logo BDSP

Base documentaire


  1. Macular corneal dystrophy in Iceland : A clinical, genealogic and immunohistochemical study of 28 patients.

    Article - En anglais

    Background 

    The frequency of different types of macular corneal dystrophy (MCD) was determined in Iceland where MCD accounts for one third of every penetrating keratoplasty.

    Methods 

    The authors determined the serum levels of antigenic keratan sulfate (aKS) in 27 patients with MCD and 53 unaffected family members by an enzyme-linked immunosorbent assay that uses an anti-KS monoclonal antibody (5-D-4).

    The authors also stained sections from 37 corneal buttons (including 2 regrafts) from 23 patients with MCD by the avidin-biotin complex method using the same anti-KS monoclonal antibody.

    Results 

    Based on the serum analyses, 22 patients had MCD type 1 and 5 had MCD type II.

    The corneas from patients without detectable KS in the serum lacked immunohistochemical reactivity to the anti-KS antibody.

    Every MCD cornea examined from individuals with normal serum KS levels showed KS reactivity.

    All 53 unaffected siblings and parents carrying the recessive gene had normal serum KS levels.

    Conclusions 

    Macular corneal dystrophy types I (78.6%) and II (21.4%) both occur in Iceland.

    Members of affected sibships had only one of these types, not both.

    Nine patients with MCD type I and four persons with MCD type II belonged to a large pedigree in which individuals have been traced as far back as the beginning of the 16th century. (...)

    Mots-clés Pascal : Dystrophie cornéenne, Macula, Islande, Iles Atlantiques, Immunohistochimie, Symptomatologie, Généalogie, Homme, Oeil pathologie, Kératopathie, Anatomopathologie

    Mots-clés Pascal anglais : Corneal dystrophy, Macula, Iceland, Atlantic Ocean Islands, Immunohistochemistry, Symptomatology, Genealogy, Human, Eye disease, Keratopathy, Pathology

    Logo du centre Notice produite par :
    Inist-CNRS - Institut de l'Information Scientifique et Technique

    Cote : 96-0351804

    Code Inist : 002B09E. Création : 10/04/1997.