statistically significant differences exist between patients with different types of glomerulonephritides (comparative MS in progress).

Conclusion
The signal intensity of urine proteins A, B, C is elevated in MN and according to our study, vary depending on types of nephropathies. This observation might suggests their differential roles in the pathophysiology of MN and its’ possible application as a non-invasive diagnostic and prognostic marker. Further studies are desired to establish the role of these urinary proteins.
P0493EPIDEMIOLOGY OF IGA NEPHROPATHY IN THE MALTESE ISLANDS
Authors: Attard Abigail , Vassallo Diana , Swayne John , Buttigieg Jesmar , Grech Edward , Vella Mario Pio , Farrugia Emanuel 
Published: June 2020

Abstract

Background and Aims
IgA nephropathy is the most common cause of glomerular disease worldwide. Natural history and clinical progression of this condition are highly heterogenous and can vary from asymptomatic haematoproteinuria, to a rapidly progressive glomerulonephritis that results in end-stage kidney disease (ESKD). The OXFORD MEST-C score is a set of consensus criteria that have been correlated with renal outcomes independent of baseline clinical features, proteinuria and blood pressure control. This is the first observational study carried out in the Maltese Islands that aims to evaluate the epidemiology and the histopathological distribution in Malta.

Method
All patients with biopsy-proven IgA nephropathy between January 2013 and July 2019 in our centre were recruited into this longitudinal retrospective observational study. Baseline data at time of biopsy included demographics, laboratory data and the MEST-C score. Study end-points included death, doubling of serum creatinine and ESKD. Data was censored on 31/12/2019.

Results
A total of 46 patients were recorded over this 6 year period, with a median follow-up of 2.7 years (Standard deviation 1.9). The median age of our cohort at time of biopsy was 46.1 years (Standard deviation 16.1). In total, 71.7% were males, 34.8% were hypertensive and 6.5% diabetic at baseline. At time of biopsy, 89.1% had an active sediment, 54.3% had established chronic kidney disease (CKD), 17.4% presented with acute kidney injury (AKI), 13% had a history of synpharyngitic haematuria, while 4.3% had a history of Henoch–Schönlein (HSP). In our cohort, 8 patients (17.4%) had progression of CKD. 4 of them had doubling of creatinine with 3 reaching ESKD. 1 patient died. Table 1 shows the distribution of histopathological findings in our cohort:

Conclusion
Despite the limited study sample, short follow-up time, and absence of treatment data, our results demonstrate that the distribution of histopathological variants in our cohort is similar to that reported elsewhere in the literature1.
Barbour SJ, Espino-Hernandez G, Reich HN, Coppo R, Roberts IS, Feehally J, et al. The MEST score provides earlier risk prediction in IgA nephropathy. Kidney Int. 2016 Jan;89(1):167–75.
P0495PAUCI-IMMUNE GLOMERULONEPHRITIS - OUR EXPERIENCE IN A TERTIARY CARE CENTER
Authors: Lingaraj Umesh , Mehta Ricken , SM Shivaprasad , V Leelavathi , A Kishan , C Sreedhara , V MYTHRI 
Published: June 2020

Abstract

Background and Aims
Aims - To study the clinicopathological profile and short-term outcome of patients with Pauci-immune Glomerulonephritis at a tertiary care center

Method

Results
In our study group we found that majority of the patients had RPGN presentation (93.3%).

Conclusion
P0497MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS IN A LMIC : EPIDEMIOLOGIC PROFILE, PROGNOSTIC FACTORS AND IMPACT OF ALPHA SMOOTH MUSCLE ACTIN
Authors: Ikram Agrebi , Khawla Kammoun , Najla Dammak , Soumaya Yaich , Tahya Boudawara , Jarraya Fayçal , Mohamed Ben hmida 
Published: June 2020

Abstract

Background and Aims
Mesangiocapillary glomerulonephritis (MCGN) is a rare kidney disease with a poor prognosis. Several factors have been described associated with poor renal prognosis. The contribution of alpha smooth muscle actin (α-SMA), which is involved in renal fibrogenesis, has been demonstrated in assessing the prognosis of several glomerular diseases, but few studies have concerned the MCGN.
The aim of our study is to determine the epidemiologic profile, to identify prognostic factors and to evaluate the contribution of α-SMA in predicting the prognosis the MCGN.

