ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: FR-PO658

Anti-Nephrin Antibodies in Idiopathic Nephrotic Syndrome in Japanese Children

Session Information

  • Pediatric Nephrology - II
    November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology

Authors

  • Horinouchi, Tomoko, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Watts, Andrew James baxter, Department of Medicine/Renal Division, Brigham and Women’s Hospital, and Harvard Medical School, Boston, Massachusetts, United States
  • Nagano, China, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Ichikawa, Yuta, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Inoki, Yuta, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Tanaka, Yu, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Kitakado, Hideaki, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Ueda, Chika, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Kondo, Atsushi, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Sakakibara, Nana, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Shima, Yuko, Department of Pediatrics, Wakayama Medical University, Wakayama, Japan
  • Nozu, Kandai, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan
  • Sampson, Matt G., Department of Medicine/Renal Division, Brigham and Women’s Hospital, and Harvard Medical School, Boston, Massachusetts, United States
  • Weins, Astrid, Department of Pathology, Brigham and Women’s Hospital, and Harvard Medical School, Boston, Massachusetts, United States
  • Iijima, Kazumoto, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan
Background

Many patients with childhood idiopathic nephrotic syndrome are steroid-sensitive, suggesting the involvement of the immune system in the pathogenesis. Several genome-wide association studies have suggested a polygenic contribution, particularly in the HLA DR/DQ region and a locus including NPHS1, but the etiology remains unclear. Anti-nephrin antibodies have recently been reported in both adults and children with biopsy proven minimal change disease (MCD), but the presence of anti-nephrin antibodies in Japanese childhood idiopathic nephrotic syndrome (INS) has not been investigated.

Methods

Anti-nephrin antibodies were measured by ELISA in paired plasma samples obtained from 14 Japanese pediatric patients with INS (male/female: 8/6), at initial disease onset (active disease) and following steroid monotherapy. Clinical characteristics were compared between the anti-nephrin antibodies positive and negative groups.

Results

The median age at the onset was 75.5 months (interquartile range (IQR): 45-113). Steroid sensitivity resulted in complete remission in 13 patients and almost complete remission in one patient after 4 weeks of glucocorticoid monotherapy. Circulating anti-nephrin antibodies were detected in seven of 14 patients during active disease. In all cases, anti-nephrin antibodies were significantly reduced following treatment concordant with clinical response. There were no differences between the positive and negative groups in pre-treatment parameters. Of the 13 patients who achieved complete remission, nine had at least one relapse during a median follow-up of 851 days (IQR: 808-973). There was also no significant difference in the relapse-free period after the onset between the two groups (P=0.658).

Conclusion

We have identified circulating anti-nephrin antibodies at initial presentation in half of Japanese pediatric INS, which is a higher proportion than previously reported for a North American cohort of adults and children with biopsy proven MCD. Further studies are needed to establish the prognostic implications of anti-nephrin antibodies in childhood INS and the relationship with the NPHS1 risk variants in this population.

Funding

  • Government Support – Non-U.S.