The Turkish Journal of Pediatrics
2010 , Vol 52 , Num 3
Clinical Course of Primary Focal Segmental Glomerulosclerosis (FSGS) in Turkish Children:A Report from the Turkish Pediatric Nephrology FSGS Study Group
Departments of Pediatric Nephrology, Faculty of Medicine, 1Hacettepe University, Ankara, 2İstanbul University, Çapa,
İstanbul, 3Çukurova University, Adana, 4Marmara University, İstanbul, 5Başkent University, Ankara, 6Gazi University,
Ankara, 7 Uludağ University, Bursa, 8Erciyes University, Kayseri, 9Ankara University, Ankara, 10Gülhane Military
Medical Academy, Ankara, 11Akdeniz University, Antalya, 12Dokuz Eylül University, İnciraltı, İzmir, 13Ege University,
İzmir, and 14Adnan Menderes University, Aydın, Turkey
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.
Keywords :
children, focal segmental glomerulosclerosis, treatment outcome.