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Challenges in primary focal segmental glomerulosclerosis diagnosis : from the diagnostic algorithm to novel biomarkers
- Source :
- Scientia, Dipòsit Digital de Documents de la UAB, Universitat Autònoma de Barcelona, Clinical Kidney Journal
- Publication Year :
- 2020
-
Abstract
- Biomarcadors; Algoritme de diagnosi; Glomerulosclerosi segmentària focal Biomarcadores; Algoritmo de diagnóstico; Glomeruloesclerosis segmentaria focal Biomarkers; Diagnosis algorithm; Focal segmental glomerulosclerosis Primary or idiopathic focal segmental glomerulosclerosis (FSGS) is a kidney entity that involves the podocytes, leading to heavy proteinuria and in many cases progresses to end-stage renal disease. Idiopathic FSGS has a bad prognosis, as it involves young individuals who, in a considerably high proportion (∼15%), are resistant to corticosteroids and other immunosuppressive treatments as well. Moreover, the disease recurs in 30–50% of patients after kidney transplantation, leading to graft function impairment. It is suspected that this relapsing disease is caused by a circulating factor(s) that would permeabilize the glomerular filtration barrier. However, the exact pathologic mechanism is an unsettled issue. Besides its poor outcome, a major concern of primary FSGS is the complexity to confirm the diagnosis, as it can be confused with other variants or secondary forms of FSGS and also with other glomerular diseases, such as minimal change disease. New efforts to optimize the diagnostic approach are arising to improve knowledge in well-defined primary FSGS cohorts of patients. Follow-up of properly classified primary FSGS patients will allow risk stratification for predicting the response to different treatments. In this review we will focus on the diagnostic algorithm used in idiopathic FSGS both in native kidneys and in disease recurrence after kidney transplantation. We will emphasize those potential confusing factors as well as their detection and prevention. In addition, we will also provide an overview of ongoing studies that recruit large cohorts of glomerulopathy patients (Nephrotic Syndrome Study Network and Cure Glomerulonephropathy, among others) and the experimental studies performed to find novel reliable biomarkers to detect primary FSGS. The authors are current recipients of research grants from the Fondo de Investigación Sanitaria-Feder, Instituto de Salud Carlos III (PI17/00257, PI18/01704 and PI18/01832) and REDinREN (RD16/0009/0030).
- Subjects :
- diagnosis algorithm
030232 urology & nephrology
Focal segmental glomerulosclerosis
Disease
030204 cardiovascular system & hematology
Otros calificadores::Otros calificadores::/efectos adversos [Otros calificadores]
urologic and male genital diseases
Surgical Procedures, Operative::Surgical Procedures, Operative::Urogenital Surgical Procedures::Urologic Surgical Procedures::Kidney Transplantation [ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT]
03 medical and health sciences
0302 clinical medicine
Idiopathic nephrotic syndrome
Glomerulopathy
Ronyons - Malalties - Diagnòstic
Other subheadings::Other subheadings::/adverse effects [Other subheadings]
medicine
Minimal change disease
AcademicSubjects/MED00340
Otros calificadores::/terapia [Otros calificadores]
Kidney transplantation
CKJ Reviews
focal segmental glomerulosclerosis
Transplantation
Kidney
business.industry
Primary Focal Segmental Glomerulosclerosis
urogenital system
Male Urogenital Diseases::Urologic Diseases::Kidney Diseases [DISEASES]
Primary FSGS
biomarkers
Other subheadings::/therapy [Other subheadings]
medicine.disease
female genital diseases and pregnancy complications
medicine.anatomical_structure
intervenciones quirúrgicas::trasplante::trasplante de órganos::intervenciones quirúrgicas::trasplante de riñón [TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS]
Nephrology
idiopathic nephrotic syndrome
primary FSGS
business
enfermedades urogenitales masculinas::enfermedades urológicas::enfermedades renales [ENFERMEDADES]
Nephrotic syndrome
Algorithm
Diagnosis algorithm
Ronyons - Trasplantació - Complicacions
Ronyons - Malalties - Tractament
Biomarkers
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Journal :
- Scientia, Dipòsit Digital de Documents de la UAB, Universitat Autònoma de Barcelona, Clinical Kidney Journal
- Accession number :
- edsair.doi.dedup.....9cce6c7da9d2681686baf2a761ef3db9