Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial kidney histology.
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 steroid-resistant nephrotic syndrome (SRNS) suspected of h...
| Published in: | Pediatric Nephrology Vol. 35; no. 12; pp. 2377 - 2382 |
|---|---|
| Main Authors: | , , , , , |
| Format: | research tables/charts Journal Article |
| Published: |
Springer Nature
Dec2020
|
| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=146680519&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 146680519 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 0931041X EF1 jtl: Pediatric Nephrology issn: 0931041X maglogo: N pubinfo: dt: Dec2020 vid: 35 iid: 12 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 146680519 146006370 146680519 146680519 10.1007/s00467-020-04760-8 146680519 ppf: 2377 ppct: 5 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Baseline characteristics and long-term outcomes of steroid-resistant nephrotic syndrome in children: impact of initial kidney histology. aug: au: Watanabe, Yoshitaka Fujinaga, Shuichiro Endo, Amane Endo, Shota Nakagawa, Mayu Sakuraya, Koji affil: Division of Nephrology, Saitama Children's Medical Center, 1-2 Shintoshin, Chuo-ku, 330-8777, Saitama-city Saitama, Japan sug: subj: Steroids Drug Resistance Nephrotic Syndrome Prognosis Nephrotic Syndrome Diagnosis Nephrotic Syndrome Pathology Disease Attributes Human Retrospective Design Prospective Studies Descriptive Statistics Kidney Failure, Acute Epidemiology Proteinuria Epidemiology Incidence Comparative Studies Glomerulosclerosis, Focal Segmental Nephrosis Disease Remission Time Factors Biopsy Infant Child, Preschool Child Nephrotic Syndrome Etiology Nephrotic Syndrome Immunology Nephrotic Syndrome Drug Therapy Age of Onset Immunosuppressive Agents Therapeutic Use Nephrotic Syndrome Symptoms Infant: 1-23 months Child, Preschool: 2-5 years Child: 6-12 years ab: 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 steroid-resistant 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. pubtype: Academic Journal doctype: research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
|---|