A Comparative Pharmacokinetic Study for Cysteamine-Containing Eye Drops as an Orphan Topical Therapy in Cystinosis.

Cystinosis is a low-prevalence lysosomal storage disease. The pathomechanism involves abnormal functioning of the cystinosine lysosomal cystine transporter (CTNS), causing intraliposomal accumulation of the amino acid cysteine disulfide, which crystallizes and deposits in several parts of the body. The most common ophthalmic complication of cystinosis is the deposition of "gold dust" cystine crystals on the cornea, which already occurs in infancy and leads to severe photosensitivity and dry eyes as it gradually progresses with age.

Zinc sulphate release and morphology of matrices prepared for the individual therapy of Wilson's disease.

Hydrophobic zinc sulphate wax matrices with different drug loadings were prepared for the individual hospital therapy of Wilson's disease. The drug release parameters, scanning electron microscopy (SEM) and energy dispersive X-ray spectroscopy (EDS) of the samples were analysed. The release mechanisms from matrices of 75% and 80% w/w zinc sulphate loadings were described with good correlation by the semi-empirical Fikkian diffusion based release model.