title: The long-term results of pediatric patients with primary focal and segmental glomerulosclerosis.
abstract: Focal and segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage renal disease (ESRD). In this retrospective study, we report on 34 pediatric patients with FSGS who were diagnosed and treated from 1992 to 2006. The mean age at onset was 6.3 + or - 4.3 years. All patients had nephrotic-range proteinuria. Microscopic hematuria was seen in three patients and hypertension was seen in 15 patients at presentation. All patients were treated with steroids (oral and/or methylprednisolone), while 23 patients received cytotoxic therapy in addition. The mean follow-up period was 8.6 + or - 3.3 years at the end of which, 59% of patients achieved complete or partial remission, 20.5% continued to have active renal disease while 20.5% of the patients developed CKD. Our study suggests that most of the patients with FSGS progress to renal insufficiency. Steroid therapy increases the chances of remission and preserves renal function in patients with sporadic primary FSGS.
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Ege University, Izmir, Turkey. betulsozeri@yahoo.com
authors.forename: Betul
authors.initials: B
authors.lastname: Sozeri
authors.forename: Sevgi
authors.initials: S
authors.lastname: Mir
authors.forename: Fatma
authors.initials: F
authors.lastname: Mutlubas
authors.forename: Sait
authors.initials: S
authors.lastname: Sen
mesh_terms: D000284:Administration, Oral; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications* / Q000188:drug therapy; D006417:Hematuria / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007676:Kidney Failure, Chronic / Q000188:drug therapy / Q000209:etiology*; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy / Q000209:etiology*; D011507:Proteinuria / Q000209:etiology; D012074:Remission Induction; D012189:Retrospective Studies; D013256:Steroids / Q000008:administration & dosage; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D013256:Steroids