ESPN 50th Annual Meeting

ESPN 2017


 
GIRLS HAVE HIGHER RISK FOR PRIMARY STEROID RESISTANT NEPHROTIC SYNDORME - Nephrosis Online STUDY
MAGDALENA DROżYńSKA-DUKLAS 1 BEATA BIENIAś 2 PRZEMYSłAW SIKORA 2 EWA GACKA 3 LIDIA HYLA-KLEKOT 3 ANDRZEJ BRODKIEWICZ 4 TOMASZ JARMOLIńSKI 5 AGNIESZKA RYBI-SZUMIńSKA 6 ANNA WASILEWSKA 6 WIOLETTA JARMUżEK 7 GRENDA RYSZARD 7 ALEKSANDRA ŻUROWSKA 1

1- DEPARTMENT OF PAEDIATRICS, NEPHROLOGY AND HYPERTENSION, MEDICAL UNIVERSITY OF GDAńSK, POLAND
2- DEPARTMENT OF PEDIATRIC NEPHROLOGY, MEDICAL UNIVERSITY OF LUBLIN, POLAND
3- DEPARTMENT OF PEDIATRIC NEPHROLOGY, CENTER FOR PEDIATRICS AND ONCOLOGY, CHORZóW, POLAND
4- DEPARTMENT OF PEDIATRICS, NEPHROLOGY WITH DIALIZOTHERAPY AND MANAGEMENT OF ACUTE POISONING, POMERANIAN MEDICAL UNIVERSITY, SZCZECIN, POLAND
5- DEPARTMENT OF PAEDIATRICS, MIęDZYRZECZ REGIONAL HOSPITAL, POLAND
6- DEPARTMENT OF PEDIATRICS AND NEPHROLOGY, MEDICAL UNIVERSITY OF BIALYSTOK, POLAND
7- DEPARTMENT OF NEPHROLOGY, KIDNEY TRANSPLANTATION AND HYPERTENSION, CHILDRENS MEMORIAL HEALTH INSTITUTE, WARSAW, POLAND
 
Introduction:

The annual incidence of idiopathic nephrotic syndrome (INS) in Europe ranges from 2 to 7 per 100000 children. Male predominance among young children and rare onset after 10yrs age has been historically reported. Almost 80% of children achieve remission following steroid treatment. The aim of the presented study was to assess  the contemporary demographics and initial response to steroids in a Caucasian cohort of INS.

Material and methods:

Data of 500 Caucasian children with 1st episode of NS was collected prospectively from 17 Pediatric Nephrology centers through a web based platform – Nephrosis Online (NOL). Standard steroid therapy was administered to 479 children with INS and steroid-responsiveness was assessed according to KDIGO Guidelines.

Results:

Mean age at onset was 5,14 yrs +/- 3,7 yrs, median age: 3,87yrs. Nearly equal distribution between genders was noted (M:269,F:210 = 1,28:1). 429/479 children (89,5%) responded to steroids (SSNS). 50/479 (10,4%) were steroidresistant (SRNS) after 8wks of therapy. Mean age at onset of SSNS was 4.8 ±3,4 yrs, with majority (91%) younger than 10 yrs. Only a slight predominance of boys was observed (M:250 vs. F:179= 1,4:1). Among SSNS children girls were slightly older (mean age for M: 4,6 vs. F: 5,2 yrs, p= 0,039).Mean age at onset of SRNS was significantly higher (6,9± 4,8 yrs), median age : 6,15 yrs. 70.% were younger than 10 yrs. Predominance of girls was present in the SRNS subgroup (M:19, F:31=1:1,63). Age at onset of SRNS was independent of sex (F: 7,1yrs vs. M:7,2yrs)

Conclusions:

In contrast to previous publications an almost equal distribution of both sexes is observed in a cohort of Caucasian children with INS. 

In this cohort female sex is associated with an increased risk of initial steroid resistance.