GLORIA

GEOMAR Library Ocean Research Information Access

X

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Online Resource
    Online Resource
    Validation of a Histologic Scoring Index for C3 Glomerulopathy
    Elsevier BV ; 2021
    In:  American Journal of Kidney Diseases Vol. 77, No. 5 ( 2021-05), p. 684-695.e1
    Details
    In: American Journal of Kidney Diseases, Elsevier BV, Vol. 77, No. 5 ( 2021-05), p. 684-695.e1
    Type of Medium: Online Resource
    ISSN: 0272-6386
    URL: Article
    DOI: 10.1053/j.ajkd.2020.11.011
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2021
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
    Online Resource
  • 2
    Online Resource
    Online Resource
    Mycophenolate Mofetil in C3 Glomerulopathy and Pathogenic Drivers of the Disease
    Ovid Technologies (Wolters Kluwer Health) ; 2020
    In:  Clinical Journal of the American Society of Nephrology Vol. 15, No. 9 ( 2020-9), p. 1287-1298
    Details
    In: Clinical Journal of the American Society of Nephrology, Ovid Technologies (Wolters Kluwer Health), Vol. 15, No. 9 ( 2020-9), p. 1287-1298
    Abstract: C3 glomerulopathy is a complement-mediated disease arising from abnormalities in complement genes and/or antibodies against complement components. Previous studies showed that treatment with corticosteroids plus mycophenolate mofetil (MMF) was associated with improved outcomes, although the genetic profile of these patients was not systematically analyzed. This study aims to analyze the main determinants of disease progression and response to this therapeutic regimen. Design, setting, participants, & measurements We conducted a retrospective, multicenter, observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. Patients diagnosed with C3 glomerulopathy ( n =81) or dense deposit disease ( n =16) between January 1995 and March 2018 were enrolled. Multivariable and propensity score matching analyses were used to evaluate the association of clinical and genetic factors with response to treatment with corticosteroids and MMF as measured by proportion of patients with disease remission and kidney survival (status free of kidney failure). Results The study group comprised 97 patients (84% C3 glomerulopathy, 16% dense deposit disease). Forty-two patients were treated with corticosteroids plus MMF, and this treatment was associated with a higher rate of remission and lower probability of kidney failure (79% and 14%, respectively) compared with patients treated with other immunosuppressives (24% and 59%, respectively), or ecluzimab (33% and 67%, respectively), or conservative management (18% and 65%, respectively). The therapeutic superiority of corticosteroids plus MMF was observed both in patients with complement abnormalities and with autoantibodies. However, patients with pathogenic variants in complement genes only achieved partial remission, whereas complete remissions were common among patients with autoantibody-mediated forms. The main determinant of no remission was baseline proteinuria. Relapses occurred after treatment discontinuation in 33% of the patients who had achieved remission with corticosteroids plus MMF, and a longer treatment length of MMF was associated with a lower risk of relapse. Conclusions The beneficial response to corticosteroids plus MMF treatment in C3 glomerulopathy appears independent of the pathogenic drivers analyzed in this study.
    Type of Medium: Online Resource
    ISSN: 1555-9041 , 1555-905X
    URL: Article
    DOI: 10.2215/CJN.15241219
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2020
    detail.hit.zdb_id: 2216582-4
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
    Online Resource
  • 3
    Online Resource
    Online Resource
    Development and validation of a nomogram to predict kidney survival at baseline in patients with C3 glomerulopathy
    Oxford University Press (OUP) ; 2022
    In:  Clinical Kidney Journal Vol. 15, No. 9 ( 2022-08-22), p. 1737-1746
    Details
    In: Clinical Kidney Journal, Oxford University Press (OUP), Vol. 15, No. 9 ( 2022-08-22), p. 1737-1746
    Abstract: C3 glomerulopathy is a rare and heterogeneous complement-driven disease. It is often challenging to accurately predict in clinical practice the individual kidney prognosis at baseline. We herein sought to develop and validate a prognostic nomogram to predict long-term kidney survival. Methods We conducted a retrospective, multicenter observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. The dataset was randomly divided into a training group (n = 87) and a validation group (n = 28). The least absolute shrinkage and selection operator (LASSO) regression was used to screen the main predictors of kidney outcome and to build the nomogram. The accuracy of the nomogram was assessed by discrimination and risk calibration in the training and validation sets. Results The study group comprised 115 patients, of whom 46 (40%) reached kidney failure in a median follow-up of 49 months (range 24–112). No significant differences were observed in baseline estimated glomerular filtration rate (eGFR), proteinuria or total chronicity score of kidney biopsies, between patients in the training versus those in the validation set. The selected variables by LASSO were eGFR, proteinuria and total chronicity score. Based on a Cox model, a nomogram was developed for the prediction of kidney survival at 1, 2, 5 and 10 years from diagnosis. The C-index of the nomogram was 0.860 (95% confidence interval 0.834–0.887) and calibration plots showed optimal agreement between predicted and observed outcomes. Conclusions We constructed and validated a practical nomogram with good discrimination and calibration to predict the risk of kidney failure in C3 glomerulopathy patients at 1, 2, 5 and 10 years.
