title: Gel formulations for treatment of the ophthalmic complications in cystinosis.
abstract: Nephropathic cystinosis is a rare autosomal recessive disease characterised by raised lysosomal levels of cystine in the cells of all organs. It is treated by regular administration of the aminothiol, cysteamine. Corneal crystal deposition is one of the most troublesome complications affecting patients and requires the hourly administration of cysteamine eye drops. In an attempt to reduce this frequency and improve the treatment, the preparation and evaluation of cysteamine containing Carbomer gel is reported. The results demonstrated that a weak gel network was formed at low shear-stress, the bioadhesion of the gel was increased with inclusion of a cysteamine derivative (e.g. mean force of 0.067N compared to 0.107N with compound included) and first-order release from the gel was observed. In conclusion these results offer the possibility to formulate cysteamine in an ocular applicable gel formulation.
authors.affiliation: School of Pharmacy and Life Sciences, The Robert Gordon University, Schoolhill, Aberdeen, UK.
authors.forename: Barbara
authors.initials: B
authors.lastname: Buchan
authors.forename: Graeme
authors.initials: G
authors.lastname: Kay
authors.forename: Anne
authors.initials: A
authors.lastname: Heneghan
authors.forename: Kerr H
authors.initials: KH
authors.lastname: Matthews
authors.forename: Donald
authors.initials: D
authors.lastname: Cairns
mesh_terms: D000180:Acrylic Resins / Q000737:chemistry*; D000268:Adhesiveness; D000818:Animals; D002417:Cattle; D003315:Cornea / Q000187:drug effects*; D003316:Corneal Diseases / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D003543:Cysteamine / Q000008:administration & dosage* / Q000737:chemistry / Q000627:therapeutic use; D003554:Cystinosis / Q000150:complications* / Q000378:metabolism; D004337:Drug Carriers / Q000737:chemistry*; D004339:Drug Compounding; D005782:Gels; D006863:Hydrogen-Ion Concentration; D066298:In Vitro Techniques; D015394:Molecular Structure; D010649:Phenylalanine / Q000737:chemistry; D012212:Rheology; D012995:Solubility
chemical_list: D000180:Acrylic Resins; D004337:Drug Carriers; D005782:Gels; D010649:Phenylalanine; C006903:carbopol 940; D003543:Cysteamine