ESPN 50th Annual Meeting

ESPN 2017


 
Steroid Dependent and Frequently Relapsing Nephrotic Syndrome is Associated with Persistent Disease Into Adulthood
TRINE KORSGAARD 1 RENE FRYDENSBJERG ANDERSEN 1 SHIVANI JOSHI 1 SøREN HAGSTRøM 2 SøREN RITTIG 1

1- PAEDIATRIC AND ADOLESCENT MEDICINE, DEPARTMENT OF CLINICAL MEDICINE, AARHUS UNIVERSITY HOSPITAL, 8200 AARHUS N, DENMARK.
2- DEPARTMENT OF PEDIATRICS, AALBORG UNIVERSITY HOSPITAL, 9000 AALBORG, DENMARK.
 
Introduction:

We conducted a retrospective clinical database on children with idiopathic nephrotic syndrome (INS) to evaluate the long-term clinical course and outcome.

Material and methods:

Patients with new-onset INS admitted to the paediatric departments in the central and northern region of Denmark between January 1998 and December 2015 were included in the study. Patients were identified by a search on ICD-10 codes in the Danish National Patient Register. All patients (n = 128) identified from the search were asked to provide with written informed consent, and data were obtained from the medical charts. Active disease above 18 years was defined as relapse within 12 months or ongoing immunosuppressive treatment. 

Results:

The annual incidence of INS in this cohort was 1.9/100,000 children aged 0-14 years. Mean age at debut was 6.9 years (range 1.6-14.5 years), and the male:female ratio was 1.2:1. Mean follow-up was 9 years. A total of 84% (92/110) patients were classified as steroid sensitive (SSNS), of whom 60% (55/92) were either steroid dependent and/or frequently relapsing (SD/FR). Of the 31 patients with SSNS who were above 18 years at last follow-up, 29% were still suffering from active disease. In the group of SD/FR patients, 27% (15/55) had reached the age of 18 years at last date of follow-up, of whom 53% (8/15) still had active disease. 

Conclusions:

The study presents clinical and long-term outcome data on an unselected Danish population of children from a well-defined geographic area. We found that more than half of patients with SD/FR continue to have active disease into adulthood suggesting that the long-term prognosis of childhood SSNS is much less favourable in this subtype.