bluuebunny/crossref_metadata_2025_split huggingface dataset:
P0439THE RELATIONSHIP BETWEEN SOCIO-ECONOMIC STATUS AND RENAL VASCULITIS IN SCOTLAND
Authors: Aiyegbusi Oshorenua , Frleta-Gilchrist Marina , MacKinnon Malcolm , Traynor Jamie , Stevens Kate , Geddes Colin C , McQuarrie Emily 
Published: June 2020

Abstract

Background and Aims
Individuals living in areas of multiple socioeconomic deprivation have reduced life expectancy and experience health inequalities. Chronic kidney disease is more common in areas of social deprivation and these patients are more likely to develop end stage kidney disease. Vasculitis is a rare but significant cause of kidney disease. The impact of socioeconomic status on disease activity or outcomes in patients with ANCA Associated Vasculitis (AAV) is yet to be fully explored. The aim of this study was to establish whether there is an association between the incidence of biopsy-proven renal vasculitis and socioeconomic status, as measured by the Scottish Index of Multiple Deprivation (SIMD).

Method
Using the Scottish renal biopsy registry, we identified all adult native renal biopsies performed across Scotland between 2014 and 2018 with a diagnosis of AAV. Patient’s postcode and SIMD (2016) rank were recorded. Patients were separated into quintiles of SIMD rank. Baseline demographics were recorded. We derived the denominator population from the 2016 SIMD census. Data were calculated per million population (PMP) served.

Results
339 biopsy proven cases of AAV were identified. 6 cases were excluded as postcode was unavailable. Overall, mean age was 65.9 (±13.0) years and 45% of patients were male. At time of diagnosis, mean estimated glomerular filtration rate (eGFR) was 61.7 (±25.7) ml/min/1.73m2 and median urinary protein creatinine ratio (uPCR) was 134mg/mmol (IQR 64-21). Microscopic Polyangiits n=205(65%) was more common than Granulomatosis with Polyangiits n=128(35%). The incidences of kidney biopsy proven AAV were similar across all quintiles of deprivation. In the most deprived 20% of population, incidence rate of kidney biopsy proven AAV was 11.2 per million person-years vs 13.0 per million person-years in least deprived 20% of population. Patients in areas of greatest relative deprivation were younger (64.0 (±12.3) vs. 68.1 (±12.7) years) and had slightly less proteinuria at diagnosis (99mg/mmol (IQR 35-211) vs. 138mg/mmol (IQR 78-281)) when compared to patients living in least deprived areas. However, there was no difference in level of renal function at diagnosis (33.8 (±29.6) ml/min/1.73m2 vs 31.5 (±23.2) ml/min/1.73m2).

Conclusion
Our complete national dataset shows that there is no significant difference in incidence of renal AAV across the spectrum of socioeconomic deprivation. The analysis of renal function at presentation suggests no evidence of an association between deprivation and delay in diagnosis in a healthcare system free at the point of access.
P0442RELATED FACTORS IN THE DEVELOPMENT OF LUPUS NEPHRITIS IN PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS
Authors: Ribas Andres , Galcerán Isabel , Outón Sara , Salman Tarek , Barrios Clara , S. Ochoa Adriana , Buxeda Anna , Sans Laia , Pascual Julio , Rodriguez Eva 
Published: June 2020

Abstract

Background and Aims
Lupus Nephritis (LN) is a severe complication of Systemic Lupus Erytemathosus (SLE). This is the main reason why identifying predisposing factors to differentiate patients at risk of developing LN is so important.

Method
Retrospective study of patients with SLE diagnosed between years 2008-2018 in our center. Demographic, clinical and analytical data have been collected.

