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Clinical course of six cases of gelatinous drop-like corneal dystrophy for twenty years and over Yuta Nochi 1 , Masahiro Yamaguchi 1 , Satoru Nakatani 1 , Toshinari Funaki 1,2 , Akira Matsuda 1 , Akira Murakami 1 1Department of Ophthalmology, Juntendo University Graduate School of Medicine 2Department of Ophthalmology, Japan Red Cross Medical Center pp.971-976
Published Date 2020/8/15
DOI https://doi.org/10.11477/mf.1410213642
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Abstract Object:Gelatinous drop-like corneal dystrophy(GDLD)is a rare autosomal recessive disorder, clinically characterized by grayish corneal deposits of amyloid which require corneal transplantation. We report six cases of long term prognosis over 20 years.

Methods:This was a retrospective, observational case series, including 6 patients(12 eyes)with GDLD treated in Juntendo University Hospital over 20 years. Age, observation period, visual acuity(logMAR units), number of corneal transplantations, and severity were evaluated. Japanese domestic classification was used to assess severity.

Results:This study involved 12 eyes of 6 patients(mean age:65.5±10.1 years;range:47-77 years). Mean age and visual acuity in first visit was 24.0±7.5 years(14〜33 years), and 1.79(0.52〜2.0), respectively. Mean follow-up period was 39.8±7.3 years(range:28-48). Corneal transplantation was performed 4.3±2.2 times(2〜9)in each eye. Mean visual acuity in last visit was 1.57, included two eyes of lost light perception, caused by retinal detachment or glaucoma. Severity were classified in a case with class Ⅱ, a case with class Ⅲ, 4 cases with class Ⅳ.

Conclusions:Visual acuity was severely reduced in most cases from first visit, and did not improve during the observation period. Most cases of GDLD were juvenile-onset and required corneal transplantation repeatedly.


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