Podocyturia is significantly elevated in untreated vs treated Fabry adult patients

Autor: Alexis Muryan, Mariano Forrester, Aníbal Stern, José Andrews, Hernán Trimarchi, Amalia Schiel, Elsa Zotta, Romina Canzonieri, Romina Iriarte, Matías Paulero, Fernando Lombi, Alicia Aráoz, Vanesa Pomeranz, Pablo Young, Tatiana Rengel, Juan Politei
Rok vydání: 2016
Předmět:
Male
0301 basic medicine
Nephrology
Time Factors
030232 urology & nephrology
Urine
urologic and male genital diseases
Bioinformatics
Podocyte
0302 clinical medicine
Risk Factors
Medicine
Kidney
Proteinuria
Podocytes
Middle Aged
female genital diseases and pregnancy complications
Isoenzymes
Treatment Outcome
medicine.anatomical_structure
Creatinine
Biomarker (medicine)
Female
medicine.symptom
Adult
medicine.medical_specialty
Adolescent
Urology
Renal function
Urinalysis
Young Adult
03 medical and health sciences
Internal medicine
Humans
Enzyme Replacement Therapy
Renal Insufficiency
Chronic
Aged
Adult patients
urogenital system
business.industry
medicine.disease
Fabry disease
Cross-Sectional Studies
030104 developmental biology
Case-Control Studies
alpha-Galactosidase
Fabry Disease
business
Biomarkers
Zdroj: Journal of Nephrology. 29:791-797
ISSN: 1724-6059
1121-8428
DOI: 10.1007/s40620-016-0271-z
Popis: 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.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).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).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.
Databáze: OpenAIRE
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