Article

The long-term results of pediatric patients with primary focal and segmental glomerulosclerosis.

Department of Pediatrics, Faculty of Medicine, Ege University, Izmir, Turkey.
Saudi journal of kidney diseases and transplantation: an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia 01/2010; 21(1):87-92.
Source: PubMed

ABSTRACT Focal and segmental glomerulosclerosis (FSGS) is a major cause of idiopathic steroid-resistant nephrotic syndrome (SRNS) and end-stage renal disease (ESRD). In this retrospective study, we report on 34 pediatric patients with FSGS who were diagnosed and treated from 1992 to 2006. The mean age at onset was 6.3 + or - 4.3 years. All patients had nephrotic-range proteinuria. Microscopic hematuria was seen in three patients and hypertension was seen in 15 patients at presentation. All patients were treated with steroids (oral and/or methylprednisolone), while 23 patients received cytotoxic therapy in addition. The mean follow-up period was 8.6 + or - 3.3 years at the end of which, 59% of patients achieved complete or partial remission, 20.5% continued to have active renal disease while 20.5% of the patients developed CKD. Our study suggests that most of the patients with FSGS progress to renal insufficiency. Steroid therapy increases the chances of remission and preserves renal function in patients with sporadic primary FSGS.

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    ABSTRACT: 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.
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