Results
We included 171 patients, 48 (28%) with diagnose of LN. Age at diagnose of SLE was 39,51 ± 15,40 years, being more frequent in women 151 (87,5%). Time of follow-up since SLE diagnose until development of LN was of 3 ± 5, 3 years. Respectful to the LN classification we found: 4 (8%) class I LN, 6 (12.5%) class II LN, 15 (31.2%) class III LN, 19 (39.5%) class IV LN and 4 (8%) class V LN. At diagnose of SLE, the following variables, where related to developing LN: CH50 [HR: 1,039; CI (95%): 1,004-1,064; p=0,024], C3 [HR: 1,029; CI (95%): 1,016-1,042; p&lt;0,001, titer of Anti- DNACrithidia [HR: 4,364; CI(95%): 1,26-15,064; p=0,02], AntiSM [HR: 4,634, CI (95%) 1,76-12,17, p=0,002], ACA IgG [HR: 7,5; CI (95%): 2,3 -24,449; p=0,001] and Lupus anticoagulant [HR: 4,97; CI (95%): 1,591-15,533; p=0,006]. Treatment with hidroxicloroquine is a protective factor against developing LN [HR: 0,17; CI (95%): 0,063-0,511; p=0.001]. At diagnose of LN, complement factors and titer of anti-DNA crithidia show a positive correlation when compared to the initial determinations: C3 [r= 0,605 (p&lt;0,001]); C1q [r= 0,861 (p=0,006)]; CH50 [r= 0,981 (p&lt;0,001), anti- DNACrithidia [r= 0,529 (p&lt;0,001)], anti-Sm [r=0.8, )p=0.001)].

Conclusion
Consumption of complement factors, high titers of anti-DNAcrithidia, Anti-SM, ACA IgG and Lupus anticoagulant are related to a future LN development at SLE diagnose. Moreover, we see an increase of their titer once we diagnose LN. Otherwise, treatment with hidroxicloroquine seems to be a protective factor.
P0449SERUM PROPROTEIN CONVERTASE SUBTILISIN/KEXIN TYPE 9 (PCSK9) IN NON-DIABETIC ASIANS WITH NEPHROTIC SYNDROME
Authors: Lim Cynthia , Mok Irene , Tan Hui Zhuan , Teng Esther W L , Chue Xiu Ping , Ang Wei Mian , Chin Yok Mooi , Foo Marjorie , Choo Jason 
Published: June 2020

Abstract

Background and Aims
Nephrotic syndrome is associated with hypercholesterolemia, while its treatment, often involving high-dose glucocorticosteroids, may cause hyperglycemia. Proprotein convertase subtilisin /kexin type 9 (PCSK9) regulates plasma cholesterol and is associated with post-transplant diabetes. Although a potential therapeutic target in nephrotic syndrome, the relationships between PCSK9 and lipid parameters are not well established. We aimed to characterize serum PCSK9 and metabolic parameters among individuals with nephrotic syndrome.

Method
Single-center prospective cohort study of non-diabetic adults newly-diagnosed with nephrotic syndrome and subsequently treated with immunosuppressant. Fasting serum PCSK9 was measured using ELISA at diagnosis. Clinical history, blood pressure, body mass index (BMI), waist-to-hip ratio (WHR) and biochemistry (fasting glucose and lipid, HbA1c and fasting serum PCSK9) were obtained at diagnosis and at 3 months during immunosuppression therapy. Spearman’s correlation was used to evaluate for associations between PCSK9 and glycemic and lipid parameters.

