bluuebunny/crossref_metadata_2025_split huggingface dataset:
#1422 Exploring BAFF, APRIL, and clinical characteristics in IgA nephropathy: a decade-long study in the Bulgarian population
Authors: Kostadinova Iva , Bogov Boris , Lyubomirova Mila , Kyurkchiev Dobroslav , Todorov Todor 
Published: May 2024

Abstract

Background and Aims
IgA nephropathy (IgAN) represents a significant global health concern, contributing substantially to chronic kidney disease and renal failure. Despite its widespread impact, the Bulgarian population lacks comprehensive epidemiological data and specific biomarkers for IgAN. This decade-long retrospective study, conducted at a tertiary Nephrology Clinic in Bulgaria, aims to explore the clinical characteristics and serum levels of BAFF and APRIL in biopsy-confirmed IgAN patients and healthy controls.

Method
The study includes 125 biopsy-confirmed IgAN patients and 30 healthy controls. Notably, 70.4% of IgAN patients were male, with an average age of 35.94 ± 11.91 years. Baseline characteristics reveal advanced disease stages at diagnosis, possibly linked to delayed biopsy (24.22 ± 32.9 months from initial symptoms) and the absence of a screening program. Analyses of serum biomarkers (C3, BAFF, and APRIL) are conducted to identify any significant variations between IgAN patients and healthy volunteers.

Results
The average baseline estimated glomerular filtration rate (eGFR) was 61.78 ± 27.72 ml/min/1.73 m², with a mean serum creatinine of 126 ± 67.83 μmol/l at the time of kidney biopsy. Among the patients, 51.2% had an eGFR below 60 ml/min, indicating CKD stage 3 or worse according to the KDIGO classification Binary logistic regression analysis identifies anemia as a significant contributor to disease progression, with a risk influence approximately 3.3 times higher for GFR decline over time. Anemia emerges as a crucial predictor for serum creatinine doubling, carrying a higher risk than hyperuricemia. Analyses of C3 (p = 0.658), BAFF (p = 0.432), and APRIL (p = 0.227), demonstrated no significant differences in their serum levels between IgAN patients and healthy volunteers.

Conclusion
This study provides comprehensive insights into IgAN in the Bulgarian population, addressing the dearth of epidemiological data and biomarkers. Anemia emerges as a significant predictor for disease progression, emphasizing its potential clinical relevance. However, C3, BAFF, and APRIL exhibit no notable variations, underscoring the need for additional biomarker exploration to enhance understanding of IgAN dynamics in this population.
#2284 Clinical characteristics and renal survival of patients with monoclonal gammopathy of renal significance (MGRS)
Authors: Tsiakas Stathis , Panagakis Anastasios , Skalioti Chrysanthi , Kapsia Eleni , Stergiou Ioanna E , Voulgarelis Michael , Boletis Ioannis , Marinaki Smaragdi 
Published: May 2024

Abstract

Background and Aims
Monoclonal gammopathy of renal significance (MGRS) comprises a diverse group of renal disorders related to a nephrotoxic monoclonal immunoglobulin. Due to the rarity of the disease and the recent definition of MGRS-associated kidney lesions, data about long-term clinical outcomes remain limited.

Method
A single-center, retrospective cohort study was conducted including data of all patients with biopsy-proven MGRS who were followed at our center between 2005 and 2023. We examined the clinicopathologic characteristics and renal survival of the patients. Disease progression was defined as an eGFR decline &gt;30% or end-stage kidney disease (ESKD). Univariate Cox regression analysis was used to assess the relationship between clinical features and disease progression.

Results
A total of 23 adults (males 43.5%) with a median age of 65 years (IQR 55-70) were included in our study. At presentation, median eGFR was 51 ml/min/1.73 m2 (IQR 40-80) and proteinuria 3.94 g/24 h (IQR 2.00-6.26). Serum or urine monoclonal protein was not detected in 4 patients (18.2%). MGRS-associated renal lesions included Ig-related amyloidosis (n = 8), monoclonal immunoglobulin deposition disease (n = 7), cryoglobulinemic glomerulonephritis (n = 2), C3 glomerulopathy (n = 2), proliferative glomerulonephritis with monoclonal IgG deposits (n = 1), immunotactoid glomerulopathy (n = 1) and miscellaneous (n = 2). Most patients were treated with a plasma cell-directed regimen (77.3%) and the rest with B-cell-directed (13.6%) or conservative therapy (9.1%). Median follow-up for the cohort was 45 months (IQR 27-67). Thirteen patients responded to treatment (52.1%), achieving a reduction in proteinuria (0.44 vs 3.94 g/24 h, p:001) and maintenance of eGFR (60 vs 56 ml/min, p:0.363) at the last follow-up visit. All responders had received clone-directed therapy. Disease progression occurred in 8 patients (34.8%), three of whom reached ESKD (13.0%). Renal survival, defined as survival without ESKD, was 84.6% at 48 months (CI 73.3-101.7). Factors that were associated with disease progression were Ig-related amyloidosis (HR: 6.94, CI: 1.30-36.84, p:0.023) and eGFR &lt; 45 ml/min at diagnosis (HR: 14.73, CI: 1.62-132.95, p:0.017).

