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[Improved prognosis of cystinosis achieved by treatment with cysteamine and by kidney transplantation].

Authors :
Oczachowska-Kulik AE
Lund AM
Skovby F
Pedersen EB
Source :
Ugeskrift for laeger [Ugeskr Laeger] 2011 Aug 15; Vol. 173 (33), pp. 1958-62.
Publication Year :
2011

Abstract

Cystinosis is a rare, autosomal recessive disease with cystine deposits in different tissues. First signs come from kidneys and eyes, but during progression of the disease other organs can also be affected. Previously, patients with cystinosis had a very poor prognosis, but it is now considerably improved due to new methods of treatment. The purpose of this paper is to give a brief review of the disease and discuss the significant improvement of the prognosis, which has been achieved by specific medical treatment with cystine-depleting agents, and, if needed, by kidney transplantation.

Details

Language :
Danish
ISSN :
1603-6824
Volume :
173
Issue :
33
Database :
MEDLINE
Journal :
Ugeskrift for laeger
Publication Type :
Academic Journal
Accession number :
21849135