A patient with hereditary Tyrosinemia Type I who is treated with nitisinone may develop corneal deposits caused by the deposition of tyrosine crystals.
Patient selection: hereditary tyrosinemia type I treated with nitisinone (which blocks the tyrosine catabolic pathway)
The corneal deposits tend to appear when the blood tyrosine concentration is elevated (often > 600 micromol/L). This usually occurs when the patient does not restrict tyrosine intake. The deposits disappear when blood tyrosine levels are reduced.
(2) itchiness or discomfort of the eyes
(3) bilateral, linear branching subepithelial corneal opacities
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