title: Differential risk of remission and ESRD in childhood FSGS.
abstract: Focal segmental glomerulosclerosis (FSGS) is the leading cause of steroid-resistant nephrotic syndrome in childhood and the most common form of end stage renal disease (ESRD) from glomerular disease. In order to assess the risk of progression of children with primary FSGS and the impact of proteinuria remission status on disease progression, we undertook this study to describe a cohort of 60 children and adolescents from the Glomerular Disease Collaborative Network. Of the 60 patients included in the cohort, 58% were African American. Median age was 16 years. Proteinuria ranged from 1.0-24.0 g/day/1.73 m(2); 57% were hypertensive, and the median estimated glomerular filtration rate (eGFR) was 90.2 ml/min/1.73 m(2). Complete remission was achieved in 20%, partial remission in 33%, and 47% have not achieved remission during follow-up with all prescribed therapy. Only ACE-I/ARB therapy was predictive of proteinuria remission in multivariate analysis (hazard ratio [HR] 3.35; 95% confidence interval [CI] 1.42-7.92). Renal survival was much improved in patients with complete or partial remission compared with no remission in univariate analysis. In multivariate analysis comparing no remission status, complete remission was associated with a 90% decreased risk of ESRD (HR 0.10, 95% CI 0.01-0.79, p =0.03). In summary, proteinuria remission status is a valid predictor of long-term renal survival in children with FSGS.
authors.affiliation: UNC Kidney Center, University of North Carolina, Chapel Hill, USA. Debbie_Gipson@med.unc.edu
authors.forename: Debbie S
authors.initials: DS
authors.lastname: Gipson
authors.forename: Hyunsook
authors.initials: H
authors.lastname: Chin
authors.forename: Trevor P
authors.initials: TP
authors.lastname: Presler
authors.forename: Caroline
authors.initials: C
authors.lastname: Jennette
authors.forename: Maria E
authors.initials: ME
authors.lastname: Ferris
authors.forename: Susan
authors.initials: S
authors.lastname: Massengill
authors.forename: Keisha
authors.initials: K
authors.lastname: Gibson
authors.forename: David B
authors.initials: DB
authors.lastname: Thomas
mesh_terms: D000293:Adolescent; D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D002648:Child; D015331:Cohort Studies; D018450:Disease Progression; D005919:Glomerular Filtration Rate; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications* / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D009404:Nephrotic Syndrome / Q000209:etiology; D011379:Prognosis; D011507:Proteinuria / Q000209:etiology; D012074:Remission Induction; D012307:Risk Factors
chemical_list: D057911:Angiotensin Receptor Antagonists; D000806:Angiotensin-Converting Enzyme Inhibitors