Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"


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Abstract
Abstract

Limbal Insufficiency Associated with KID-Syndrome

Messmer E. M.1, Rittinger O.2, Janecke A.3, Kenyon K. R.4, Kampik A.1
1Augenklinik, Ludwig-Maximillian-Universität, München; 2St. Johanns-Spital, Landesklinik für Kinder- und Jugendheilkunde, Salzburg/A; 3Institut für Med. Biologie und Humangenetik, Innsbruck/A; 4Cornea Consultants, Boston/USA

Purpose: The keratitis, ichtyosis, and deafness (KID) syndrome is a rare congenital disorder of the ectoderm characterized by diffuse hyperkeratotic erythroderma, keratitis with vascularisation of the cornea, and severe neurosensory hearing loss. Heterozygous missense mutations in the connexin-26 gene, GjB2, have been described in a number of KID-patients. We report on a 12-year old boy with proven KID-syndrome and bilateral limbal insufficiency.
Method: Our patient suffers from profound sensorineural hearing loss, cubital contractures, palmoplantar hyperkeratosis, depigmented scanty hair, dystrophic toenails and non-inflammatory papules of the skin. He presented at our hospital at the age of 8 because of bilateral corneal conjunctivalization extending paracentrally with recurrent corneal erosions. His visual acuity was 0.1 in both eyes. Due to the progressive nature of the disease bilateral keratectomy, limbal transplantation from die HLA-related parents, amnion transplantation and temporal tarsorrhaphy were performed. A systemic immunosuppression with cyclosporine A was instituted. Seven months later pannus formation again re


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