Method
Our study is retrospective over a period of 16 years (January 1996- December 2011) including all cases of primary MCGN aged more than 15 years, collected at the nephrology department of the university hospital Hedi Chaker Sfax. We reviewed available renal biopsies for the study of the expression of α-SMA by immunohistochemistry.

Results
We collected 118 cases of primary MCGN, with mean age of 45 ± 19 years. The incidence of MCGN has decreased from 10 cases / year between 1996 and 1999 to 5 cases / year between 2008 and 2011. 78.8 % of patients had renal failure at the moment of diagnosis. The study of the expression of α-SMA by immunohistochemistry was made in 16 cases. After a mean follow-up of 51.9 ± 44 months, progression to end stage renal failure (ESRF) was observed in 43.5% of cases. Poor prognostic factors were initial renal failure and sclerotic glomeruli (respectively p at 0,04 and 0,03), but interstitial fibrosis degree was not a prognosis predictor parameter while important interstitial expression of α-SMA was a marker of the evolution to ESRF (p = 0.03).

Conclusion
MCGN has significantly declined over recent years due to improve of socio-economic conditions. The presence of renal failure and sclerotic glomeruli at diagnosis were associated with a poor prognosis. The interstitial expression of α-SMA may be a useful marker to predict the progression of primary MCGN even before the installation of fibrosis.
P0501THE EPIDEMIOLOGICAL FEATURES OF PIRMARY GLOMERULAR DISEASES IN TURKEY: THE MULTICENTER STUDY OF TURKISH SOCIETY OF NEPHROLOGY GLOMERULAR DISEASES (TSN-GOLD) WORKING GROUP
Authors: Turkmen Aydin , Sumnu Abdullah , Cebeci Egemen , Yazici Halil , Eren Necmi , Seyahi Nurhan , Dilek Kamil , Dede Fatih , Derici Ülver , Unsal Abdulkadir , SAHIN Garip , Sipahioglu Murat , Gok Mahmut , Tatar Erhan , Dursun Belda , Sipahi Savaş , Yılmaz Mürvet , Suleymanlar Gultekin , Ulu Memnune Sena , Gungor Ozkan , Kutlay Sim , Bicik Bahçebaşı Zerrin , Sahin İdris , Kurultak Ilhan , Turkmen Kultigin , Yilmaz Zulfikar , Turan Kazancioglu Rumeyza , Cavdar Caner , Candan Ferhan , Aydin Zeki , Oygar Deren , Gul Cuma Bulent , Arici Mustafa , Paydas Saime , Guven Taymez Dilek , Kucuk Mehmet , Trablus Sinan , Turgutalp Kenan , Koc Leyla , Sezer Siren , Duranay Murat , Bardak Simge , Altintepe Luftullah , Arikan Izzet Hakki , Azak Alper , Odabas Ali Riza , Manga Sahin Gulizar , Ozturk Savas 
Published: June 2020

Abstract

Background and Aims
The largest data on the epidemiology of primary glomerular diseases (PGD) are obtained from the databases of countries or centers. Here, we presented the extended results of the Primary Glomerular Diseases Study of the Turkish Society of Nephrology Glomerular Diseases (TSN-GOLD)Working Group.

Method
The data of patients who underwent renal biopsy and diagnosed as primary glomerular disease were recorded in the database prepared for the study. Between May 2009 and May 2019, a total of 4399 patients from 47 centers were evaluated. Basal data of 3875 patients were analyzed after exclusion of those lacking light microscopy and immunofluorescence findings.

Results
The mean age was 41.5 ± 14.9 years. Of the patients, 1690 were female (43.6%) and 2180 (56.3%) were male. Nephrotic syndrome was the most common biopsy indication (51.7%). This was followed by asymptomatic urinary abnormalities (18.3%) and nephritic syndrome (17.8%). The most common PGH was IgA nephropathy (25.7%), followed by membranous nephropathy (25.6%) and FSGS (21.9%). The mean total number of glomeruli per biopsy was 17 ± 10. Mean baseline systolic blood pressure was 130 ± 20 mmHg and diastolic blood pressure was 81 ± 12 mmHg. Median proteinuria was 3300 (IQR: 1467-6307) mg / day, mean serum creatinine, estimated GFR and albumin values were 1.4 ± 1.5 mg / dl, 80.7 ± 39.1 ml / min and 3.2 ± 0.9 g / dl, respectively.

