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Effects of long-term cysteamine treatment in patients with cystinosis

Cystinosis is a rare autosomal-recessive lysosomal storage disease with high morbidity and mortality. It is caused by mutations in the CTNS gene that encodes the cystine transporter, cystinosin, which leads to lysosomal cystine accumulation. Patients with infantile nephropathic cystinosis, the most...

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Publicado en:Pediatr Nephrol
Main Authors: Ariceta, Gema, Giordano, Vincenzo, Santos, Fernando
Formato: Artigo
Idioma:Inglês
Publicado: Springer Berlin Heidelberg 2017
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC6394685/
https://ncbi.nlm.nih.gov/pubmed/29260317
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00467-017-3856-4
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