Conclusion
MGRS is a heterogeneous clinical entity manifesting with varying degrees of proteinuria and kidney injury. Impaired kidney function at diagnosis and Ig-related amyloidosis correlated with worse renal prognosis in our cohort. Early identification and clone-directed treatment of MGRS may improve long-term renal survival.
#514 Efficacy of continues low dose rituximab in PLA2R-associated primary membranous nephropathy
Authors: Cui Zhuan , Wang Song , Wanf Yue , Zheng Danxia 
Published: May 2024

Abstract

Background and Aims
We reported that rituximab 100 mg monthly showed an effective regimen for low anti-phospholipase A2 receptor (anti-PLA2R) primary membranous nephropathy (PMN), especially for those elderly and susceptible PMN. Here we attempted to compare effect of the rituximab (RTX) 100 mg monthly vs traditional corticosteroid-cyclophosphamide (GC-Cy) for the treatment of PLA2R-associated PMN in our hospital.

Method
All the included PMN patients were treated in our hospital between June 2017 and June 2022 and were retrospectively analyzed. The inclusion criteria:(1) age &gt;18 years old, (2) biopsy-proven PMN, (3) anti-PLA2R titers &gt;20 RU/ml, (4) nephrotic syndrome (NS, both proteinuria &gt;3.5 g/24 and serum albumin &lt;30 g/L), (5) eGFR &gt;30 ml/ min. 1.73 m2, (6) followed up at least 12 months. Patients were stratified into the RTX and GC-Cy groups according to their received treatment. In the RTX group, patients received RTX 100 mg monthly until the NS was remission or at least serum anti-PLA2R less than 2 RU/ml. In the GC-Cy group, patients received prednisolone (0.5-1.0 mg/kg/day) or methylprednisolone (similar dose) orally for 1-2 months, then dosage was reduced gradually, and the total duration about one year; meanwhile received intravenous cyclophosphamide (0.6-1.0 g every month), total doses 6-12 g.

Results
The baseline parameters (included age, gender, anti-PLA2R antibody, urinary protein, serum albumin, and eGFR) did not differ significantly between the two groups (see Table 1). Total RTX dosed were 819 ± 240 (400-1200 mg) in the RTX group. After different treatment, anti-PLA2R antibody was significantly higher in rituximab group than in the GC-Cy group at 3 months (P &lt; 0.05), but were not significantly different between the two groups at 6, 9, and 12 months (P &gt; 0.05, see Fig. 1A); urinary proteins were significantly higher in RTX group than in the GC-Cy group at 3, 6, and 9 months (P &lt; 0.001), but were not significantly different between the two groups at 12 months (P &gt; 0.05, see Fig. 1B); serum albumin were significantly lower in RTX group than in the GC-Cy group at 3, 6, 9 and 12 months (P &lt; 0.01, see Fig. 1C). The total remission rate was not significantly different between the two groups at 12 months (P &gt; 0.05, see Fig. 1D).

Conclusion
Compared to traditional GC-Cy treatment, RTX 100 mg monthly appeared as a weak but lasting effective regimen for anti-PLA2R-associated PMN.
#2653 Long term renal outcome of patients with idiopathic membranous nephropathy – Croatian single center cohort
Authors: Brinar Ivana Vukovic , Prlic Margareta Fistrek , Ivandic Ema , Ćorić Marijana , Bulimbasic Stela , Kurtov Karlo , Kovačić Ines Bosnić , Kos Jelena , Dika Zivka , Jelakovic Ana , Jelaković Bojan 
Published: May 2024

Abstract

Background and Aims
Membranous nephropathy (MN) is the most prevalent cause of nephrotic syndrome in adult population. Our aim was to assess long term renal outcome of primary membranous nephropathy patients.

Method
In this retrospective study adult patients with biopsy proven membranous nephropathy from 2000 till 2015 with follow up of at least 5 years from UHC Zagreb were included. Extensive clinical workup was done to exclude patients with secondary MN. Except otherwise stated, data are shown as N (%) or median with interquartile range (IQR) and accompanied p levels.