    Type of Medium: Online Resource
    ISSN: 2048-8505 , 2048-8513
    URL: Article
    DOI: 10.1093/ckj/sfac108
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 2656786-6
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
    Online Resource
  • 4
    Online Resource
    Online Resource
    Longitudinal change in proteinuria and kidney outcomes in C3 glomerulopathy
    Oxford University Press (OUP) ; 2022
    In:  Nephrology Dialysis Transplantation Vol. 37, No. 7 ( 2022-06-23), p. 1270-1280
    Details
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 37, No. 7 ( 2022-06-23), p. 1270-1280
    Abstract: The association between a change in proteinuria over time and its impact on kidney prognosis has not been analysed in complement component 3 (C3) glomerulopathy. This study aims to investigate the association between the longitudinal change in proteinuria and the risk of kidney failure. Methods This was a retrospective, multicentre observational cohort study in 35 nephrology departments belonging to the Spanish Group for the Study of Glomerular Diseases. Patients diagnosed with C3 glomerulopathy between 1995 and 2020 were enrolled. A joint modelling of linear mixed-effects models was applied to assess the underlying trajectory of a repeatedly measured proteinuria, and a Cox model to evaluate the association of this trajectory with the risk of kidney failure. Results The study group consisted of 85 patients, 70 C3 glomerulonephritis and 15 dense deposit disease, with a median age of 26 years (range 13–41). During a median follow-up of 42 months, 25 patients reached kidney failure. The longitudinal change in proteinuria showed a strong association with the risk of this outcome, with a doubling of proteinuria levels resulting in a 2.5-fold increase of the risk. A second model showed that a ≥50% proteinuria reduction over time was significantly associated with a lower risk of kidney failure (hazard ratio 0.79; 95% confidence interval 0.56–0.97; P  & lt; 0.001). This association was also found when the ≥50% proteinuria reduction was observed within the first 6 and 12 months of follow-up. Conclusions The longitudinal change in proteinuria is strongly associated with the risk of kidney failure. The change in proteinuria over time can provide clinicians a dynamic prediction of kidney outcomes.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    URL: Article
    DOI: 10.1093/ndt/gfab075
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2022
    detail.hit.zdb_id: 1465709-0
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
    Online Resource
  • 5
    Online Resource
    Online Resource
    Clinical Profiles and Patterns of Kidney Disease Progression in C3 Glomerulopathy
    Ovid Technologies (Wolters Kluwer Health) ; 2023
    In:  Kidney360 Vol. 4, No. 5 ( 2023-5), p. 659-672
    Details
    In: Kidney360, Ovid Technologies (Wolters Kluwer Health), Vol. 4, No. 5 ( 2023-5), p. 659-672
    Abstract: Kidney survival in C3 glomerulopathy is significantly higher in patients with a disease chronicity score 〈 4 and proteinuria 〈 3.5 g/d, regardless of baseline eGFR. A faster eGFR decline in C3 glomerulopathy is associated with higher probability of kidney failure. Patients with glomerulopathy with a progressive reduction in proteinuria over time did not reach kidney failure. Background C3 glomerulopathy is a rare kidney disease, which makes it difficult to collect large cohorts of patients to better understand its variability. The aims of this study were to describe the clinical profiles and patterns of progression of kidney disease. Methods This was a retrospective, observational cohort study. Patients diagnosed with C3 glomerulopathy between 1995 and 2020 were enrolled. Study population was divided into clinical profiles by combining the following predictors: eGFR under/above 30 ml/min per 1.73 m 2 , proteinuria under/above 3.5 g/d, and histologic chronicity score under/above 4. The change in eGFR and proteinuria over time was evaluated in a subgroup with consecutive measurements of eGFR and proteinuria. Results One hundred and fifteen patients with a median age of 30 years (interquartile range 19–50) were included. Patients were divided into eight clinical profiles. Kidney survival was significantly higher in patients with a chronicity score 〈 4 and proteinuria 〈 3.5 g/d, both in those presenting with an eGFR under/above 30 ml/min per 1.73 m 2 . The median eGFR slope of patients who reached kidney failure was −6.5 ml/min per 1.73 m 2 per year (interquartile range −1.6 to −17). Patients who showed a reduction in proteinuria over time did not reach kidney failure. On the basis of the rate of eGFR decline, patients were classified as faster eGFR decline (≥5 ml/min per 1.73 m 2 per year), slower ( 〈 5 ml/min per 1.73 m 2 per year), and those without decline. A faster eGFR decline was associated with higher probability of kidney failure. Conclusions Kidney survival is significantly higher in patients with a chronicity score 〈 4 and proteinuria 〈 3.5 g/d regardless of baseline eGFR, and a faster rate of decline in eGFR is associated with higher probability of kidney failure.
    Type of Medium: Online Resource
    ISSN: 2641-7650
    URL: Article
    DOI: 10.34067/KID.0000000000000115
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2023
    Location Call Number Limitation Availability
    BibTip Others were also interested in ...
    Online Resource
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...