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Abstract: SA-PO585

Clinical Presentation and Management of Nephrotic Syndrome in the First Year of Life: A Report From the Pediatric Nephrology Research Consortium (PNRC)

Session Information

  • Pediatric Nephrology - II
    November 05, 2022 | Location: Exhibit Hall, Orange County Convention Center‚ West Building
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1800 Pediatric Nephrology

Authors

  • Kamigaki, Yu, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Constantinescu, Alexandru R., Joe DiMaggio Children's Hospital, Hollywood, Florida, United States
  • Mattoo, Tej K., Wayne State University School of Medicine, Detroit, Michigan, United States
  • Greenbaum, Larry A., Emory University School of Medicine, Atlanta, Georgia, United States
  • Muff-Luett, Melissa A., Children's Hospital and Medical Center, Omaha, Nebraska, United States
  • Annaim, Ali, Medical University of South Carolina, Charleston, South Carolina, United States
  • Wenderfer, Scott E., The University of British Columbia Faculty of Medicine, Vancouver, British Columbia, Canada
  • Plautz, Emilee, University of Minnesota, Minneapolis, Minnesota, United States
  • Rheault, Michelle N., University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, United States
  • Myette, Robert L., Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada
  • Twombley, Katherine, Medical University of South Carolina, Charleston, South Carolina, United States
  • Kallash, Mahmoud, Nationwide Children's Hospital, Columbus, Ohio, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Vasylyeva, Tetyana L., Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, Texas, United States
Background

Nephrotic syndrome (NS) in the first year of life is called congenital (CNS) if diagnosed between 0-3 months, or infantile (INS) if diagnosed between 3-12 months of age. The aim of this study was to determine if there were clinically meaningful differences between CNS vs. INS patients regarding clinical presentation, management, and outcomes.

Methods

11 PNRC sites participated in the study, using IRB-approved retrospective chart reviews of CNS and INS patients born between 1998-2019. Data were collected on patient characteristics, pertinent laboratory tests, need and frequency of albumin infusions, and type and timing of nephrectomy and renal replacement therapy (RRT).

Results

The study included 69 patients, 49 with CNS and 20 with INS, median ages at diagnosis of 1 month and 6 months, respectively. Patients were similar with respect to nutrition, thyroxin supplementation, IVIG/SCIG prophylaxis, and thrombosis prophylaxis. Within the first 2 months after diagnosis, daily albumin infusions were more frequently needed in CNS vs. INS (79 vs. 30%; p=0.006), while weekly infusions were more frequently needed in INS vs. CNS (50 vs. 3%; p=0.001). Moreover, within the final 6 months preceding RRT, albumin infusions were more frequently required in CNS vs. INS (51 vs. 15%; p=0.007). Nephrectomy was also performed more frequently in CNS vs. INS (78 vs. 50%: p=0.024). Dialysis was similarly required in CNS vs. INS (73 vs. 55%; p=0.14). Pre-emptive kidney transplantations were similarly performed in CNS vs. INS patients (6% vs. 5%). Notably, management without either nephrectomy or RRT was more frequent in INS vs. CNS (40% vs. 16%; p=0.035). Sequences of interventions (i.e., Nephrectomy->RRT->Transplant [TXP]) were similar between the groups, although RRT->bilateral nephrectomy->TXP tended to occur more frequently in CNS vs. INS (61 vs. 36%; p=0.06).

Conclusion

Compared to children with INS, those with CNS had more severe disease courses, requiring more frequent albumin infusions, and earlier onset of RRT. In addition, almost 25% of children with CNS or INS were able to be managed without need for either nephrectomy or RRT.