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NEPTUNE Scoring for MCD and FSGS

Fill out the following form to generate a table or narrative for standardized biopsy reporting for podocytopathies. The features marked by asterisks below (*) were identified and described in Zee et al. (JASN 33, 2022) as the most predictive of clinical outcomes (40% decline in eGFR or kidney replacement therapy, complete proteinuria remission, and/or immunosuppressant treatment response). Other items are included for completeness and convenience.

The scoring system is intended to be applied to any kidney biopsy that has a conventional diagnosis of any form of MCD (i.e., idiopathic, monogenic, drug-induced, etc.), including classical MCD or MCD-Like (partial effacement or full effacement with global sclerosis exceeding that expected for age), or FSGS (i.e., idiopathic/primary, secondary, monogenic, etc. and any of the historically used sub-types). The value of these parameters has not been tested in other glomerular diseases accompanied by segmental sclerosis or minimal change disease (e.g., diabetic nephropathy with segmental sclerosis or IgA nephropathy with minimal change disease or MEST score S1).

Any entries left blank or marked N/A will not be included in the narrative. The table and narrative can be copied and the data can also be exported. Please read over the narrative and check for accuracy before use.
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 # glomeruli  
OR   % of total glomeruli
 # glomeruli  
OR   % of total glomeruli
 # glomeruli  
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OR   % of total glomeruli
 # glomeruli   
OR   % of total glomeruli
 # glomeruli   
OR   % of total glomeruli
 # glomeruli   
OR   % of total glomeruli
 # glomeruli   
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OR   % of total glomeruli
 # glomeruli  
OR   % of total glomeruli
 # glomeruli  
OR   % of total glomeruli
 # glomeruli  
OR   % of total glomeruli
 # glomeruli  
OR   % of total glomeruli
 # glomeruli  
OR   % of total glomeruli
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 %
 %
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 %
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Zee J, Liu Q, Smith AR, Hodgin JB, Rosenberg A, Gillespie BW, Holzman LB, Barisoni L, Mariani LH. Kidney Biopsy Features Most Predictive of Clinical Outcomes in the Spectrum of Minimal Change Disease and Focal Segmental Glomerulosclerosis. J Am Soc Nephrol. 2022 May 17;ASN.2021101396. doi: 10.1681/ASN.2021101396.

Hodgin JB, Mariani LH, Zee J, Liu Q, Smith AR, Eddy S, Hartman J, Hamidi H, Gaut JP, Palmer MB, Nast CC, Chang A, Hewitt S, Gillespie BW, Kretzler M, Holzman LB, Barisoni L; Nephrotic Syndrome Study Network (NEPTUNE). Quantification of Glomerular Structural Lesions: Associations With Clinical Outcomes and Transcriptomic Profiles in Nephrotic Syndrome. Am J Kidney Dis. 2022 Jun;79(6):807-819.e1. doi: 10.1053/j.ajkd.2021.10.004. Epub 2021 Dec 3. PMID: 34864148

Royal V, Zee J, Liu Q, Avila-Casado C, Smith AR, Liu G, Mariani LH, Hewitt S, Holzman LB, Gillespie BW, Hodgin JB, Barisoni L. Ultrastructural Characterization of Proteinuric Patients Predicts Clinical Outcomes. J Am Soc Nephrol. 2020 Apr;31(4):841-854. doi: 10.1681/ASN.2019080825. Epub 2020 Feb 21. PMID: 32086276; PMCID: PMC7191920.

Zee J, Hodgin JB, Mariani LH, Gaut JP, Palmer MB, Bagnasco SM, Rosenberg AZ, Hewitt SM, Holzman LB, Gillespie BW, Barisoni L. Reproducibility and Feasibility of Strategies for Morphologic Assessment of Renal Biopsies Using the Nephrotic Syndrome Study Network Digital Pathology Scoring System. Arch Pathol Lab Med. 2018 May;142(5):613-625. doi: 10.5858/arpa.2017-0181-OA. Epub 2018 Feb 19. PMID: 29457738; PMCID: PMC5946059.

Mariani LH, Martini S, Barisoni L, Canetta PA, Troost JP, Hodgin JB, Palmer M, Rosenberg AZ, Lemley KV, Chien HP, Zee J, Smith A, Appel GB, Trachtman H, Hewitt SM, Kretzler M, Bagnasco SM. Interstitial fibrosis scored on whole-slide digital imaging of kidney biopsies is a predictor of outcome in proteinuric glomerulopathies. Nephrol Dial Transplant. 2018 Feb 1;33(2):310-318. doi: 10.1093/ndt/gfw443. PMID: 28339906; PMCID: PMC5837529.

Barisoni L, Gimpel C, Kain R, Laurinavicius A, Bueno G, Zeng C, Liu Z, Schaefer F, Kretzler M, Holzman LB, Hewitt SM. Digital pathology imaging as a novel platform for standardization and globalization of quantitative nephropathology. Clin Kidney J. 2017 Apr;10(2):176-187. doi: 10.1093/ckj/sfw129. Epub 2017 Feb 18. PMID: 28584625; PMCID: PMC5455257.

Barisoni L, Troost JP, Nast C, Bagnasco S, Avila-Casado C, Hodgin J, Palmer M, Rosenberg A, Gasim A, Liensziewski C, Merlino L, Chien HP, Chang A, Meehan SM, Gaut J, Song P, Holzman L, Gibson D, Kretzler M, Gillespie BW, Hewitt SM. Reproducibility of the NEPTUNE descriptor-based scoring system on whole-slide images and histologic and ultrastructural digital images. Mod Pathol. 2016 Jul;29(7):671-84. doi: 10.1038/modpathol.2016.58. Epub 2016 Apr 22. PMID: 27102348; PMCID: PMC5515468.

Nast CC, Lemley KV, Hodgin JB, Bagnasco S, Avila-Casado C, Hewitt SM, Barisoni L. Morphology in the Digital Age: Integrating High-Resolution Description of Structural Alterations With Phenotypes and Genotypes. Semin Nephrol. 2015 May;35(3):266-78. doi: 10.1016/j.semnephrol.2015.04.006. PMID: 26215864; PMCID: PMC4764351.

Haas M, Seshan SV, Barisoni L, Amann K, Bajema IM, Becker JU, Joh K, Ljubanovic D, Roberts ISD, Roelofs JJ, Sethi S, Zeng C, Jennette JC. Consensus Definitions for Glomerular Lesions by Light and Electron Microscopy: Recommendations from a Working Group of the Renal Pathology Society. Kidney Int. 2020 Nov;98(5):1120-1134. doi: 10.1016/j.kint.2020.08.006. PMID: 32866505.
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