Results
Among 15 adults with nephrotic syndrome, the majority was female (60%) and Chinese (80%). Median age was 40 (IQR 26, 51) years. The most frequent diagnoses were minimal change disease or focal segmental nephrosclerosis (n=7) and lupus nephritis (n=5); the minority had IgA nephropathy, membranous nephropathy or vasculitis. At diagnosis, median eGFR was 97.9 (59.5, 120.9) ml/min/1.73 m2, UPCR 7.6 (6.1, 10.4) g/g, LDL-cholesterol 4.6 (3.1, 6.7) mmol/L and fasting serum PCSK9 317.6 (276.8, 470.1) ng/ml. PCSK9 correlated positively with hypertriglyceridemia (r=0.55, p=0.03) at baseline.
Most patients (n=14, 93%) were treated with prednisolone (monotherapy or in combination with other immunosuppressant) and 1 received tacrolimus monotherapy. At 3 months, the majority (n= 11, 73%) had achieved clinical remission.
Fasting serum PCSK9 was higher at 3 months [median 430.6 (283.1, 496.0) ng/ml] compared to baseline, although the difference was not statistically significant. At 3 months, PCSK9 correlated positively with age (r=0.58, p=0.03), LDL-cholesterol (r=0.90, p=0.04) and change in BMI (r=0.72, p=0.01).

Conclusion
PCSK9 is associated with metabolic parameters at diagnosis and during the treatment course in patients with nephrotic syndrome treated with immunosuppressant.
P0452GLYCOSURIA IN MEMBRANOUS NEPHROPATHY: AN UNDERESTIMATED MARKER?
Authors: Ormonde Carolina , Laranjinha Ivo , Gaspar Augusta , Gonçalves Margarida , Gil Célia , Dickson Jorge , Machado Domingos 
Published: June 2020

Abstract

Background and Aims
Membranous nephropathy (MN) is one of the most common causes of nephrotic syndrome in adults. Some studies have suggested that glycosuria is present in the most severe cases of MN and this finding may be associated with tubular injury caused by the overload of filtered proteins. The aim of this study was to evaluate the association between glycosuria in MN and severity and prognosis of the disease.

Method
We conducted a 36-month retrospective cohort study with all patients diagnosed in the last 10 years in our centre with primary MN confirmed by renal biopsy. Data collection was made 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 MN.

Results
We studied 27 patients with primary MN. Four patients (14.8%) presented glycosuria at the time of diagnosis. Glycosuric and non-glycosuric groups had comparable demographic data. We found that glycosuric patients had higher baseline albuminuria (10.7±8.6 vs 4.6±4.1 g/g, p=0.029) and lower estimated glomerular filtration rate (eGFR) (39.2±24.6 vs 75.2±30.7 ml/min/1.73m2, p=0.037). We also found that patients with glycosuria had higher interstitial fibrosis and tubular atrophy (IFTA) (33.8%±21.4 vs 10.7%±17.1, p=0.035) but no differences in the percentage of glomerular sclerosis. There were no differences between groups in serum albumin, haemoglobin or therapy. At the end of the follow-up period there was no difference in eGFR decline rate (ml/min/year), 3-year eGFR, 3-year albuminuria and 3-year chronic kidney disease stage 5D incidence.

Conclusion
Prevalence of glycosuria in primary MN is approximately 15%. Its presence at the time of diagnosis was associated with more severe clinical and histological markers: lower eGFR, higher albuminuria and higher IFTA. However, we did not find association between glycosuric status at diagnosis and disease progression. Prospective, bigger and longer studies are needed to evaluate these questions.
P0457MALE LUPUS NEPHRITES: WHAT ABOUT RISK FACTORS FOR RENAL FAILURE ? EXPERIENCE OF ONE DEPARTMENT
Authors: Melek Kharroubi , Trabelsi Raja , Ounissi Mondher , Chargui Soumaya , Jerbi Mouna , Gaid Hanen , Hedri Hafedh , Harzallah Amel , Ben abdallah Taib , Goucha Rim 
Published: June 2020

Abstract

Background and Aims
The occurrence of renal involvement during the clinical course of systemic lupus erythematous (SLE) is generally considered to be the most important factor influencing the prognosis in terms of morbidity and mortality. The factors influencing prognosis in lupus nephritis (LN) are variable in literature. The aim of our study was to analyze the clinicopathological correlations, and risk factors associated with renal failure in male patients with LN.

