The DOI matches its Crossref record; 4 of the 4 references it cites were found in a registry or an index.
Fabry disease: progression of nephropathy, and prevalence of cardiac and cerebrovascular events before enzyme replacement therapy
Open accessAbstract
BACKGROUND: In Fabry disease, progressive glycolipid accumulation leads to organ damage and early demise, but the incidence of renal, cardiac and cerebrovascular events has not been well characterized. METHODS: We conducted a retrospective chart review of 279 affected males and 168 females from 27 sites (USA, Canada, Europe). The pre-defined study endpoints included progression of renal, cardiac and cerebrovascular involvement and/or death before the initiation of enzyme replacement therapy. RESULTS: The mean rate of estimated glomerular filtration rate (eGFR) decline for patients was -2.93 for males, and -1.02 ml/min/1.73 m(2)/year for females. Prevalence and severity of proteinuria, baseline eGFR <60 ml/min/1.73 m(2) and hypertension were associated with more rapid loss of eGFR. Advanced Fabry nephropathy was more prevalent and occurred earlier among males than females. Cardiac events (mainly arrhythmias), strokes and transient ischaemic attacks occurred in 49, 11, 6% of males, and in 35, 8, 4% of females, respectively. The mean age at death for 20 male patients was 49.9 years. CONCLUSIONS: Baseline proteinuria, reduced baseline eGFR, hypertension and male gender were associated with more rapid progression of Fabry nephropathy. The eGFR progression rate may increase with advancing nephropathy, and may differ between subgroups of patients with Fabry disease.
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Brenner, B. M., Cooper, M. E., de Zeeuw, D., Keane, W. F., Mitch, W. E., Parving, H.-H., Remuzzi, G., Snapinn, S. M., Zhang, Z., & Shahinfar, S. (2001) · New England Journal of Medicine · doi:10.1056/nejmoa011161 · Cited by 7,493
Lewis, E. J., Hunsicker, L. G., Clarke, W. R., Berl, T., Pohl, M. A., Lewis, J. B., Ritz, E., Atkins, R. C., Rohde, R., & Raz, I. (2001) · New England Journal of Medicine · doi:10.1056/nejmoa011303 · Cited by 5,987
The effect of angiotensin-converting-enzyme inhibition on diabetic nephropathy
Lewis, E. J., Hunsicker, L. G., Bain, R. P., & Rohde, R. D. (1993) · New England Journal of Medicine · doi:10.1056/nejm199311113292004 · Cited by 5,394
Assessing Kidney Function — Measured and Estimated Glomerular Filtration Rate
Stevens, L. A., Coresh, J., Greene, T., & Levey, A. S. (2006) · New England Journal of Medicine · doi:10.1056/nejmra054415 · Cited by 2,952
Cited by
Germain, D. P. (2010) · Orphanet Journal of Rare Diseases · doi:10.1186/1750-1172-5-30 · Cited by 1,198
Treatment of Fabry’s Disease with the Pharmacologic Chaperone Migalastat
Germain, D. P., Hughes, D. A., Nicholls, K., Bichet, D. G., Giugliani, R., Wilcox, W. R., Feliciani, C., Shankar, S. P., Ezgu, F., Amartino, H., et al. (2016) · New England Journal of Medicine · doi:10.1056/nejmoa1510198 · Cited by 555
Self-citationX‐chromosome inactivation in female patients with Fabry disease
Echevarria, L., Benistan, K., Toussaint, A., Dubourg, O., Hagege, A. A., Eladari, D., Jabbour, F., Beldjord, C., De Mazancourt, P., & Germain, D. P. (2016) · Clinical Genetics · doi:10.1111/cge.12613 · Cited by 424
Hughes, D. A., Nicholls, K., Shankar, S. P., Sunder-Plassmann, G., Koeller, D., Nedd, K., Vockley, G., Hamazaki, T., Lachmann, R., Ohashi, T., et al. (2017) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2016-104178 · Cited by 386
Self-citationTen-year outcome of enzyme replacement therapy with agalsidase beta in patients with Fabry disease
Germain, D. P., Charrow, J., Desnick, R. J., Guffon, N., Kempf, J., Lachmann, R. H., Lemay, R., Linthorst, G. E., Packman, S., Scott, C. R., et al. (2015) · Journal of Medical Genetics · doi:10.1136/jmedgenet-2014-102797 · Cited by 368
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Metadata
- Authors
- Schiffmann, R., Warnock, D. G., Banikazemi, M., Bultas, J., Linthorst, G. E., Packman, S., Sorensen, S. A., Wilcox, W. R., & Desnick, R. J.
- Venue
- Nephrology Dialysis Transplantation
- Publisher
- Oxford University Press (OUP)
- Published
- 2009-02-13
- Volume
- 24
- Issue
- 7
- Pages
- 2102-2111
- ISSN
- 0931-0509 (ISSN-L)
- 1460-2385
- OpenAlex
- W2126269101
- Cited by
- 368
- References
- 4
- FWCI
- 14.57
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- Schiffmann, R., Warnock, D. G., Banikazemi, M., Bultas, J., Linthorst, G. E., Packman, S., Sorensen, S. A., Wilcox, W. R., & Desnick, R. J. (2009). Fabry disease: progression of nephropathy, and prevalence of cardiac and cerebrovascular events before enzyme replacement therapy. Nephrology Dialysis Transplantation. https://doi.org/10.1093/ndt/gfp031
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