Conclusion
In Turkey, the incidence of IgA nephropathy patients have become more common than membranous nephropathy among PGD patients diagnosed with renal biopsy.
P0502BIOBANKING FOR GLOMERULAR DISEASES: A STUDY DESIGN AND PROTOCOL FOR KOREA RENAL BIOBANK NETWORK SYSTEM TOWARD NEXT-GENERATION ANALYSIS (KORNERSTONE)
Authors: Kang Eunjeong , Kim Yaerim , Kim Yong Chul , Lee Soojin , Han Seungyeup , Choe Minsun , Hwang Jin Ho , Lee Sunhwa , Park Ji In , Park Jung Tak , Lim Beom Jin , Lee Jung Pyo , An Jung Nam , Ryu Dong-Ryeol , Kim Jung-Hyun , Kang Hee Gyung , Lee Hyun Soon , Moon Kyung Chul , Joo Kwon Wook , Oh Kook-Hwan , Han Seung Seok , Lee Hajeong , Kim Dong Ki 
Published: June 2020

Abstract

Background and Aims
Glomerular diseases, a set of debilitating and complex disease entities, are related to mortality and morbidity. To gain insight into pathophysiology and novel treatment targets of glomerular disease, various types of biospecimens linked to deep clinical phenotyping including clinical information, digital pathology, and well-defined outcomes are required. We provide the rationale and design of the KOrea Renal biobank NEtwoRk System TOward Next-generation analysis (KORNERSTONE).

Method
The KORNERSTONE, which has been initiated by Korea Centres for Disease Control and Prevention, is designed as a multi-centre, prospective cohort study and biobank for glomerular diseases. Clinical data, questionnaires will be collected at the time of kidney biopsy and subsequently every one year after kidney biopsy. All of the clinical data will be extracted from the electrical health record and automatically uploaded to the web-based database. High-quality digital pathologies are obtained and
connected in the database. Various types of biospecimens are collected at baseline and during follow-up: serum, urine, buffy coat, stool, glomerular complementary DNA (cDNA), tubulointerstitial cDNA. All data and biospecimens are processed and stored in a standardised manner. The primary outcomes are mortality and end-stage renal disease. The secondary outcomes will be deterioration renal function, remission of proteinuria, cardiovascular events and quality of life.

Disussion
Ethical approval has been obtained from the institutional review board of each participating centre and ethics oversight committee. The KORNERSTONE is designed to deliver pioneer insights into glomerular diseases. The study design allows comprehensive, integrated and high-quality data collection on baseline laboratory findings, clinical outcomes including administrative data and digital pathologic images. This may provide various biospecimens and information to many researchers, establish the rationale for future more individualised treatment strategies for glomerular diseases.

Conclusion
In conclusion, we describe the objectives and clinical protocol for the KORNERSTONE. As the first large-scale glomerulonephropathy cohort study with the integration of clinical data, biospecimens and digital pathologic images in Korea, the KORNERSTONE will help to clarify the natural course, complication profiles, and novel treatment targets of the Asian population with glomerular disease.
P0518VNN1 MEDIATES RENAL MALADAPTIVE REPAIR AFTER AKI BY INDUCING PREMATURE SENESCENCE OF RENAL TUBUAR CELLS
Authors: He Yani , Chen Kehong , Chen Jia 
Published: June 2020

Abstract

Background and Aims
Renal maladaptive repair after acute kidney injury (AKI) can easily progress to chronic kidney disease. Sustained renal interstitial damage caused by accelerated senescence of renal tubular cells leads to renal fibrosis. Vanin-1 (VNN1) is an extracellular enzyme with panthenylmethylaminease activity that indirectly reduces the synthesis of glutathione, causing oxidative stress. The purpose of this study was to investigate the expression and of VNN1 in renal tissue and to study its role in senescence of renal tubular cells after ischemia reperfusion (I/R).