Method
We retrospectively studied all male patients with kidney biopsy-proven lupus nephritis (LN) treated in our department during the period between 1979 and 2016. Looking for predictive factors related to renal prognosis, we analyzed clinical, biological and histological data by multivariate analysis using the comparison of the survival rates by the log-rank test.

Results
We collected 41 native renal biopsies showing LN. Patients were aged 32.17 years (17-65 years) at the time of diagnosis of LN. Diagnosis of SLE was made according to the criteria of the American College of Rheumatology. The most common clinical presentation was nephrotic syndrome (61%), and the most frequent pathological finding in sediment was proteinuria (85%). At the time of diagnosis, 17% of patients had hypertension and 39 % of patients had an eGFR under than 60 ml/min/1.73 m2. LN was of class I, II, III, IV and V in 7.5%, 12.5%, 27.5%, 47.5% and 41.5% of the cases, respectively. Fourteen patients developed end-stage renal failure. The presence of hematuria, renal failure at the time of diagnosis, nephrotic syndrome, low complement, proliferative glomerulonephritis (class IV), high activity index score of LN and thrombotic microangiopathy were significantly associated with poor renal prognosis with (p=0.0053), (p=0.0002), (p=0.0186), (p=0.0287), (p=0.0005), (p=0.058), (p=0.0117), respectively.

Conclusion
Renal failure at the time of diagnosis and active proliferative lesions should be diagnosed and treated as soon as possible because they influence the renal prognosis in lupus man.
P0461BASELINE SERUM C3 LEVELS IN PATIENTS WITH ANCA ASSOCIATED VASCULITIS AND ITS CLINICAL IMPLICATION
Authors: Maldonado Maria , Santacruz Juan , Afonso Sara , Olea Teresa , León Lina , Santos Carlos , Vega Cristina , Rivas Begoña , Selgas Rafael 
Published: June 2020

Abstract

Background and Aims
The evolution of renal disease and its global impairment in patients diagnosed with ANCA-associated vasculitis (AAV) has been related to baseline C3 serum levels in several studies.
The objective of this study is to evaluate in patients diagnosed with AAV and renal impairment the association between baseline serum C3 levels and the clinical outcomes.

Method
Our cohort included 56 patients diagnosed with AAV diagnosed from January 1986 to January 2018 in Hospital La Paz. According to C3 serum level measurements, patients were divided into low and normal concentration groups at onset. Histological, clinical and laboratory data were compared between groups.

Results
Among the 56 AAV cases, 58.9% were women and 96.5% Caucasian with an age mean and SD of 61.08 ± 15.11 years. Mean follow-up was 10.03 ± 7.02 years. MPO ANCAs were found in 69.8% of the patients and PR3 ANCAs in 22.64%. AAV diagnosis was earlier in PR3 AVV (47.59 ± 13.62 years) compared to MPO AVV (65.66 ± 11.53 years) (p=0.001). At diagnosis, baseline serum C3 was measured in 53 patients (94.6%) being lower in MPO AAV (112.79 ± 33.98) vs PR3 AAV (132.41 ± 20.69) (p=0.023). Low C3 was way more frequent in patients with higher percentage of glomeruli showing &gt;50% of sclerosis (100% vs 32.5%) (p=0.007). Staphylococcus aureus nasal carriers were more frequent in patients with low C3 (27.2% vs 3.1%, p=0.045). 15 patients (27.77%) died during follow-up and patient survival median was 21.75 years (IC 95%: 15.41-28.08). 17 patients (31.48%) reached end-stage renal disease (ESRD) during follow-up and renal survival median was 20.1 years (IC 95%: 19.27-21.05).
There were no statistical differences in the levels of baseline serum C3 according to sex, relapses, extrarenal manifestations, treatment, malignancy, cardiovascular events and time to ESRD or death.

