The Difficult Child with Difficult Nephrotic Syndrome

---

Sanjeev Gulati

Fortis Hospital, India

: J Nephrol Ren Dis

Abstract

---

Nephrotic syndrome is one of the commonest renal disorders ssn in children. We have also evaluated newer therapeutic alternatives in children with nephrotic syndrome. Majority of the children have relapses. In a randomized control trial we compared abrupt stoppage of prednisone with slow tapering over 2 months and found that the tapering protocol was associated with a much lower relapse rate. In another controlled trial, we found that intravenous cyclophosphamide is a useful and cost effective alternative, in maintaining remission in children, who are frequent relapser and steroid dependant as compared to oral cyclophosphamide and that too at a lesser cumulative dose with much lesser side-effects. The treatment of steroid resistant (SR) idiopathic focal segmental glomerulosclerosis (FSGS) continues to pose a therapeutic challenge. Although cyclosporine is one of the alternatives available for these children it is an expensive medication besides having the disadvantage of its inherent nephrotoxicity. We evaluated the efficacy of intravenous pulse cyclophosphamide therapy in children with steroid resistant FSGS. These children who are not only prone to develop chronic renal failure but also have a risk of recurrence of disease in the post transplant period. We observed that monthly intravenous pulse cyclophosphamide infusions are a safe, effective and economical therapeutic modality in steroid resistant children with idiopathic FSGS. In an another study, we evaluated the role of histopathology in the management of steroid resistant nephrotic in children. We found that children with SRNS secondary to MCD, are more likely to achieve remission as compared to non- MCD subtypes and have a better long-term prognosis. Hence kidney biopsy is of significant prognostic value in SRNS. We were amongst the first groups to evaluate the role of Tacrolimus in steroid resistant Nephrotic syndrome. Our results suggest that TAC is an effective therapeutic modality for SRNS, including the subgroup of children nonresponsive to the current therapeutic modalities like cyclophosphamide and cyclosporine.

Biography

---

I am currently working as Principal Director and Head , Nephrology at Fortis Hospitals, Vasant Kunj, New Delhi. I am the President , Indian Nephrology Society, ex Vice President of Indian Society of Organ Transplantation and Member , Governing Body of Indian Society of Nephrology. I also work at Kasturba Medical College as an Adjunct Porfessor in Nephrology. I was previously working as Additional Professor in Nephrology at Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow. I have also worked as Associate Professor in Nephrology at McMaster University, Hamilton, Canada.