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Treatment of corneal cystine crystal accumulation in patients with cystinosis

Authors Shams F, Livingstone I, Oladiwura D, Ramaesh K

Received 9 May 2014

Accepted for publication 4 July 2014

Published 10 October 2014 Volume 2014:8 Pages 2077—2084


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 5

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

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