Results
Between 2000 and 2015, 48 patients were diagnosed to have idiopathic/primary membranous nephropathy, out of which 33 (68%) MN patients had follow up of at least 5 years and where further analyzed. They were predominantly male 20 (60.6%) of average age of 52 years (min – max 19 - 76). High risk MN patient 25 (75%) were treated with immunosuppressive therapy, dominantly with cyclophosphamide and corticosteroids. During the median follow up of 10 (7 – 14) years, 19 (57%) accomplished complete remission and 11 (33%) partial remission. One (3%) patient did not achieve remission and 2 (6.1%) were in relapse of nephrotic syndrome at last follow up. Proteinuria significantly decreased (6.2 (3.3 – 10.9) vs. 0.2 (0.14 – 0,90); p &lt; 0.001) and there was no significant change in creatinine level (87 (42.8 – 101.5) vs. 93 (68.5 – 140.5; p = 0.332)). In follow up there were 5 (15%) carcinomas detected; one of each origin (colon, pancreas, lung, prostate, lymph nodes) approximately 7,8 years after the diagnose of MN. Limitation of this study could be selection bias but there was no difference in renal function at presentation of all MN patients and those who had at least 5 years follow up.

Conclusion
Renal long-term outcome of patients with idiopathic MN in our cohort was excellent. Nevertheless, there is high prevalence of cancer and therefore regular screening for cancer disease should take place in this population of patients. Also, drugs with cancerogenic potential should be avoided, as well as bear in mind cumulative dose of drugs applied.
#3051 Light chain only variant of proliferative glomerulonephritis with monoclonal immunoglobulin deposits: a case report
Authors: Martins Ana Cristina , Laranjinha Ivo 
Published: May 2024

Abstract

Background and Aims
Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is an entity characterized by glomerular deposition of monoclonal immunoglobulins, typically IgG. Recently, a cohort of 17 patients presenting with light chain only variant of PGNMID was published. Strikingly, in this cohort, glomerular deposition of C3 was detected in all cases. We herein report a case of PGNMID with a rare presentation on the kidney biopsy.

Case presentation
We present the case of a 73-year-old female with a past medical history of rheumatoid arthritis and hypertension, under methotrexate 10 mg per week, prednisone 2.5 mg per day, amlodipine, nebivolol, azilsartan, and a recurrent naproxen intake. She presented with fatigue, anorexia, weight loss of about 12% of her body weight, epistaxis, oral bleeding ulcers, and lower extremities oedema. Pancytopenia, acute kidney injury (urea 150 mg/dL, creatinine 2.92 mg/dL), and nephrotic syndrome (serum albumin of 2.7 mg/dL and proteinuria of 12 g/day) were noted. Further evaluation showed an imbalance of the free light chains’ ratio with kappa light chain's level of 1250 mg/dL. Serum protein electrophoresis and immunofixation were normal. A CT scan showed numerous cervical, mediastinal, and iliac enlarged lymph nodes.
Kidney biopsy showed an increase in mesangial cells and matrix and capillary walls’ thickening without nodular lesions under a light microscope. Immunofluorescence microscopy showed granular deposits of kappa light chains and no deposition IgA, IgG, IgM, C1q, C3, fibrin and lambda. Considering the previous literature, C3 was reanalyzed, with a consistent negative staining. Based on the above findings, a diagnosis of Light chain only variant of PGNMID was made. Concomitantly, evaluation of the bone marrow sample showed Small lymphocytic lymphoma. The patient was started on Bortezomib, cyclophosphamide and dexamethasone. Three months after proteinuria was 0.47 g/g and creatinine 1.7 mg/dL.

Conclusion
This case highlights the rare occurrence of a PGNMID with light chains only deposition (without C3 co-deposition) as a complication of small lymphocytic lymphoma. This contributes to the growing body of evidence surrounding this entity, with the particularity being that C3 was not detected. The authors hypothesized some pathophysiologic mechanisms to justify the absence of C3 co-deposition.
#1522 Comparative analysis of clinical and pathological traits in IgA nephropathy with nephrotic range proteinuria based on serum albumin level
Authors: Eum Sang Hun , Hong Suyeon , Hong Yu Ah , Ban Tae Hyun , Koh Eun Sil , Kim Yong Kyun , Chung Byung Ha , Shin Seok Joon , Yoon Hye Eun 
Published: May 2024

Abstract

Background and Aims
Presently, the clinical outcomes associated with IgA nephropathy exhibiting heavy proteinuria and the potential correlation between decreasing serum albumin levels and adverse prognosis have not been conclusively established. The present study was designed to elucidate the clinical and pathological characteristics of IgA nephropathy patients who present with nephrotic-range proteinuria, particularly in relation to serum albumin levels.

