Podocyturia in Fabry disease: a 10-year follow-up.

Autor:	Vujkovac B; Department of Internal Medicine, General Hospital Slovenj Gradec, Slovenj Gradec, Slovenia., Srebotnik Kirbiš I; Faculty of Medicine, Institute of Pathology, University of Ljubljana, Ljubljana, Slovenia., Keber T; Department of Internal Medicine, General Hospital Slovenj Gradec, Slovenj Gradec, Slovenia., Cokan Vujkovac A; Department of Internal Medicine, General Hospital Slovenj Gradec, Slovenj Gradec, Slovenia., Tretjak M; Department of Internal Medicine, General Hospital Slovenj Gradec, Slovenj Gradec, Slovenia., Radoš Krnel S; PHARMEC s.p., Ljubljana, Slovenia.
Jazyk:	angličtina
Zdroj:	Clinical kidney journal [Clin Kidney J] 2021 Sep 16; Vol. 15 (2), pp. 269-277. Date of Electronic Publication: 2021 Sep 16 (Print Publication: 2022).
DOI:	10.1093/ckj/sfab172
Abstrakt:	Background: Fabry disease (FD) is a rare X-linked disorder of sphingolipid metabolism that results in chronic proteinuric nephropathy. Podocytes are one of the most affected renal cells and play an important role in the development and progression of kidney disease. Detached podocytes found in urine (podocyturia) are considered as a non-invasive early marker of kidney injury; however, the dynamics of podocyte loss remains unknown. Methods: In this 10-year follow-up study, podocyturia and other renal clinical data were evaluated in 39 patients with FD. From 2009 to 2019, podocyturia was assessed in 566 fresh urine samples from 13 male and 26 female FD patients using immunocytochemical detection of podocalyxin. Results: Podocyturia (number of podocytes per 100 mL of urine) was found in 311/566 (54.9%) of the samples, more frequently (68.9 ± 21.9% versus 50.6 ± 25.9%; P = 0.035) and with higher values (364 ± 286 versus 182 ± 180 number of podocytes per gram of creatinine (Cr) in urine; P = 0.020) in males compared with females. The mean number of assessed samples for each patient was 14.5 (range 3-40) and the frequency of samples with podocyturia ranged from 0% to 100% (median 57%). Podocyturia was already present in 42.9% of patients <20 years of age and in 89.5% of normoalbuminuric patients. Podocyturia correlated with albuminuria (urine albumin:Cr ratio) ( r = 0.20, P < 0.001) and a higher incidence and values of podocyturia were observed in patients with lower estimated glomerular filtration rate. Conclusions: Our data demonstrated that podocyturia is an early clinical event in the development of nephropathy. In addition, we found podocyturia to be a discontinuous event with wide variability. (© The Author(s) 2021. Published by Oxford University Press on behalf of the ERA.)
Databáze:	MEDLINE
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