title: Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group.
abstract: The clinical course of focal segmental glomerulosclerosis (FSGS) is heterogeneous in children. To evaluate the clinical course and the predictors of outcome in Turkish children with primary FSGS, a retrospective study was conducted by the Turkish Pediatric Nephrology Study Group in 14 pediatric nephrology centers. Two hundred twenty-two patients (92 boys, 130 girls, aged 1-16 years) with biopsy-proven primary FSGS were included. One hundred forty-eight patients were followed-up for a median of 51 months (range: 0.26-270). The clinical course was characterized by complete remission in 50 (33.8%), persistent proteinuria in 50 (33.8%) and progression to renal failure in 48 (32.4%) patients. Progression to end-stage renal disease (ESRD) was significantly higher in patients who did not attain remission. Complete remission, partial remission and progress to renal failure were recorded in 37%, 32% and 28%, respectively, of the patients (n = 73) treated with prednisone combined cyclophosphamide/cyclosporine A. However, in patients (n = 33) treated with pulse methyl prednisolone plus oral prednisone (up to 20 months) combined with cyclophosphamide, complete remission in 51.5% and partial remission in 27.3% of the patients were noted. Progression to renal failure was observed in 9.1% of this group of patients. Multivariate analysis showed that only plasma creatinine at presentation was an independent predictive value for outcome. Patients with serum creatinine level higher than 1.5 mg/dl had 6.6 times increased rate of progression to renal failure. Failure to achieve remission is a predictor of renal failure in children with primary FSGS. The use of immunosuppressive treatment in conjunction with prolonged steroid seems beneficial in primary FSGS in children.
authors: Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
authors: Nesrin
authors: N
authors: Beşbaş
authors: Fatih
authors: F
authors: Ozaltin
authors: Sevinç
authors: S
authors: Emre
authors: Ali
authors: A
authors: Anarat
authors: Harika
authors: H
authors: Alpay
authors: Ayşin
authors: A
authors: Bakkaloğlu
authors: Esra
authors: E
authors: Baskin
authors: Necla
authors: N
authors: Buyan
authors: Osman
authors: O
authors: Dönmez
authors: Ruhan
authors: R
authors: Düşünsel
authors: Mesiha
authors: M
authors: Ekim
authors: Faysal
authors: F
authors: Göko
authors: Ayfer
authors: A
authors: Gür-Güven
authors: Salih
authors: S
authors: Kavukçu
authors: Sevgi
authors: S
authors: Mir
authors: Ferah
authors: F
authors: Sönmez
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003404:Creatinine / Q000097:blood; D018450:Disease Progression; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000097:blood / Q000150:complications* / Q000188:drug therapy; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D020551:Pulse Therapy, Drug; D012189:Retrospective Studies; D014421:Turkey
chemical_list: D005938:Glucocorticoids; D003404:Creatinine; D008775:Methylprednisolone