Method
This retrospective cohort study was conducted using data from biopsy-confirmed IgA nephropathy patients presenting with nephrotic-range proteinuria between 2017 and 2022 from eight university-affiliated hospitals in South Korea. Patients were stratified into two groups based on a serum albumin level of 3.0 g/dl. The study involved a comparative assessment of clinical and pathological characteristics across these two groups, as well as an evaluation of clinical prognosis with respect to the end-stage kidney disease (ESKD).

Results
The analysis included a total of 127 patients. Of these, 86 patients had a serum albumin level above 3.0 g/dl (designated as the preserved albumin group, or PA group), while 41 patients had levels below 3.0 g/dl (designated as the reduced albumin group, or RA group). The baseline characteristics of the two groups were found to be statistically similar. The mean age of the patients was 46.46 years, and 46.46% were male. In the laboratory findings, the RA group had significantly elevated erythrocyte sedimentation rate compared to the PA group. Additionally, total cholesterol and low-density lipoprotein-cholesterol levels were significantly higher in the RA group compared to the PA group. Proteinuria was observed to be significantly higher in the RA group. Pathological examinations demonstrated that in electron microscopy (EM) findings, the RA group exhibited a more pronounced diffuse foot process effacement. Over the course of one year, the trend in creatinine changes exhibited differences between the two groups over time (time x group interaction P = 0.002). Furthermore, it appeared that the reduction in the quantity of proteinuria was more substantial within the RA group (time x group interaction P = 0.001). During a median follow-up period of 25.22 months (ranging from 11.98 to 49.23 months), 14 patients (11.02%) in the PA group progressed to ESKD, compared with 10 patient (24.39 %) in the RA group. However, there was no statistically significant difference between the two groups in terms of progression to ESKD (P = 0.330).

Conclusion
In patients diagnosed with IgA nephropathy presenting with nephrotic-range proteinuria, a decline in serum albumin levels was associated with diffuse foot process effacement, and the decrease in serum albumin was not found to be associated with adverse prognosis.
#1834 Prevalence of nephrolithiasis in patients with idiopathic hypercalciuria after two decades of follow-up
Authors: Suarez German Perez , Nanwani Avinash Chandu , Herrera Aidana Maria Barrera , Yánez María Isabel Luis , Canton Cesar García , Garcia-Nieto Victor Manuel 
Published: May 2024

Abstract

Background and Aims
Idiopathic hypercalciuria (IH) is the main metabolic abnormality that causes kidney stones. It is linked to high intestinal calcium absorption and osteopenia. However, not all patients diagnosed with IH develop kidney stones. In this study, we examined a cohort of patients diagnosed of IH during childhood to determine the prevalence of lithiasis in adulthood.

Method
Longitudinal study that evaluated the prevalence of lithiasis in 35 patients (12 female, 23 male) diagnosed of IH during childhood (mean age of 7.7 ± 3 years). The patients were followed until adulthood (mean age 28.2 ± 3.1 years) with a median follow-up of 18.2 years (IQR: 12.2-19.3). IH diagnosis was made by urinary calcium excretion higher than 4 mg/Kg/day in two consecutive samples. In both periods, clinical and demographic data were collected, including urinalysis and urinary ratios (calcium/creatinine, calcium/citrate, oxalate/creatinine, and citrate/creatinine) in an isolated sample of the first morning urine, as well as the performance of renovesical ultrasound.

Results
In pediatric age, 8 patients (26%) presented nephrolithiasis on ultrasound, and 14 (43.8%) in adulthood, 6 of them experiencing episodes of nephritic colic. During the study, we observed a decrease in urinary calcium excretion, as expressed by the calcium/creatinine ratio (adult age 0.16 ± 0.9 mg/dl vs. childhood 0.28 ± 0.1 mg/dl, p = 0.000), and an hypocitraturia increase (citrate/creatinine ratio in childhood 887 ± 349 mg/dl and in adulthood 357.7 ± 363 mg/dl, p = 0.000). A decreased citrate/creatinine ratio in adulthood was associated with nephrolithiasis (p &lt; 0.007). Only 4 patients (50%) had nephrolithiasis at both ages.

