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Kidney transplantation and enzyme replacement therapy in patients with Fabry disease.
J Nephrol. 2013 Jul-Aug; 26(4):645-51.JN

Abstract

During Fabry disease, progressive glycosphingolipid deposition in the kidney causes gradual deterioration of renal function with proteinuria, uremia and hypertension. This results in end-stage renal disease (ESRD) which is one of the leading causes of morbidity and premature mortality in affected patients. Given the excellent graft and patient survival generally nowadays, kidney transplantation is the first choice to correct renal dysfunction and improve the overall prognosis of patients with renal failure because of Fabry disease. The benefit of enzyme-replacement therapy (ERT) in kidney transplanted Fabry patients has been controversially discussed and long-term trials focusing on the effectiveness of agalsidase in this patient population are needed.

Authors+Show Affiliations

FGM, Center of Internal Medicine, Department of Nephrology and Rheumatology, Müllheim, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Review

Language

eng

PubMed ID

23023720

Citation

Cybulla, Markus, et al. "Kidney Transplantation and Enzyme Replacement Therapy in Patients With Fabry Disease." Journal of Nephrology, vol. 26, no. 4, 2013, pp. 645-51.
Cybulla M, Kurschat C, West M, et al. Kidney transplantation and enzyme replacement therapy in patients with Fabry disease. J Nephrol. 2013;26(4):645-51.
Cybulla, M., Kurschat, C., West, M., Nicholls, K., Torras, J., Sunder-Plassmann, G., & Feriozzi, S. (2013). Kidney transplantation and enzyme replacement therapy in patients with Fabry disease. Journal of Nephrology, 26(4), 645-51. https://doi.org/10.5301/jn.5000214
Cybulla M, et al. Kidney Transplantation and Enzyme Replacement Therapy in Patients With Fabry Disease. J Nephrol. 2013 Jul-Aug;26(4):645-51. PubMed PMID: 23023720.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Kidney transplantation and enzyme replacement therapy in patients with Fabry disease. AU - Cybulla,Markus, AU - Kurschat,Christine, AU - West,Michael, AU - Nicholls,Kathy, AU - Torras,Joan, AU - Sunder-Plassmann,Gere, AU - Feriozzi,Sandro, Y1 - 2012/09/19/ PY - 2012/07/10/accepted PY - 2012/10/2/entrez PY - 2012/10/2/pubmed PY - 2013/11/13/medline SP - 645 EP - 51 JF - Journal of nephrology JO - J. Nephrol. VL - 26 IS - 4 N2 - During Fabry disease, progressive glycosphingolipid deposition in the kidney causes gradual deterioration of renal function with proteinuria, uremia and hypertension. This results in end-stage renal disease (ESRD) which is one of the leading causes of morbidity and premature mortality in affected patients. Given the excellent graft and patient survival generally nowadays, kidney transplantation is the first choice to correct renal dysfunction and improve the overall prognosis of patients with renal failure because of Fabry disease. The benefit of enzyme-replacement therapy (ERT) in kidney transplanted Fabry patients has been controversially discussed and long-term trials focusing on the effectiveness of agalsidase in this patient population are needed. SN - 1724-6059 UR - https://www.unboundmedicine.com/medline/citation/23023720/Kidney_transplantation_and_enzyme_replacement_therapy_in_patients_with_Fabry_disease_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=23023720.ui DB - PRIME DP - Unbound Medicine ER -