Afroze Ramzan Sherali, Khemchand N Moorani, Saeed Hassan Chishty.
Response to cyclosporin in children with primary focal segmental glomerulosclerosis.
Pak Paed J Jan ;34(1):10-4.

Introduction: Focal segmental glomerulosclerosis (FSGS) is the most common biopsy finding in children with steroid dependent (SD) and steroid resistant nephrotic syndrome (SRNS) and its management is challenging. Cyclosporin (CS) has been used in steroid resistant FSGS with variable response. Objectives: To determine the response to CS in primary FSGS. Setting: The Kidney Center Postgraduate Training Institute and National Institute Child Health, Karachi Duration: From October 01, 2005 to August 30, 2008. Patients and Methods: Biopsy proven cases of 2-15 years age with either SDNS or SRNS were included. All received cyclophosphamide before CS therapy. Cyclosporin (5mg/kg/day) was given for 12 months along with low dose oral Prednisolone (0.5 mg/kg on alternate days). Response was assessed at 3 months. Complete remission was defined if urine for protein was nil or trace, partial remission if urine protein was > ++ without edema and CS resistant if proteinuria and edema persisted. Follow up with urinalysis, serum creatinine (S.Cr), CS trough level and for side effects was done for 2 years. Results: Among 30 patients, 12 were SDNS and 18 SRNS. Sixteen (53.33%) were boys and 14 girls (46.66%). Most common age group was 6-10 years (60%). BP and Serum Cr was within normal range for age. Complete remission was observed in 56.6% and 30% showed partial response. Thus overall response rate was 86.6 %. Four patients (13.33%) were resistant to CS. There was no rise in S.Cr (>25% basal) during CS therapy except in 3 (10%) from CS resistant group. Twelve (40%) relapsed after stopping CS and 2 were lost to followup. Gingival hyperplasia (46.66%) and hypertrichosis (36.66%) were the most common side effects. Conclusion: Cyclosporin in combination with low dose prednisolone was effective in more than 80% of patients with FSGS.

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