ESPN 51th Annual Meeting

ESPN 2018


 
CLINICAL MANIFESTATIONS AND SERUM GROWTH FACTORS IN CHILDREN WITH FSGS
INA KAZYRA 1 HANNA KRyLOVA-ALEFIRENKO 1 Tatiana Letkovskaya 1 Alexander Sukalo 1

1- BELARUSIAN STATE MEDICAL UNIVERSITY
 
Introduction:

Focal-segmental glomerulosclerosis (FSGS) is generally characterized by poor renal survival. However the clini­cal course and prognosis of FSGS is heteroge­neous in children with a number of clinical, laboratory and pathological  features have been shown to be a predictor of disease outcome.

We conducted our study to characterize clinical presentations of FSGS in children and to analyze potential impact of transforming growth factor 1ß (TGF1ß) and vascular-endothelial growth factor (VEGF) on clinical course of FSGS in pediatric patients.

Material and methods:

52 children with morphologically approved FSGS were enrolled. Clinical data, including blood and urinalyses, blood pressure, eGFR (Schwartz method) and actual treatment were analyzed introspectively and blood samples were obtained.  Histological data, including percentage of sclerotic glomeruli, severity of interstitial fibrosis and mesangial proliferation were analyzed retrospectively. Serum levels of TGF1ß and VEGF were determined in 52 and children suffering from primary (47) or secondary (5) FSGS and 36 healthy controls, with no statistically significant differences in age and gender between study and control groups. 

The age of patients in study group varied from 1 to 17 years (10,9±4,87, Me 11,5), males and  females 28 and 24 patients, accordingly.


Results:

Clinical presentations included nephrotic syndrome (NS) in 33/52 patients. Among this patients 11 were steroid sensitive (SSNS), but steroid dependent or had frequent relapses. 8 NS patients were steroid resistant (SRNS) and 14 most severe patients presented with SRNS with hematuria and hypertension. Also clinical presentations included non-nephrotic range proteinuria  in 15 patients, of which in 10 it was accompanied by hematuria. 4 patients by the moment of enrolling in the study have progressed to chronic kidney disease stage 3 or more, two of this patients required kidney replacement therapy.

In study group 75% of cases presented with arterial hypertension. Almost all (20/22) steroid resistant nephrotic patients were hypertensive, while half (5/11) of  steroid sensitive patients were normotensive.

Serum level of TGF1ß was significantly higher in study group comparing with healthy controls (p<0,001), while the level of VEGF didnt differ significantly in this groups. Among FSGS patients the VEGF level was significantly higher in steroid sensitive NS cases comparing with steroid resistant NS (p=0,028) and non-nephrotic range proteinuric patients (p=0,017). TGF1ß levels didnt differ between this groups of patients.

The following analysis of implication TGF1ß and VEGF serum levels in clinical or morphological presentation failed to reveal any statistically significant differences. 

Conclusions:

Children with FSGS are demonstrated to have significantly higher level of serum TGF1ß, but not VEGF, comparing with healthy subjects. Children with FSGS presenting with SSNS are shown to have  higher levels of VEGF than patients with SRNS or non-nephrotic range proteinuria, which might be considered  when choosing a treatment regimen.