Management and Outcome in Childhood Nephrotic Syndrome: A Retrospective Study

Background: The Nephrotic Syndrome (NS) is caused by renal diseases that increase the permeability across the glomerular filtration barrier. Despite its low incidence in general population, it is a common cause of chronic kidney disease in childhood. Although corticosteroid response is achieved in the majority of patients at the onset of disease, many patients are resistant or dependent to steroid treatment during follow-up.

Objective: Our aim was to characterize a population of hospitalized children diagnosed with NS according to demographic data, clinical data, management and outcomes.

Methods: A clinical retrospective analysis was performed between 2007 and 2018.

Results: A total of 22 children were enrolled, with a median age of 5.1 years at the onset of the disease. There was no gender predominance and no secondary causes were identified. We found that 68% (n=15) of patients presented with generalized edema and 18% (n=4) with palpebral edema. Median hospital length of stay was 8 days. All patients were initially treated with corticosteroids, and 82% (n=18) responded with proteinuria remission during hospital stay. Eighteen percent (n=4) were initially steroid-resistant without remission and subsequently transferred to a tertiary hospital. During follow-up, relapses were found in 68% (n=15). Steroid-dependent and steroid-resistant disease was found in 50% (n=11) and 14% (n=3) of patients, respectively. Forty-five percent (n=10) were subsequently treated with additional immunosuppression, mainly cyclophosphamide. Renal biopsies were performed in 32% (n=7) of patients.

Conclusions: In contrast to literature, in our cohort there was no gender predominance of NS. We emphasize the initial good corticosteroid response in the majority of our patients, but high incidence of relapses, steroid dependent and resistant disease during follow-up.