Document Detail

Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea.
MedLine Citation:
PMID:  7831029     Owner:  NLM     Status:  MEDLINE    
PURPOSE: To investigate the clinical course of patients with familial subepithelial amyloidosis of the cornea (FSA). METHODS: The authors retrospectively investigated the clinical course of seven Japanese patients with FSA. Corneal specimens obtained at the time of keratoplasty were examined histopathologically. RESULTS: The mean follow-up period was 20.6 years, including four patients followed for more than 25 years. In all patients, the initial symptoms of photophobia and epiphora started in the first decade of life. All except one patient had their first keratoplasty before 30 years of age. The seven Japanese patients with FSA had a total of 35 keratoplasties, each of which was followed by a severe recurrence of disease. In each patient, subepithelial haziness developed in the graft within 1 year of keratoplasty (mean, 7.9 months). Amyloid deposition typically recurred within a few years (mean, 26.6 months) followed by a deterioration of vision. There was a high incidence of postkeratoplasty complications such as wound dehiscence, glaucoma, and cataract. Histopathologic findings demonstrated that, in the early phase of recurrence, amyloid was deposited between the basal cell of the epithelium and Bowman's layer. CONCLUSION: Patients with FSA have ocular symptoms with a deterioration in vision from an early decade of life. Conventional surgical approaches were complicated by subepithelial haziness in the first postoperative year, which was followed by a severe recurrence in each patient. New surgical approaches may be indicated in FSA.
J Shimazaki; T Hida; M Inoue; H Saito; K Tsubota
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Ophthalmology     Volume:  102     ISSN:  0161-6420     ISO Abbreviation:  Ophthalmology     Publication Date:  1995 Jan 
Date Detail:
Created Date:  1995-02-17     Completed Date:  1995-02-17     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7802443     Medline TA:  Ophthalmology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  139-44     Citation Subset:  IM    
Department of Ophthalmology, Tokyo Dental College, Chiba, Japan.
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MeSH Terms
Amyloidosis / pathology*,  physiopathology,  surgery*
Corneal Diseases / pathology*,  physiopathology,  surgery*
Corneal Transplantation
Follow-Up Studies
Middle Aged
Postoperative Complications
Retrospective Studies
Time Factors

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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