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A 12-year-old girl with a history of steroid & Cyclosporine resistant nephrotic syndrome owing to focal and segmental glomerulosclerosis (FSGS) has progressed to end-stage renal disease for which she underwent hemodialysis for 18 months before she successfully received at the age of 9 years a fully matched kidney transplant from her sister.
The post transplantation period was marked by an early and massive proteinuria indicating recurrent FSGS for which she received 12 sessions of plasma pheresis (PP); unfortunately, she did not appear to have any response to the PP therapy, thereafter a conservative management comprising essentially enalapril and losartan has been initiated and was also not successful during the first four months, however a very gradual response has been noticed to occur after five months of conservative therapy and ultimately, the patient attained complete remission after 21 months of treatment. Amazingly, 15 moths after discontinuation of enalapril and losartan, she remained in a complete and sustained remission with a good renal allograft function.
To the best of our knowledge, this is the first case ever reported in the literature of a “spontaneous” remission of post transplant recurrent FSGS.
FSGS, Recurrent. Remission. Plasma pheresis. Children