Treatment of corneal cystine crystal accumulation in patients with cystinosis

Fatemeh Shams, Iain Livingstone, Dilys Oladiwura, Kanna Ramaesh Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland Abstract: Cystinosis is a rare autosomal recessive disorder characterized by the accumulation of cystine within the cells of different organs. Infantile nephr...

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Autores principales:	Shams F, Livingstone I, Oladiwura D, Ramaesh K
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2014
Materias:	Ophthalmology RE1-994
Acceso en línea:	https://doaj.org/article/4f0ecb88e1684962b0f8c0b90a9310b3
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spelling	oai:doaj.org-article:4f0ecb88e1684962b0f8c0b90a9310b32021-12-02T06:32:30ZTreatment of corneal cystine crystal accumulation in patients with cystinosis1177-5483https://doaj.org/article/4f0ecb88e1684962b0f8c0b90a9310b32014-10-01T00:00:00Zhttp://www.dovepress.com/treatment-of-corneal-cystine-crystal-accumulation-in-patients-with-cys-peer-reviewed-article-OPTHhttps://doaj.org/toc/1177-5483 Fatemeh Shams, Iain Livingstone, Dilys Oladiwura, Kanna Ramaesh Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland Abstract: Cystinosis is a rare autosomal recessive disorder characterized by the accumulation of cystine within the cells of different organs. Infantile nephropathic cystinosis is the most common and severe phenotype. With the success of renal transplantation, these patients are now living longer and thus more long-term complications within different organs are becoming apparent. Ophthalmic manifestations range from corneal deposits of cystine crystals to pigmentary retinopathy. With increasing age, more severe ocular complications have been reported. Photophobia is a prominent symptom for patients. With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating. This review revisits the basic pathogenesis of cystinosis, the ocular manifestations of the disease, and the treatment of corneal crystals. Keywords: cystinosis, cysteamine, cornea, ocular, crystals, lysosomeShams FLivingstone IOladiwura DRamaesh KDove Medical PressarticleOphthalmologyRE1-994ENClinical Ophthalmology, Vol 2014, Iss default, Pp 2077-2084 (2014)
institution	DOAJ
collection	DOAJ
language	EN
topic	Ophthalmology RE1-994
spellingShingle	Ophthalmology RE1-994 Shams F Livingstone I Oladiwura D Ramaesh K Treatment of corneal cystine crystal accumulation in patients with cystinosis
description	Fatemeh Shams, Iain Livingstone, Dilys Oladiwura, Kanna Ramaesh Department of Ophthalmology, Gartnavel General Hospital, Glasgow, Scotland Abstract: Cystinosis is a rare autosomal recessive disorder characterized by the accumulation of cystine within the cells of different organs. Infantile nephropathic cystinosis is the most common and severe phenotype. With the success of renal transplantation, these patients are now living longer and thus more long-term complications within different organs are becoming apparent. Ophthalmic manifestations range from corneal deposits of cystine crystals to pigmentary retinopathy. With increasing age, more severe ocular complications have been reported. Photophobia is a prominent symptom for patients. With prolonged survival and increasing age, this symptom, along with corneal erosions and blepharospasm, can become debilitating. This review revisits the basic pathogenesis of cystinosis, the ocular manifestations of the disease, and the treatment of corneal crystals. Keywords: cystinosis, cysteamine, cornea, ocular, crystals, lysosome
format	article
author	Shams F Livingstone I Oladiwura D Ramaesh K
author_facet	Shams F Livingstone I Oladiwura D Ramaesh K
author_sort	Shams F
title	Treatment of corneal cystine crystal accumulation in patients with cystinosis
title_short	Treatment of corneal cystine crystal accumulation in patients with cystinosis
title_full	Treatment of corneal cystine crystal accumulation in patients with cystinosis
title_fullStr	Treatment of corneal cystine crystal accumulation in patients with cystinosis
title_full_unstemmed	Treatment of corneal cystine crystal accumulation in patients with cystinosis
title_sort	treatment of corneal cystine crystal accumulation in patients with cystinosis
publisher	Dove Medical Press
publishDate	2014
url	https://doaj.org/article/4f0ecb88e1684962b0f8c0b90a9310b3
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Treatment of corneal cystine crystal accumulation in patients with cystinosis

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