Evolution of ocular manifestations in XXXX : a long-term study of a population treated with @entity8488 .

@entity1 @entity227 @entity267 @entity8488 @entity8518 @entity1434 @entity2687 @entity989 @entity1020 @entity3917
BACKGROUND: @entity8518 is characterized by an accumulation of @entity1434 crystals within most body tissues. Renal transplantation and oral @entity8488 have improved the general prognosis of the disease, and ocular manifestations are now the most common complication. This long-term follow-up study describes the sequence of ocular manifestations in @entity1 with @entity8518 treated with oral and topical @entity8488 . METHODS: Data were recorded for all @entity1 with @entity8518 examined between 1980 and 2000. For each @entity1 , @entity3917 and @entity227 were evaluated and slit-lamp and fundus examinations were performed. For some @entity1 , an electroretinogram was also performed. RESULTS: Twenty-nine @entity1 were observed during this period. They received oral and topical @entity8488 . Photophobia and loss of @entity227 generally began by 10 years of age but were severe only after 15 years of age. Peripheral corneal epithelial infiltration appeared in the first few years of life. Infiltration evolved toward the depth and center of the @entity267 during the second decade of life. @entity989 was present in 51.7% of the @entity1 , with 3 cases of @entity1020 and 3 cases of flattening on electroretinogram. CONCLUSIONS: Photophobia and @entity2687 , although generally severe after 15 years of age, could be treated with topical @entity8488 and corneal transplantation. Retinal infiltration, previously described as frequent and potentially blinding, is currently observed in only half of these @entity1 , with mild @entity227 . This could be related to the treatment with oral @entity8488 reaching the retinal vascularization.
