Cystinosis is a rare genetic disorder characterized by the accumulation of cystine crystals in different tissues and organs. Although renal damage prevails during initial stages, the deposition of cystine crystals in the cornea causes severe ocular manifestations. At present, cysteamine is the only topical effective treatment for ocular cystinosis. The lack of investment by the pharmaceutical industry, together with the limited stability of cysteamine, make it available only as two marketed presentations (Cystaran® and Cystadrops®) and as compounding formulations prepared in pharmacy departments. Even so, new drug delivery systems (DDSs) need to be developed, allowing more comfortable dosage schedules that favor patient adherence. In the last decades, different research groups have focused on the development of hydrogels, nanowafers and contact lenses, allowing a sustained cysteamine release. In parallel, different determination methods and strategies to increase the stability of the formulations have also been developed. This comprehensive review aims to compile all the challenges and advances related to new cysteamine DDSs, analytical determination methods, and possible future therapeutic alternatives for treating cystinosis.

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RESUMENObjetivo: Determinar la relación entre los diferentes componentes ópticos oculares y la emetropización en función de la longitud axial (LA). Métodos: Participaron 109 jóvenes universitarios divididos en cinco grupos según el error refractivo: emétropes, hipermétropes, miopes bajos, miopes moderados y miopes altos. La medida de los pará-metros intraoculares y el análisis de la topografía corneal se realizó mediante ultrasonografía y videoqueratoscopia respectivamente. Resultados: Se encontró una correlación estadísti-camente significativa entre la profundidad de la cámara anterior (PCA) y la LA en ojos con LA menor de 24 mm (r = 0,441; p < 0,001). Sin embargo en ojos con LA mayor no se encontró tal relación (r = 0,098; p = 0,527). El espesor del cristalino (EC) mostró una correlación inversa con la LA para los ojos con LA < 24 mm (r = 0,391; p < 0,001), siendo nula en el caso de ojos con LA ≥ 24 ARTÍCULO ORIGINAL ABSTRACT Purpose: To report the relationship between different optical ocular components and the influence of axial length on emmetropization. Methods: 109 young university students, divided into five groups, were enrolled in this study: emmetropes, hyperopes, low myopes, moderate myopes and high myopes. Intraocular parameters and topographic corneal analyses were performed by ultrasonography and videokeratoscopy respectively. Results: Anterior chamber depth and axial length were found to correlate significantly in eyes with axial lengths less than 24 mm (r = 0.441; p < 0.001) However this correlation was not found in eyes with longer axial lengths (r = 0.098; p = 0.527). Lens thickness showed an inverse correlation with axial length for shorter eyes (r = 0.391; p < 0.001), whereas any correlation in longer eyes was associated with moderate to high levels of myopia. Anterior corneal curvature only correlated, although weakly,

RESUMENObjetivo: La topografía y espesor de la córnea tienen una gran importancia clínica en la adaptación de lentes de contacto y en cirugía refractiva. Sin embargo, la repetibilidad en las medidas de estos parámetros se ve comprometida por el fenómeno natural de las variaciones diurnas. Nuestro objetivo es determinar las variaciones diurnas de espesor y curvatura corneal central y paracentral durante un periodo de 10 horas. Material y método: Se determinó la curvatura y espesor corneal en los ojos derechos de 10 varones jóvenes mediante Orbscan y videoqueratoscopio respectivamente. Ambos parámetros fueron determinados para la zona central y paracentral a 1 y 2 mm del centro de la córnea, cada dos horas durante un periodo de 10 horas. Resultados: La córnea presentaba su espesor máxi-mo y curvatura mínima al abrir el ojo. Se observó una variación estadísticamente significativa de espesor (ANOVA, método de Schefféc p< 0,05) y de curvatura (ANOVA, método de Tamhane p < 0,05) a lo largo del día en todas las localizaciones estudiadas, si bien la variación y los rangos de valores en la zona paracentral eran mayores. Los valo-ARTÍCULO ORIGINAL ABSTRACT Objective: Corneal topography and thickness has clinical importance in contact lens fitting and refractive surgery, however repeated measurement of corneal thickness and curvature is complicated by the natural phenomenon of diurnal variation. Our aim was determine diurnal variations of central and paracentral corneal thickness and curvature over a period of ten hours. Materials and methods: Corneal thickness and curvature of 10 right eyes of 10 young healthy men were determined by Orbscan Topography System and EyeSys videokeratoscope respectively. Both parameters were determined for central and paracentral regions 1 mm and 2 mm from the centre of the cornea at 2-hour internals for ten hours. Results: The cornea was thickest and flattest on awakening. There was a difference in corneal thickness (ANOVA, Schfféc method p< 0.05) and curvature (ANOVA, Tamhane method p< 0.05) over time for all the corneal locations studied, with greater changes observed in the peripheral corneal data. The minimum value was 10 and 8 hours after eye opening for central and paracentral zones, respectively. Change in central and paracentral corneal