Method
hirty male wild BALB/c mice were randomly divided into control group, sham group and I/R group. In the I/R group, the bilateral renal pedicle was ligatured for 35 min followed by reperfusion. The expression of VNN1 and aging markers (P16, P21, SA-b-gal) were detected. Furthermore, wild type mice and VNN1 knockout mice were used to compare the degree of renal tissue and functional damage and the senescence of renal tubular cells after I/R injury.

Results
Compared with sham group, Scr, BUN and renal injury score increased significantly in I/R group at the early stage (3d) of renal injury. Renal fibrosis was observed in the later stage (28-42d). The expression of VNN1 in renal tubular cells of I/R group increased after I/R injury. VNN1 was coexpressed with P16, suggesting that VNN1 might be related to cellular stress senescence. The SCr, BUN of VNN1 KO mice was significantly lower than that of wild type mice at 7-28 d after renal reperfusion. The renal interstitial injury score of VNN1 KO mice was significantly lower than that of wild type mice, and the renal interstitial fibrosis level was significantly higher than that of wild type mice at 42d after reperfusion. The results suggest that VNN1 KO promotes renal repair of AKI. The ratio of P16 positive tubule cells in VNN1 KO mice was significantly higher than that in wild-type mice at 7d after renal reperfusion, suggesting that VNN1 could promote the senescence of renal tubule cells during AKI repair.

Conclusion
VNN1 mediates renal maladaptive repair after AKI by inducing premature senescence of renal tubular cells.
P0522INHIBITING 15-PGDH PREVENTS ISCHEMIC RENAL INJURY BY A PGE2/EP4 SIGNALING PATHWAY MEDIATING VASODILATION, INCREASED RENAL BLOOD FLOW, AND INCREASED ADENOSINE/A2A RECEPTOR
Authors: KIm Byeong Woo , Kim Sun hee , Bae Ki beom 
Published: June 2020

Abstract

Background and Aims
We demonstrate the marked activity of SW033291, an inhibitor of 15-hydoxyprostaglandin dehydrogenase (15-PGDH), in preventing acute kidney injury (AKI) in a murine model of ischemia reperfusion injury (IRI). AKI due to ischemic injury represents a significant clinical problem. Prostaglandin E2 (PGE2) is vasodilator in the kidney, but is rapidly degraded in vivo due to catabolism by 15-PGDH. We investigated the potential of SW033291, a potent and specific 15-PGDH inhibitor, as prophylactic treatment for ischemic AKI.

Method
10-week aged male C57/BL6 mice were randomly allocated to five groups (n=8 to 15 in each group): the sham-control group, sham-SW033291 group, IRI-vehicle group, IRI-indomethacin group and the IRI-SW033291 group. IRI were induced by clamping bilateral renal artery for 30 min followed by 24 hours of reperfusion. Vehicle, indomethacin, or SW033291 were intraperitoneally administered three times at 1 hour before, immediately after, and 12 hours after IRI. Renal function, histological changes, and renal blood flow were compared and the relevant parameters of oxidative stress and inflammation were detected.

Results
Prophylactic administration of SW033291 significantly increased renal tissue PGE2 levels and increased post-AKI renal blood flow and renal arteriole area. In parallel, prophylactic SW033291 decreased post-AKI histologic injury score and tubular apoptosis and markedly reduced biomarkers of renal injury that included BUN, creatinine, NGAL and KIM-1. Prophylactic SW033291 also reduced post-AKI induction of proinflammatory cytokines, high mobility group box 1 (HMGB1), and malondialdehyde (MDA). Protective effects of SW033291 were mediated by PGE2 signaling as they could be blocked by pharmacologic inhibition of PGE2 synthesis or of the EP4 type PGE2 receptor. Consistent with activation of PGE2 signaling, SW033291 induced renal levels of both EP4 and of cyclic adenosine monophosphate (cAMP), along with other vasodilatory effectors including AMP, adenosine, and the adenosine A2A receptor (A2A). Protective effects of SW0333291 could largely be achieved with a single prophylactic dose of the drug.

Conclusion
Inhibiting 15-PGDH may thus represent a novel strategy for prophylaxis of ischemic AKI in multiple clinical settings, including renal transplantation and cardiovascular surgery.