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Abstract: FR-PO1170

Morbidity of Childhood Nephrotic Syndrome: The Childhood Nephrotic Syndrome Observational Study, a Midwest Pediatric Nephrology Consortium Study

Session Information

  • Pediatric Nephrology - I
    October 26, 2018 | Location: Exhibit Hall, San Diego Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1600 Pediatric Nephrology

Authors

  • Wang, Chia- Shi, Emory University, Atlanta, Georgia, United States
  • Akchurin, Oleh M., Weill Cornell College of Medicine, New York, New York, United States
  • Greenbaum, Larry A., Emory University, Atlanta, Georgia, United States
  • Mahan, John D., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Smoyer, William E., Nationwide Children's Hospital, Columbus, Ohio, United States
  • Rheault, Michelle N., University of Minnesota, Minneapolis, Minnesota, United States

Group or Team Name

  • CNOS investigators
Background

Childhood nephrotic syndrome (NS) is one of the most common pediatric chronic kidney diseases. Systematic studies of the complexity of treatment and disease complications are rare.

Methods

The Childhood Nephrotic Syndrome Study (CNOS) is an international, prospective, observational study of children 1-18 yrs with idiopathic NS enrolled within 2 mos of NS diagnosis. Demographic and clinical data are collected at presentation, 3 mos, and yearly. We describe healthcare utilization, treatment, treatment response, and disease complications among CNOS participants followed for at least 1 yr. Changes in corticosteroid (CST) treatment response from initial episode were noted.

Results

As of April 1, 2018, 127 had completed 12 mos follow-up. All patients were hospitalized at least once, with a total of 265 hospitalizations (2.1 hospitalizations/patient-year). The most common primary reason for hospitalization (82/265, 31% hospitalizations) was edema. Hospitalizations were complicated by blood clots in 3 patients (2.4%) and infections in 13 (10.2%). The majority of patients (77.2%) responded to the initial course of CST; however, an additional 5/98 (5.1%) were CST-resistant at 1 yr (i.e., late steroid-resistance). Frequently relapsing or CST-dependent NS was seen in 38/127 (29.9%). Second-line therapies were used in 69/127 (54.3%, Table). One patient (0.8%) reached end-stage renal disease.

Conclusion

Healthcare utilization and disease morbidity are high in childhood NS. Treatment is complex, with a large proportion of patients exposed to immunosuppressive agents in addition to CST, even among those initially responsive to CST. Further research and quality improvement efforts are needed to decrease hospitalizations and morbidity in children with NS.

Immunosuppressive therapies in first year of NS diagnosis
Treatment, n (%)Steroid-responsive NS (N=98; 77.2%)Steroid-resistant NS (N=29; 22.8%)Total* (N=127; 100%)
Intravenous corticosteroids 6 (6.1%)2 (6.9%)8 (6.3%)
Cyclophosphamide 3 (3.1%)03 (2.4%)
Cyclosporin 1 (1.0%)
4 (13.8%)5 (3.9%)
Tacrolimus 12 (12.2%)15 (51.7%)27 (21.3%)
Mycophenolate mofetil 17 (17.3%)8 (27.6%)25 (19.7%)
Rituximab 2 (2.0%)02 (1.6%)

*Disease classification based on response to initial course of CST

Funding

  • Private Foundation Support