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Journal of Nephrology

, Volume 29, Issue 6, pp 791–797 | Cite as

Podocyturia is significantly elevated in untreated vs treated Fabry adult patients

  • Hernán TrimarchiEmail author
  • Romina Canzonieri
  • Amalia Schiel
  • Juan Politei
  • Aníbal Stern
  • José Andrews
  • Matías Paulero
  • Tatiana Rengel
  • Alicia Aráoz
  • Mariano Forrester
  • Fernando Lombi
  • Vanesa Pomeranz
  • Romina Iriarte
  • Pablo Young
  • Alexis Muryan
  • Elsa Zotta
Original Article

Abstract

Background

Proteinuria suggests kidney involvement in Fabry disease. We assessed podocyturia, an early biomarker, in controls and patients with and without enzyme therapy, correlating podocyturia with proteinuria and renal function.

Methods

Cross-sectional study (n = 67): controls (Group 1, n = 30) vs. Fabry disease (Group 2, n = 37) subdivided into untreated (2A, n = 19) and treated (2B, n = 18). Variables evaluated: age, gender, creatinine, CKD-EPI, proteinuria, podocyte count/10 20× microscopy power fields, podocytes/100 ml urine, podocytes/g creatininuria (results expressed as median and range).

Results

Group 1 vs. 2 did not differ concerning age, gender and CKD-EPI, but differed regarding proteinuria and podocyturia. Group 2A vs. 2B: age: 29 (18–74) vs. 43 (18–65) years (p = ns); gender: males n = 3 (16 %) vs. n = 9 (50 %). Proteinuria was significantly higher in Fabry treated patients, while CKD-EPI and podocyturia were significantly elevated in untreated individuals. Significant correlations: group 2A: age-proteinuria, ρ = 0.62 (p = 0.0044); age-CKD-EPI, ρ = −0.84 (p < 0.0001); podocyturia-podocytes/100 ml urine, ρ = 0.99 (p = 0.0001); podocyturia-podocytes/g creatininuria ρ = 0.86 (p = 0.0003), podocytes/100 ml urine-podocytes/g urinary creatinine, ρ = 0.84 (p = 0.0004); proteinuria-CKD-EPI, ρ = −0.68 (p = 0.0013). Group 2B: podocyturia-podocytes/100 ml urine, ρ = 0.88 (p < 0.0001); podocyturia-podocytes/g creatininuria, ρ = 0.84 (p < 0.0001); podocytes/100 ml urine-podocytes/g creatininuria, ρ = 0.94 (p < 0.0001); CKD-EPI-proteinuria, ρ = -0.66 (p = 0.0028).

Conclusions

Patients with Fabry disease display heavy podocyturia; those untreated present significantly higher podocyturia, lower proteinuria and better renal function than those who are treated, suggesting that therapy may be started at advanced stages. Podocyturia may antedate proteinuria, and enzyme therapy may protect against podocyte loss.

Keywords

Fabry disease proteinuria Podocyte Podocyturia Α-galactosidase 

Notes

Acknowledgments

We wish to thank Dr Rosanna Coppo for reviewing our manuscript, and Ms. Laura Ares and Ms. Marina Fernandez for their professional assistance.

Compliance with ethical standards

Conflict of interests

Hernán Trimarchi is a consultant to Genzyme for the product alpha galactosidase-β.

Ethical approval

This article does not contain any studies with human participants or animals performed by any of the authors.

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Copyright information

© Italian Society of Nephrology 2016

Authors and Affiliations

  • Hernán Trimarchi
    • 1
    • 6
    Email author
  • Romina Canzonieri
    • 2
  • Amalia Schiel
    • 2
  • Juan Politei
    • 3
  • Aníbal Stern
    • 2
  • José Andrews
    • 1
  • Matías Paulero
    • 1
  • Tatiana Rengel
    • 1
  • Alicia Aráoz
    • 4
  • Mariano Forrester
    • 1
  • Fernando Lombi
    • 1
  • Vanesa Pomeranz
    • 1
  • Romina Iriarte
    • 1
  • Pablo Young
    • 5
  • Alexis Muryan
    • 2
  • Elsa Zotta
    • 4
  1. 1.NephrologyHospital Británico de Buenos AiresBuenos AiresArgentina
  2. 2.Biochemistry ServicesHospital Británico de Buenos AiresBuenos AiresArgentina
  3. 3.Neurology DepartmentFundación para el Estudio de las Enfermedades Metabólicas FESENBuenos AiresArgentina
  4. 4.IFIBIO Houssay, UBA CONICET Facultad de MedicinaUniversidad de Buenos AiresBuenos AiresArgentina
  5. 5.Internal MedicineHospital Británico de Buenos AiresBuenos AiresArgentina
  6. 6.Servicio de NefrologíaHospital Británico de Buenos AiresBuenos AiresArgentina

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