Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial k
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BRIEF REPORT
Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial kidney histology Yoshitaka Watanabe 1,2 & Shuichiro Fujinaga 1
&
Amane Endo 3 & Shota Endo 1 & Mayu Nakagawa 1 & Koji Sakuraya 1
Received: 11 July 2020 / Revised: 2 August 2020 / Accepted: 3 September 2020 # IPNA 2020
Abstract Background Although many pediatric nephrologists consider focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) as separate clinical entities, whether the initial histology could affect clinical courses in children with steroidresistant nephrotic syndrome (SRNS) suspected of having an immune-based etiology remains unknown, especially for long-term outcomes. Methods We retrospectively reviewed long-term outcomes (> 3 years; median follow-up, 9.1 years) of 21 children with initial SRNS (FSGS, N = 9; MCD, N = 12) who achieved complete remission with immunosuppressive agents, including cyclosporine. Results At NS onset, incidence of acute kidney injury (67% vs. 8%, P < 0.05) and proportion of patients with non-selective proteinuria (56% vs. 0%, P < 0.01) were significantly higher in the FSGS group than the MCD group. Furthermore, median days until complete remission after treatment was significantly longer in the FSGS group than the MCD group (116 days vs. 45 days, P < 0.001). Although subsequent biopsy histology of the 12 patients in the MCD group was still identical in all MCD, three of nine patients in the FSGS group were reclassified from FSGS to MCD at second biopsy. At last visit, all patients maintained complete remission, and none developed chronic kidney disease. Conclusions Initial presentation in the FSGS group was characterized by more severe clinical manifestations than the MCD group. If complete remission is achieved, FSGS and MCD in children with immune-mediated SRNS may constitute a single disease spectrum because the long-term outcomes are favorable, irrespective of initial histology. Keywords Initial steroid-resistant nephrotic syndrome . Immune-based etiology . Focal segmental glomerulosclerosis . Minimal change disease . Single disease spectrum . Children
Introduction Steroid-resistant nephrotic syndrome (SRNS) is considered a heterogeneous pathological entity, including immunemediated and monogenic etiologies [1]. Focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are the two key histological diagnoses in children with SRNS as an immune-mediated disease. At present, there are
* Shuichiro Fujinaga [email protected] 1
Division of Nephrology, Saitama Children’s Medical Center, 1-2 Shintoshin, Chuo-ku, Saitama-city Saitama 330-8777, Japan
2
Children’s Medical Center, Showa University Northern Yokohama Hospital, Yokohama, Japan
3
Department of Pediatrics and adolescent medicine, Juntendo University, Tokyo, Japan
controversies about whether FSGS and MCD in this cohort are distinct glomerular lesions or belong to the same disease spectrum [2–4], because the clinical manifestations such as ma
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