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Lattice corneal dystrophy type 1: an epithelial or stromal entity?
1*Department of Ophthalmology, Johannes Gutenberg University Mainz, Mainz, Germany; and †Department of Ophthalmology, University Medical Center UKS, Saarland, Germany.
Cornea
|July 24, 2014
Summary
Lattice corneal dystrophy type 1 (LCD1) involves both epithelial and stromal cells. Recurrences in grafts suggest epithelial origin, while absent lattice lines indicate stromal involvement, pointing to an epithelial-stromal disease.
Area of Science:
- Ophthalmology
- Genetics
- Corneal Diseases
Background:
- Lattice corneal dystrophy type 1 (LCD1) is a hereditary condition affecting the cornea.
- The precise origin of LCD1, whether epithelial or stromal, has been debated.
- Understanding the cellular origin is crucial for managing and potentially treating LCD1.
Observation:
- In advanced LCD1, superficial haze and stromal lattice lines are observed.
- Post-keratoplasty, corneal grafts in LCD1 patients developed diffuse opacities and erosions.
- Lattice line formation was notably absent in corneal grafts up to 18 years after surgery.
Findings:
- Genetic analysis identified mutations in the transforming growth factor beta-induced (TGFBI) gene in LCD1 patients.
- Superficial opacities in grafts are interpreted as epithelial cell products.
- The absence of lattice lines in grafts suggests they originate from keratocytes.
Implications:
- LCD1 appears to be an epithelial-stromal disease, involving both corneal epithelial cells and keratocytes.
- This understanding refines the pathophysiology of LCD1.
- Further research can explore targeted therapies based on the dual cellular origin.
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