Conclusion
These results suggest that hypercalciuria tends to correct spontaneously in adulthood, and although the prevalence of nephrolithiasis increased, it is more related to hypocitraturia.
#1246 The impact of severe nephrotic syndrome on thyroid function
Authors: Matyjek Anna , Rozanska Maria , Niemczyk Stanisław , Kronbichler Andreas 
Published: May 2024

Abstract

Background and Aims
Nephrotic syndrome (NS) is a clinical condition characterized by massive urinary loss of proteins, mostly albumin, but also hormones and their carrier proteins among others. The most frequently reported hormonal complication is thyroid dysfunction. The study aimed to assess the frequency of thyroid dysfunction in NS and its association with carrier protein levels and indicators of disease severity.

Method
A case-control study was conducted involving patients with newly diagnosed or relapsed severe NS, defined as proteinuria &gt; 3.5 g/day and serum albumin ≤ 2.5 g/dL (NS group), and patients without proteinuria (control group). The evaluation included thyroid hormone profiles, serum levels of carrier proteins (prealbumin – PAB, and thyroid-binding globulin – TBG), and markers reflecting the course of NS.

Results
Forty-two nephrotic and 40 matched non-proteinuric patients were enrolled. The NS group comprised 67% males with a mean age of 49 years and an average proteinuria of 8.9 g/day (Table 1). The leading cause of NS was minimal change disease (22 patients), followed by membranous nephropathy (11 patients). The other causes included: amyloidosis (3 patients: AL – 2 cases, A – 1 case), focal segmental glomerulosclerosis (2 patients) and lupus nephritis (1 case). In 5 cases, no histological diagnosis was made.
Thyroid dysfunction secondary to NS was observed in the majority of patients, occurring 18-times more frequently than in the control group (67% vs 10%, OR = 18, 95% CI: 5.3-60.7). Euthyroid sick syndrome was the most commonly seen in NS (15 cases, 36%), followed by overt (8 cases, 19%) and subclinical hypothyroidism (5 cases, 12%). According to the current guidelines, levothyroxine supplementation was required in 11 patients (26% of the NS group, 36% of those with thyroid dysfunction).
Serum TSH correlated inversely with PAB (R = −0.63, p &lt; 0.001), and positively with TBG (R = 0.39, p = 0.010), while no relationships were found between carrier proteins and free hormones (fT3 or fT4). The only indicator of NS severity related to thyroid function was serum albumin. It correlated, however, only with TSH (R = −0.41, p = 0.007), not with free hormones. Additionally, neither histological type, nor clinical course of NS distinguished between euthyroidism, euthyroid sick syndrome, or hypothyroidism (Table 2).

Conclusion
Thyroid dysfunction is a common complication of severe NS. Although euthyroid sick syndrome predominates, the proportion of patients with hypothyroidism who may potentially require levothyroxine replacement therapy remains substantial.
#822 Effect of renal Doppler ultrasound on the detection of nutcracker syndrome in children presenting orthostatic proteinuria
Authors: Lee Keum Hwa , Hwang Eun Jae , Kim Ji Hong , Park Se Jin , Shin Jae Il 
Published: May 2024

Abstract

Background and Aims
To assess the Doppler sonographic findings of the left renal vein (LRV) in children with orthostatic proteinuria compared to normal urine children.

Method
Renal Doppler ultrasound examinations were routinely performed on 50 consecutive children with orthostatic proteinuria and 53 normal urine children who have visited to the pediatric nephrology clinic at Severance hospital. The peak velocity (PV) was measured at the hilar portion of the left renal vein (LRV) and at the LRV between the aorta and the superior mesenteric artery.

Results
Age and sex did not differ between the two groups. The peak velocity ratios between aortomesenteric and hilar portions were 7.79+/−2.65 in children with orthostatic proteinuria and 6.32+/−3.01 in healthy normal children (p=0.01). The protein/creatinine ratio in the first morning urine did not differ between the two groups (0.09+/−0.03 in children with orthostatic proteinuria and 0.08+/−0.02 in healthy normal children (p = 0.144). However, the protein/creatinine ratio in the afternoon urine in children with orthostatic proteinuria (0.49+/−0.46) was significantly higher than that in healthy normal children (0.10+/−0.04) (p &lt; 0.001). The peak velocity ratios between aortomesenteric and hilar portions positively correlated with the protein/creatinine ratio between the afternoon urine and the first morning urine (R = 0.21, P = 0.034).

Conclusion
Nutcracker syndrome may be an important cause of orthostatic proteinuria in children. Renal Doppler ultrasound of the LRV may be a useful screening tool in patients with suspected orthostatic proteinuria to evaluate whether the cause is nutcracker syndrome.