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      Apheresis in Adult With Refractory Idiopathic Nephrotic Syndrome on Native Kidneys

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          Abstract

          Background

          Apheresis is the gold standard for idiopathic nephrotic syndrome (INS) relapse after transplantation, but it remains unknown whether such treatment is useful for adults with refractory INS on native kidneys.

          Methods

          This retrospective study included patients older than 16 years with biopsy-proven refractory (persistent nephrotic syndrome on corticosteroids plus at least 1 immunosuppressive drug) INS treated by apheresis and followed for at least 3 months.

          Results

          Between September 1997 and January 2020, 21 patients (focal segmental glomerulosclerosis: 12, minimal change nephrotic syndrome: 9, men: 67%, median age: 34 years) were identified. At last follow-up (12 months), 7 of 21 patients were in complete or partial remission. Remission was associated with older age (51 vs. 30 years, P = 0.05), lower proteinuria (3.9 vs. 7.3 g/d, P = 0.03), and lower estimated glomerular filtration rate (eGFR) (28.0 vs. 48.5 ml/min per 1.73 m 2, P = 0.05) at apheresis. The need for dialysis before apheresis (odds ratio [OR] 22.0 [1.00–524], P = 0.026), age ≥50 years (OR: 22.6 [1.00–524], P = 0.006), a marked (>4.5 g/d) decrease in proteinuria (OR: 9.17 [1.15–73.2], P = 0.041), and a short (<12 months) time between diagnosis and apheresis (OR: 10.8 [1–117], P = 0.043) were significantly associated with remission. Three of 7 patients in remission who were initially on dialysis became dialysis-free; by contrast, none of the 14 patients without remission was initially on dialysis, but 5 of 14 had become dialysis-dependent ( P = 0.01).

          Conclusion

          Apheresis may result in remission in adult patients with refractory INS, particularly in those at risk of renal failure, with limited sensitivity to medical treatments, if apheresis is initiated within a year of diagnosis.

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          Most cited references44

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          Equations to estimate glomerular filtration rate (GFR) are routinely used to assess kidney function. Current equations have limited precision and systematically underestimate measured GFR at higher values. To develop a new estimating equation for GFR: the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. Cross-sectional analysis with separate pooled data sets for equation development and validation and a representative sample of the U.S. population for prevalence estimates. Research studies and clinical populations ("studies") with measured GFR and NHANES (National Health and Nutrition Examination Survey), 1999 to 2006. 8254 participants in 10 studies (equation development data set) and 3896 participants in 16 studies (validation data set). Prevalence estimates were based on 16,032 participants in NHANES. GFR, measured as the clearance of exogenous filtration markers (iothalamate in the development data set; iothalamate and other markers in the validation data set), and linear regression to estimate the logarithm of measured GFR from standardized creatinine levels, sex, race, and age. In the validation data set, the CKD-EPI equation performed better than the Modification of Diet in Renal Disease Study equation, especially at higher GFR (P < 0.001 for all subsequent comparisons), with less bias (median difference between measured and estimated GFR, 2.5 vs. 5.5 mL/min per 1.73 m(2)), improved precision (interquartile range [IQR] of the differences, 16.6 vs. 18.3 mL/min per 1.73 m(2)), and greater accuracy (percentage of estimated GFR within 30% of measured GFR, 84.1% vs. 80.6%). In NHANES, the median estimated GFR was 94.5 mL/min per 1.73 m(2) (IQR, 79.7 to 108.1) vs. 85.0 (IQR, 72.9 to 98.5) mL/min per 1.73 m(2), and the prevalence of chronic kidney disease was 11.5% (95% CI, 10.6% to 12.4%) versus 13.1% (CI, 12.1% to 14.0%). The sample contained a limited number of elderly people and racial and ethnic minorities with measured GFR. The CKD-EPI creatinine equation is more accurate than the Modification of Diet in Renal Disease Study equation and could replace it for routine clinical use. National Institute of Diabetes and Digestive and Kidney Diseases.
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              Focal Segmental Glomerulosclerosis.

              Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease worldwide. The presumed etiology of primary FSGS is a plasma factor with responsiveness to immunosuppressive therapy and a risk of recurrence after kidney transplant-important disease characteristics. In contrast, adaptive FSGS is associated with excessive nephron workload due to increased body size, reduced nephron capacity, or single glomerular hyperfiltration associated with certain diseases. Additional etiologies are now recognized as drivers of FSGS: high-penetrance genetic FSGS due to mutations in one of nearly 40 genes, virus-associated FSGS, and medication-associated FSGS. Emerging data support the identification of a sixth category: APOL1 risk allele-associated FSGS in individuals with sub-Saharan ancestry. The classification of a particular patient with FSGS relies on integration of findings from clinical history, laboratory testing, kidney biopsy, and in some patients, genetic testing. The kidney biopsy can be helpful, with clues provided by features on light microscopy (e.g, glomerular size, histologic variant of FSGS, microcystic tubular changes, and tubular hypertrophy), immunofluorescence (e.g, to rule out other primary glomerulopathies), and electron microscopy (e.g., extent of podocyte foot process effacement, podocyte microvillous transformation, and tubuloreticular inclusions). A complete assessment of renal histology is important for establishing the parenchymal setting of segmental glomerulosclerosis, distinguishing FSGS associated with one of many other glomerular diseases from the clinical-pathologic syndrome of FSGS. Genetic testing is beneficial in particular clinical settings. Identifying the etiology of FSGS guides selection of therapy and provides prognostic insight. Much progress has been made in our understanding of FSGS, but important outstanding issues remain, including the identity of the plasma factor believed to be responsible for primary FSGS, the value of routine implementation of genetic testing, and the identification of more effective and less toxic therapeutic interventions for FSGS.
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                Author and article information

                Contributors
                Journal
                Kidney Int Rep
                Kidney Int Rep
                Kidney International Reports
                Elsevier
                2468-0249
                06 May 2021
                August 2021
                06 May 2021
                : 6
                : 8
                : 2134-2143
                Affiliations
                [1 ]Service de Néphrologie-Hypertension, Dialyses, Transplantation Rénale, CHRU Tours, Tours, France
                [2 ]Service de Néphrologie, CHR Orléans, Orléans, France
                [3 ]Service de Néphrologie, Assistance Publique des Hôpitaux de Paris (AP-HP), Hôpital Necker, Paris, France
                [4 ]Service de Néphrologie-Dialyse-Transplantation, CHU d’Angers, Angers, France
                [5 ]Service de Néphrologie, Hémodialyse, Aphérèses et Transplantation Rénale, CHU Grenoble-Alpes, Grenoble, France
                [6 ]CHU Saint-Etienne, Hôpital Nord Avenue Albert Raimond, Saint Priest en Jarez, France
                [7 ]Aix-Marseille University, C2VN, INSERM, INRAE, AP-HM, Centre de Néphrologie et Transplantation Rénale, Marseille, France
                [8 ]Service de Néphrologie-Dialyse-Transplantation Rénale, CHU Limoges, Limoges, France
                [9 ]Service de Néphrologie-Dialyse et Transplantation, CHU Clermont-Ferrand, Clermont-Ferrand, France
                [10 ]Service de Néphrologie, CHU Rouen, Rouen, France
                [11 ]Service Néphrologie Hémodialyse, CH de la côte Basque, Bayonne, France
                [12 ]Service de Néphrologie, Transplantation et Dialyse, CHU Bordeaux, Bordeaux, France
                [13 ]Service de Néphrologie, AP-HP, Hôpital Tenon, Paris, France
                [14 ]Service de Néphrologie, Centre Hospitalier de La Rochelle, La Rochelle, France
                [15 ]Service de Néphrologie, Hôpital François Mitterrand, Dijon, France
                [16 ]Service de Néphrologie et Hémodialyse, Centre Hospitalier de Laval, Laval, France
                [17 ]Service de Néphrologie-Dialyses-Aphérèse, Hôpital Caremeau, CHU Nîmes, Nîmes, France
                [18 ]EA4245, Université François-Rabelais, Tours, France
                [19 ]INI-CRCT, Vandœuvre-lès-Nancy, France
                [20 ]Service de Néphrologie et Transplantation, Centre de Référence Maladie Rare « Syndrome Néphrotique Idiopathique », Fédération Hospitalo-Universitaire « Innovative Therapy for Immune Disorders », AP-HP, Hôpitaux Universitaires Henri Mondor, Créteil, France
                [21 ]University of Paris Est Créteil, Institut National de la Santé et de la Recherche Médicale (INSERM) U955, Institut Mondor de Recherche Biomédicale (IMRB), Equipe 21, Créteil, France
                Author notes
                [] Correspondence: Jean-Michel Halimi, Service Néphrologie-Hypertension, Dialyses, Transplantation Rénale, CHRU Tours, Hôpital Bretonneau, 2 Boulevard Tonnellé, 37000 Tours, France. jmhalimi@ 123456univ-tours.fr
                [22]

                JMH and VA contributed equally to this work and are joint senior authors.

                Article
                S2468-0249(21)01142-6
                10.1016/j.ekir.2021.04.029
                8343786
                34386662
                ea2146e1-8cfd-4f8e-8e7c-caf0c0119619
                © 2021 Published by Elsevier, Inc., on behalf of the International Society of Nephrology.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 2 April 2021
                : 22 April 2021
                : 26 April 2021
                Categories
                Clinical Research

                apheresis,focal segmental glomerulosclerosis,minimal change nephrotic syndrome,nephrotic syndrome

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