Trine Korsgaard1, René Frydensbjerg Andersen1, Shivani Joshi2, Søren Hagstrøm3,4, Søren Rittig5. 1. Department of Pediatric and Adolescent Medicine, Aarhus University Hospital, Skejby, Palle Juul-Jensens Boulevard 99, DK-8200, Aarhus N, Denmark. 2. Department of Clinical Medicine, Child and Youth Research Laboratory, Aarhus University, Palle Juul-Jensens Boulevard 99, DK-8200, Aarhus N, Denmark. 3. Department of Pediatrics, Aalborg University Hospital, Reberbansgade 15, DK-9000, Aalborg, Denmark. 4. Center for Clinical Research, North Denmark Regional Hospital, Bispensgade 37, DK-9800, Hjoerring, Denmark. 5. Department of Pediatric and Adolescent Medicine, Aarhus University Hospital, Skejby, Palle Juul-Jensens Boulevard 99, DK-8200, Aarhus N, Denmark. soren.rittig@skejby.rm.dk.
Abstract
BACKGROUND: Childhood steroid-sensitive nephrotic syndrome (SSNS) has previously been assumed to be a disease of childhood. This has been challenged by few studies reporting that some patients with childhood SSNS may continue to relapse into adulthood. The aim of this study was to investigate the long-term outcome of childhood SSNS presenting data from an unselected well-defined cohort of Danish patients. METHODS: We conducted a retrospective study of the clinical outcome from a population of patients consecutively admitted to the pediatric departments in the central and northern region of Denmark from 1998 to 2015. Patients were followed until August 2017. Data were collected from the patient's medical records. RESULTS: Long-term outcome was studied in 39 adult patients with childhood onset SSNS. A total of 31% (12/39) had active disease in adulthood. Univariate analysis showed that more severe forms of SSNS (e.g., steroid dependent/frequent relapsing (SD/FR) nephrotic syndrome) in childhood were associated with active disease in adulthood. Comparing adult patients with SD/FR showed a significantly higher number of relapses/patient/year from late childhood and adolescence in the group with active disease vs. non-active disease (1.06 (95%CI: 0.32-1.81) vs. 0.19 (95%CI: 0.06-0.31, p = 0.005). CONCLUSION: In general, one third of all patients with SSNS during childhood continue to have active disease during early adulthood, in particular patients with SD/FR continue to suffer from active disease. The present data illustrates that SSNS is not just a disease of childhood but persists in adulthood in a significant number of patients.
BACKGROUND: Childhood steroid-sensitive nephrotic syndrome (SSNS) has previously been assumed to be a disease of childhood. This has been challenged by few studies reporting that some patients with childhood SSNS may continue to relapse into adulthood. The aim of this study was to investigate the long-term outcome of childhood SSNS presenting data from an unselected well-defined cohort of Danish patients. METHODS: We conducted a retrospective study of the clinical outcome from a population of patients consecutively admitted to the pediatric departments in the central and northern region of Denmark from 1998 to 2015. Patients were followed until August 2017. Data were collected from the patient's medical records. RESULTS: Long-term outcome was studied in 39 adult patients with childhood onset SSNS. A total of 31% (12/39) had active disease in adulthood. Univariate analysis showed that more severe forms of SSNS (e.g., steroid dependent/frequent relapsing (SD/FR) nephrotic syndrome) in childhood were associated with active disease in adulthood. Comparing adult patients with SD/FR showed a significantly higher number of relapses/patient/year from late childhood and adolescence in the group with active disease vs. non-active disease (1.06 (95%CI: 0.32-1.81) vs. 0.19 (95%CI: 0.06-0.31, p = 0.005). CONCLUSION: In general, one third of all patients with SSNS during childhood continue to have active disease during early adulthood, in particular patients with SD/FR continue to suffer from active disease. The present data illustrates that SSNS is not just a disease of childhood but persists in adulthood in a significant number of patients.
Authors: Rasmus Ehren; Marcus R Benz; Paul T Brinkkötter; Jörg Dötsch; Wolfgang R Eberl; Jutta Gellermann; Peter F Hoyer; Isabelle Jordans; Clemens Kamrath; Markus J Kemper; Kay Latta; Dominik Müller; Jun Oh; Burkhard Tönshoff; Stefanie Weber; Lutz T Weber Journal: Pediatr Nephrol Date: 2021-06-06 Impact factor: 3.714