Gelatinous drop-like corneal dystrophy is a rare disease, described for the first time by Nakaizumi (Japan) in 1914. We report 5 cases of this primary corneal amyloidosis. Three of our patients are brothers, the two other cases are isolated cases. Photophobia and progressive loss of vision are the first presenting signs. Corneal lesions are typically bilateral white nodular deposits beneath the epithelium. Patients underwent repeated keratoplasties with a mean interval of 5 years because of the recurrence of the disease on the corneal graft. Diagnosis of corneal amyloidosis was confirmed in all cases by histology. We compared our results with the data reported in the literature.
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J Fr Ophtalmol
October 2024
Department of Ophthalmology, Guangxi University of Chinese Medicine, Nanning, China; Department of Ophthalmology, Jingliang Eye Hospital, Guangxi Medical University, Nanning, China. Electronic address:
Cornea
November 2024
Department of Ophthalmology, Saarland University Medical Center, Homburg/Saar, Germany.
Purpose: To present ocular clinical, histological, systemic, and genetic findings of a patient with familial lecithin-cholesterol acyltransferase (LCAT) deficiency caused by a novel genetic variant of the LCAT gene associated with secondary corneal amyloidosis.
Methods: Case report.
Results: A 74-year-old woman presented with decreased visual acuity (VA), sensitivity to light, and progressive whitening of both corneas for approximately 20 years.
Eur J Ophthalmol
July 2024
Department of Ophthalmology, Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Rio de Janeiro, Brazil.
Introduction: Genetic mutations or inflammatory, degenerative, or neoplastic conditions can trigger amyloidosis. Hereditary gelsolin amyloidosis is a genetic disorder primarily marked by amyloid fibrils composed of misfolded gelsolin fragments.
Case Report: We present three sisters with AGel amyloidosis, illustrating its clinical diversity.
Indian J Ophthalmol
June 2023
Gurunanak Eye Centre, Maulana Azad Medical College, New Delhi, India.
Purpose: To evaluate the effectiveness of repeat deep anterior lamellar keratoplasty (DALK) in patients of previous failed DALK.
Methods: : A retrospective analysis of records of seven patients who had undergone repeat DALK following the failure of the primary DALK was done. The indications for repeat surgery, time elapsed since the first surgery, and pre- & postoperative best-corrected visual acuity (BCVA) were noted for all the patients.
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