POS-460 FOCAL SEGMENTAL GLOMERULOSCLEROSIS: ABOUT A SERIES OF 18 CHILDREN.

Abstract

Focal segmental glomerulosclerosis (FSGS) is a glomerular histopathological finding characterized by segmental (affecting part of the flocculus) and focal (affecting only certain glomeruli) sclerosis. It is severe kidney disease with a complex pathophysiology. To determine the epidemiology and outcome of FSGS in our patients, we conducted a retrospective study on 18 cases diagnosed between January 2010 and December 2019. Out of 345 renal biopsies performed in that period of time, the prevalence of FSGS was of 5.2%. The mean age of these FSGS patients was 9 years old. Boys were predominant with a sex ratio of 2. Consanguinity was found in one-third of our cases. Nephrotic syndrome was the main revealing pattern and was always steroid resistant (100%). Meanwhile, hypertension was found in 30% of our children, and hematuria in 47% of them. Two cases had kidney failure at diagnosis. Primitive FSGS was found in 39% of the cases, four of them reached complete remission after stating calcineurin inhibitors (CIN), while the remaining cases were resistant to CIN, and one case developed chronic kidney disease after only seven months of acute onset. As for adaptative FSGS, it was more common in our children, with 61% of cases. The outcome was marked by increased evolution towards chronic kidney disease and poor response to immunosuppressive therapies. Finally, two cases of FSGS were seen after the prolonged use of CNI inhibitors for the treatment of nephrosis. One of these patients ultimately evolved into chronic kidney disease. Management of FSGS is still challenging, especially in children. More studies, especially in the field of genetics, are needed to improve the outcome of this dreadful glomerulopathy.