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S. Choi, E.–K. Kim, J.–W. Moon, J.–H. Lee, H.–K. Lee; The Clinical Features of Homozygous Granular Corneal Dystrophy Type II (Avellino Corneal Dystrophy) With Age and After Conventional Treatments . Invest. Ophthalmol. Vis. Sci. 2006;47(13):5904.
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To determine the clinical features of homozygous granular corneal dystrophy type II (GCDII) with age and with several kinds of treatment in eighteen homozygous patients in several different conditions.
Eighteen homozygous GCDII patients of thirteen families comfirmed with DNA analysis were enrolled. Their clinical features including age at detection of deposits by parents, best–corrected visual acuity and disease progression were evaluated. We also investigated the clinical outcome and the recurrence of dystrophy after surgery for those twelve homozygous GCDII patients who had undergone phototherapeutic keratectomies or various keratoplasties.
The deposits could be found by the parents at a very early age, for the most part, 3 to 4 years. Furthermore, the dystrophic process progressed rapidly with age and the vision decreased to hand movement (HM) if not treated in virgin homozygous patients. All of the patients who had undergone surgeries acquired better vision right after surgery. Corneal deposits, however, reappeared after certain period of time and the disease showed unusually early and severe recurrence as the treatments were repeated.
The clinical features of homozygous GCDII are characterized by a severe granular type of corneal dystrophy, early onset, rapid progression, and early and severe recurrence after surgical treatment.
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