Chronic clinical course of two patients with severe corneal dystrophy caused by homozygous R124H mutations in the βig-h3 gene

2000 ◽  
Vol 129 (5) ◽  
pp. 663-665 ◽  
Author(s):  
Yuichi Kaji ◽  
Shiro Amano ◽  
Tetsuro Oshika ◽  
Tomohiko Usui ◽  
Mayumi Kitagawa ◽  
...  
2021 ◽  
Author(s):  
Maximiliano Barrera-Sanchez ◽  
Julio C. Hernandez-Camarena ◽  
Raul E. Ruiz-Lozano ◽  
Jorge E. Valdez-Garcia ◽  
Alejandro Rodriguez-Garcia

Abstract Purpose To describe the demographic characteristics and clinical course of Fuchs endothelial corneal dystrophy (FECD) in a Mexican-mestizo population. Methods A retrospective observational and longitudinal study was performed in consecutive patients with the clinical diagnosis of Fuchs endothelial corneal dystrophy seen at our institution. Initial and last follow-up best corrected visual acuity, slit-lamp findings, and specular microscopy endothelial morphometric parameters were analyzed. Results One hundred and two eyes belonging to 51 patients were included in the analysis. Median age at the time of diagnosis was 69 years (range, 25–87 years) with a female-to-male ratio of 3.3:1. Visual loss (59.8%) and fluctuating matutine vision (7.8%) were the most common complaints at presentation. Regarding FECD staging, 65 (63.7%) were classified as stage-I FECD, 21 (20.6%) stage-II, and 15 (14.7%) as stage-III. A high percentage of eyes (44%) presented visual impairment (≤ 20/40) at presentation, and the presence of isolated corneal guttata was the most common stage of presentation (64%) at slit-lamp examination. Fifty-nine (57.8%) eyes did not require any medical or surgical management, and 17% of the evaluated eyes finally required a corneal transplantation, being penetrating keratoplasty with 8 (44.4%) eyes the most frequent technique performed. Conclusion Demographical characteristics of Fuchs dystrophy regarding age at presentation, gender distribution, and clinical stage at the time of diagnosis did not differ significantly from other international reports. Almost 20% of these patients will require keratoplasty during the disease, emphasizing the need for safer and more reproducible keratoplasty techniques.


Author(s):  
Maximiliano Barrera-Sanchez ◽  
Julio C. Hernandez-Camarena ◽  
Raul E. Ruiz-Lozano ◽  
Jorge E. Valdez-Garcia ◽  
Alejandro Rodriguez-Garcia

2005 ◽  
Vol 173 (4S) ◽  
pp. 21-21
Author(s):  
Frank Christoph ◽  
Steffen Weikert ◽  
Markus Müller ◽  
Kurt Miller

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