Conclusion
Baseline serum C3 was lower in MPO AV, in Staphylococcus aureus nasal carriers and higher severity histological damage (glomerular sclerosis &gt;50%). Despite previous evidence, in our cohort, time to ESRD and time to death seemed not to be affected.
P0466LUPUS NEPHRITIS IN SOUTH MOROCCO OVER 6 YEARS
Authors: BENHAMOU Ramia , Sara Allibou , Yahioui Mariem , Chettati Meriem , Fadili Wafaa , Essaadouni Lamia , Laouad Inass 
Published: June 2020

Abstract

Background and Aims
Lupus nephritis is one of the most serious manifestation of systemic lupus. The study aimed at assessing the epidemiology and clinical features of systemic lupus erythematosus with lupus nephritis (LN) in south Morocco.

Method
A retrospective study was conducted in the Marrakesh-Tensift region by charts review from 2014 to 2019 (included). Patients with a SLE diagnosed with lupus nephritis over 6 years according to ACR/EULAR criteria were included.

Results
In total, 146 patients met inclusion criteria: 133 were women (94%) and mean age at diagnosis was 31,195 ± 13.5 years. Five percent of all patients had a least one parent with autoimmune disease, and 4,1% with lupus. The main manifestations were musculoskeletal (89%) and mucocutaneous with acute or subacute lesions (68,5%), alopecia (50%), ulcers (3,4%) and discoid lupus (10,27%). Cardiac manifestations in 19,2% and neurological features in 17,9% and psychiatric features in 13,7%. At the moment of diagnosis antinuclear antibodies were positive in 89% (threshold &gt;1/80), and associated to anti-DNA in 91 patients (62,3%), anti-SSA (21,2%), and anti-Sm (34,9%). APL syndrome was diagnosed in 8,2%. Kidney biopsy showed predominantly class IV in 54% and classe I-II in 28%. The average annual standardized incidence of LN between 2014 and 2019 was 2,4 cases per 1,000,000 inhabitants (95% confidence interval [CI]: 5.0–6.0). The prevalence was 21 cases per 1,000,000 inhabitants in 2018 (95% CI: 70–79).

Conclusion
Lupus nephritis in the multi-ethnic population of south moroccan is characterized by high incidence and high rates of diffuse proliferative nephritis.
P0469VALIDATING THE DIAGNOSTIC ACCURACY OF MEMBRANOUS NEPHROPATHY IN THE HEALTH IMPROVEMENT NETWORK (THIN) DATABASE
Authors: Wheatley Roseanna , Hamilton Patrick , Blaikie Kieran , Rao Anirudh , Kanigicherla Durga , Brenchley Paul 
Published: June 2020

Abstract

Background and Aims
Membranous nephropathy (MN) is among the most common causes of nephrotic syndrome in adults worldwide. Despite this, there is currently no robust data on the epidemiology of MN in the UK population. The Health Improvement Network (THIN) is an electronic medical record database that holds longitudinal anonymised patient records for over 17 million patients and has shown to be generalisable to the UK regarding demographics and crude prevalence’s of major conditions. To our knowledge, accuracy of the read codes for glomerular disease is yet to be validated. This will be the first study into MN validating the diagnostic accuracy using the THIN database.

Method
THIN database was interrogated for patients with MN using read codes. Two cohorts were considered: Definite cohort, defined as read codes expected to correspond to a diagnosis of MN, and Probable cohort, defined as read codes that could correspond to a diagnosis of MN. In order to confirm the diagnosis of MN, a short questionnaire was sent to the GP practice of a randomly selected cohort of patients asking if the diagnosis of MN was correct, and that the diagnosis had been confirmed by a specialist renal centre, with or without a renal biopsy.

