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J Nephrol. 2016 Dec;29(6):791-797. Epub 2016 Feb 3.

Podocyturia is significantly elevated in untreated vs treated Fabry adult patients.

Author information

1
Nephrology, Hospital Británico de Buenos Aires, Buenos Aires, Argentina. htrimarchi@hotmail.com.
2
Servicio de Nefrología, Hospital Británico de Buenos Aires, Perdriel 74, 1280, Buenos Aires, Argentina. htrimarchi@hotmail.com.
3
Biochemistry Services, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.
4
Neurology Department, Fundación para el Estudio de las Enfermedades Metabólicas FESEN, Buenos Aires, Argentina.
5
Nephrology, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.
6
IFIBIO Houssay, UBA CONICET Facultad de Medicina, Universidad de Buenos Aires, Buenos Aires, Argentina.
7
Internal Medicine, Hospital Británico de Buenos Aires, Buenos Aires, Argentina.

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; Podocyte; Podocyturia; proteinuria; Α-galactosidase

PMID:
26842625
DOI:
10.1007/s40620-016-0271-z
[Indexed for MEDLINE]

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