Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group

• Published in: Turkish journal of pediatrics Vol. 52; no. 3; pp. 255 - 261
• Main Authors: Beşbaş, Nesrin, Ozaltin, Fatih, Emre, Sevinç, Anarat, Ali, Alpay, Harika, Bakkaloğlu, Ayşin, Baskin, Esra, Buyan, Necla, Dönmez, Osman, Düşünsel, Ruhan, Ekim, Mesiha, Göko, Faysal, Gür-Güven, Ayfer, Kavukçu, Salih, Mir, Sevgi, Sönmez, Ferah
• Format: Journal Article
• Language: English
• Published: Turkey Hacettepe University Faculty of Medicine 01-05-2010
• Subjects: Adolescent; Child; Child, Preschool; Creatinine - blood; Disease Progression; Female; Glomerulosclerosis, Focal Segmental - blood; Glomerulosclerosis, Focal Segmental - complications; Glomerulosclerosis, Focal Segmental - drug therapy; Glucocorticoids - administration & dosage; Humans; Infant; Kidney Failure, Chronic - etiology; Male; Methylprednisolone - administration & dosage; Pulse Therapy, Drug; Retrospective Studies; Turkey; Turkey
• ISSN: 0041-4301

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.