Primary focal segmental glomerular sclerosis in children: Epidemiology and prognosis

Focal segmental glomerulosclerosis (FSGS) is the morphologic description of a glomerular lesion which is "focal", meaning a few but not all of the total sampled glomeruli have and "segmental" solidification of the tuft that is an accumulation of extracellular matrix with obliteration of the capillary lumina (sclerosis). It represents 20% of nephrotic syndrome in children and adults. To study the role of epidemiology, clinical presentation, histology, and treatment in the prognosis of HSF child, we retrospectively analyzed 23 children with primary focal segmental glomerulosclerosis (FSGS) hospitalized in pediatric nephrology unit of Children's Hospital Harrouchi Abderrahim, CHU Ibn Rochd Casablanca from January 2000 to December 2012. The main age at onset was 7.5 years with a male predominance. Hematuria was seen in 22% of patients, hypertension in 48% of patients, and moderate renal insufficiency in one patient at presentation. According to the histological classification of Columbia, 40% of patients have a non-specific HSF (NOS), including six patients who have responded to treatment and one patient progressed to renal failure, 13% have a perihilar HSF (PH) with a good prognosis, 8% have a HSF cell (CELL), which evolved to renal failure, 35% of HSF was a tubular pole (TIP) including five patients responded to treatment and 4% was a HSF collapsing (COL) having a renal failure at admission. The FSGS's prognosis is related to several predictive factors. (C) 2013 Association Societe de nephrologie. Published by Elsevier Masson SAS. All rights reserved.