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Indications for kidney biopsy in idiopathic childhood nephrotic syndrome

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Abstract

Background

Most cases of childhood nephrotic syndrome (NS) are due to minimal change disease (MCD), while a minority of children have focal segmental glomerulosclerosis (FSGS) and an unfavorable clinical course, requiring a kidney biopsy to confirm diagnosis. We hypothesized that clinical characteristics at diagnosis and initial response to corticosteroid treatment accurately predict FSGS and can be used to guide consistent practice in the indications for kidney biopsy.

Methods

This was a case control study (1990–2012). Inclusion criteria included age 1–17 years, meeting the diagnostic criteria for NS, and having biopsy-proven FSGS or MCD. Clinical characteristics at diagnosis included age, kidney function [estimated glomerular filtration rate (eGFR)], hypertension, hematuria, nephritis (reduced eGFR, hematuria, hypertension), and response to steroids.

Results

From a total of 169 children who underwent kidney biopsy for NS we included 65 children with MCD and 22 with FSGS for analysis. There were no significant between-group differences in age, sex, or eGFR at the time of diagnosis. The FSGS group had a higher proportion of hypertension (40 vs. 15%; p = 0.02), hematuria (80 vs. 47%; p = 0.01), and nephritis (22 vs. 2%; p = 0.004) and was more likely to be steroid resistant after 6 weeks of treatment than the MCD group (67 vs. 19%; p < 0.001). As predictors of FSGS, hematuria had a high sensitivity of 0.80 [95% confidence interval (CI) 0.56–0.93] and low specificity of 0.53 (95% CI 0.39–0.66), nephritis had a low sensitivity of 0.22 (95% CI 0.07–0.48) and high specificity of 0.98 (95% CI 0.88–0.99), and steroid resistance had a low sensitivity of 0.67 (95% CI 0.43–0.85) and high specificity of 0.81 (95% CI 0.68–0.90). The combination of steroid resistance after 6 weeks of therapy and/or nephritis at diagnosis yielded the optimal sensitivity and specificity at 0.80 (95% CI 0.56–0.93) and 0.75 (95% CI 0.60–0.86), respectively, confirmed by the highest receiver operator characteristic area under the curve of 0.77.

Conclusion

Steroid resistance after 6 weeks of therapy and/or nephritis at initial presentation is an accurate predictor of FSGS in children with NS and will be used as the indication for kidney biopsy in our newly developed clinical pathway. This approach will maximize the yield of diagnostic FSGS biopsies while minimizing the number of unnecessary MCD biopsies.

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Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, University of British Columbia, Vancouver, British Columbia, Canada

    Alanoud Alshami, Abishek Roshan, Marisa Catapang, Jasper J. Jöbsis, Trevor Kwok, Nonnie Polderman, Jennifer Sibley, Matt Sibley, Cherry Mammen & Douglas G. Matsell

  2. British Columbia Children’s Hospital, 4480 Oak Street, Room K4-150, Vancouver, BC, V6H 2V2, Canada

    Douglas G. Matsell

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  1. Alanoud Alshami
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  2. Abishek Roshan
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  3. Marisa Catapang
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on behalf of the Pediatric Nephrology Clinical Pathway Development Team

Corresponding author

Correspondence to Douglas G. Matsell.

Ethics declarations

This was a case control study approved by the University of British Columbia and the British Columbia Children’s Hospital Research Ethics committees. Informed consent was waived as the study was deemed minimal risk and data were obtained through a chart review.

Conflict of interest

The authors declare no conflict of interest.

Electronic supplementary material

ESM 1

Identification of cases. A total of 420 children with the diagnosis “nephrotic syndrome” were identified, 348 from a clinical database and 169 from a biopsy database, with an overlap of 97 children. After exclusions, a total number of 178 children were identified: 22 were classified as cases of FSGS, all confirmed by biopsy, while 156 were identified as MCD. Of these, 91 were “presumed MCD”, and 65 were “biopsy-proven MCD”. (DOCX 41 kb)

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Alshami, A., Roshan, A., Catapang, M. et al. Indications for kidney biopsy in idiopathic childhood nephrotic syndrome. Pediatr Nephrol 32, 1897–1905 (2017). https://doi.org/10.1007/s00467-017-3687-3

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  • DOI: https://doi.org/10.1007/s00467-017-3687-3

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