Results
267 patients with a record of MN were identified from the THIN database. 235 of the patients had Definite cohort read codes, with a mean age at diagnosis of 57 years. There were 155 (66.2%) male and 79 (33.8%) female patients. 32 patients were identified in the Probable cohort. GP questionnaires were sent to 71 randomly selected patients with 61 responses (85.9% response rate). This represented 23% (n=53) of the total Definite cohort and 25% (n=8) of the total Probable cohort. Of the 61 returned questionnaires, an MN diagnosis was confirmed in 96% (n=51) of patients with a definite read code and 25% (n=2) with a probable read code. Amongst the confirmed MN diagnoses in the Definite cohort, 88% (n=45) of the patients had primary MN.

Conclusion
The THIN database is a valid data resource for studying MN in patients with a read code from the Definite cohort list. Read codes from the Probable cohort list cannot be used unless confirmed on a case by case basis such as through the GP. The results of this study will feed into a larger project with an aim to describe accurately the epidemiology of MN in the UK population, and report the incidence and prevalence of specific secondary associations of MN. Once these factors are fully understood, diagnostic and care pathways for MN can be developed.
P0473LONGITUDINAL CHANGES OF IGA1 O-GLYCOFORM IN IGA NEPHROPATHY
Authors: OHYAMA YUKAKO , Yamaguchi Hisateru , Nakajima Kazuki , Inaguma Daijo , Hasegawa Midori , Tsuboi Naotake , Renfrow Matthew B , Novak Jan , Yuzawa Yukio , Takahashi Kazuo 
Published: June 2020

Abstract

Background and Aims
Elevation of circulatory IgA1 with galactose-deficient (Gd) hinge-region (HR) O-glycans (Gd-IgA1) has been detected in most IgA nephropathy (IgAN) patients based on lectin ELISA. However, new approaches are needed for molecular-level characterization of IgA1 HR glycoform(s) in IgAN. We established a high-throughput method for analysis of IgA1 HR O-glycoforms using liquid chromatography-high-resolution mass spectrometry (LC-HRMS). To identify IgAN-associated IgA1 HR O-glycoforms and to assess their changes after therapy (with or without corticosteroids (CS)), we profiled IgA1 HR glycopeptides from sera collected at two time points (before and after therapy) from Japanese IgAN patients.

Method
Of the 10 Japanese IgAN patients recruited, 4 received CS treatment (CS group) and 6 have not (non-CS group). Japanese healthy volunteers (HC, n=10) were recruited as controls. Serum IgA1 was purified by affinity chromatography from HC and IgAN patients before and after therapy. After neuraminidase treatment and trypsin digestion, IgA1 HR glycosylation heterogeneity was analyzed by LC-HRMS. The relative abundance (RA, %) for each glycopeptide was calculated as percentage to the total IgA1 HR glycopeptide. The amount of each glycopeptide was then calculated by multiplying serum IgA concentration (mg/dL) by RA.

Results
Approximately 60% of IgA1 HR O-glycoforms in IgAN patients and HC were Gd O-glycoforms; these glycoforms contained one to three Gd-glycan(s), designated as 1 Gd-glycoform, 2 Gd-glycoform and 3 Gd-glycoform, respectively. In IgAN patients, the RA of non Gd-IgA1 glycoforms was elevated (P=0.002) and correlated with proteinuria (g/gCr) at renal biopsy (P=0.039, R=0.657). The amounts of non Gd- and 1Gd-glycoforms were higher in IgAN patients compared to HC (each P&lt;0.001). After several years of follow up (2.77 years (1.44-3.85)), the RA of non Gd-glycoforms decreased in CS group of IgAN patients (P=0.039) whereas it remained unchanged in the non-CS group (P=0.488). The amount of non Gd-glycoforms exhibited similar trends, i.e., decreased in CS group (P=0.068) whereas it remained unchanged in the non-CS group (P=0.943).

Conclusion
This study profiled serum IgA1 for IgAN-associated IgA1 HR O-glycoforms at the molecular level and assessed their changes in response to CS vs. non-CS therapy. IgA1 HR O-glycoforms altered by treatment may serve as a biomarker(s) for monitoring patients’ responses to therapy.