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Kidney Week

Abstract: SA-PO678

Response to Glucocorticoids Remains a Valid Approach to Initial Evaluation of Children with Idiopathic Nephrotic Syndrome

Session Information

  • Pediatric Glomerular Disease
    November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Swiatecka-Urban, Agnieszka, Children's Hospital of Pittsburgh of UMPC, Pittsburgh, Pennsylvania, United States
  • Narla, Deepti, Akron children's Hospital, Akron, Ohio, United States
Background

The therapeutic approach to childhood Idiopathic Nephrotic Syndrome (INS) is based on the ISKDC and APN studiesfrom the 1960’s and 1970’s. Because most patients had minimal change disease (MCD) sensitive to glucocorticoids, the therapeutic response to the medication was recommended for initial evaluation and therapy. Since the 1990’s, several single-center studies have reported greater frequency of resistance to glucocorticoids among MCD patients and of Focal Segmental Glomerulosclerosis (FSGS). The trend is concerning, causes are unknown, and data are limited. Although initially considered a resistant lesion, more aggressive therapy can induce a response in more than 50% of FSGS patients. However, unnecessary exposure to long-term glucocorticoid therapy in resistant patients may cause side effects without a benefit. Our objective was to determine whether response to glucocorticoids remains a valid approach to initial evaluation of INS children.

Methods

A retrospective review identified 110 patients with INS treated at our center. Glucocorticoid doses and definitions of steroid sensitive (SS) and steroid resistance (SR) INS were according to ISKDC. Statistical analysis was performed by fisher exact test.

Results

The mean age was 5.9 years with equal number of males and females. Caucasians (80%) and African Americans (15%) were the predominant ethnicities. The proportion of SRINS in our study was higher, compared to ISKDC (33% vs. 20%). However, it has not been increasing with time (35% during 2003-2007 compared to 30% during 2008-2012). MCD was almost 3 times as common among the SRINS patients in our study, compared to ISKDC (52% vs. 18%). FSGS was similar among SRINS patients, compared to ISKDC (39% vs. 36%). Among patients with kidney biopsy, SSINS characterized 77% of MCD and 42% of FSGS, compared to 93-98% and 17-30% in the ISKDC studies.

Conclusion

In summary, despite the changing characteristics and different study populations, the therapeutic response to glucocorticoids is a valid approach for the initial evaluation and therapy for INS children at our center. An international study would help to monitor the changing characteristics and elucidate the role of patient demographics, ethnicity, and environmental factors that cannot be assessed by smaller studies.

Funding

  • Clinical Revenue Support