Recurrence of corneal dystrophy resulting from an R124H Big-h3 mutation after phototherapeutic keratectomy

Cornea. 2002 Aug;21(6):570-3. doi: 10.1097/00003226-200208000-00007.

Abstract

Purpose: The purpose of the study was to investigate the recurrence-free interval after phototherapeutic keratectomy (PTK) in patients with corneal dystrophies resulting from an Arg124His (R124H) mutation of the Big-h3 gene.

Methods: Patients with corneal dystrophy resulting from a genetically confirmed Big-h3 R124H mutation were examined with a slit lamp. The patients were divided into two groups on the basis of the mutation genotype, and the recurrence-free interval was analyzed.

Results: In the 4 eyes of 3 homozygous patients, the mean (+/- standard deviation [SD]) recurrence-free interval was 9.5 +/- 3.1 months, whereas in the 7 eyes of 4 heterozygous patients it was 38.4 +/- 6.2 months. The former interval was statistically shorter than the latter (Kaplan-Meier survival analysis with log-rank test, p = 0.004).

Conclusions: These results strongly suggest that the mutation genotype of Big-h3 gene determined the recurrence-free interval as well as the clinical picture after PTK. Therefore, PTK should be considered for patients with Big-h3 R124H corneal dystrophy, on the basis of the expected recurrence-free interval deduced from molecular analysis of the zygosity of the Big-h3 R124H mutation.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Corneal Dystrophies, Hereditary / genetics*
  • Corneal Dystrophies, Hereditary / pathology
  • Corneal Dystrophies, Hereditary / surgery
  • Corneal Opacity / genetics*
  • Corneal Opacity / pathology
  • Corneal Opacity / surgery
  • Extracellular Matrix Proteins*
  • Female
  • Heterozygote
  • Homozygote
  • Humans
  • Lasers, Excimer
  • Male
  • Middle Aged
  • Neoplasm Proteins / genetics*
  • Photorefractive Keratectomy
  • Point Mutation*
  • Recurrence
  • Time Factors
  • Transforming Growth Factor beta*
  • Visual Acuity

Substances

  • Extracellular Matrix Proteins
  • Neoplasm Proteins
  • Transforming Growth Factor beta
  • betaIG-H3 protein