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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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Related Experiment Video

Updated: Jan 26, 2026

Generation of Induced Pluripotent Stem Cells from Turner Syndrome 45XO Fetal Cells for Downstream Modelling of Neurological Deficits Associated with the Syndrome
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Orbscan mapping in Ehlers-Danlos syndrome.

Konrad Pesudovs1

  • 1Ultralase, Leeds, United Kingdom. konrad@pesudovs.com

Journal of Cataract and Refractive Surgery
|August 18, 2004
PubMed
Summary

Patients with Ehlers-Danlos syndrome (EDS) have thin corneas but may not develop keratoconus. Advanced corneal mapping offers insights into EDS-related corneal changes.

Area of Science:

  • Ophthalmology
  • Genetics
  • Biomedical Engineering

Background:

  • Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
  • Classic (type I) EDS is characterized by skin hyperextensibility, joint hypermobility, and tissue fragility.
  • Corneal abnormalities, including thinning and keratoconus, can occur in EDS patients.

Observation:

  • A patient with classic EDS presented with blue sclera, myopia, astigmatism, and significant limbus-to-limbus corneal thinning.
  • Orbscan pachymetry mapping detailed central corneal thickness (360µm OD, 383µm OS) and midperipheral thinning.
  • Despite corneal thinning and presumed biomechanical weakness, regular corneal topography was maintained.

Findings:

  • The study highlights a case of classic EDS with severe corneal thinning but without keratoconus.

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  • Orbscan imaging effectively visualized the extent of limbus-to-limbus corneal thinning.
  • Corneal biomechanical integrity may be preserved to some extent even with significant thinning in EDS.

    • Implications:

    • Laser refractive surgery remains contraindicated in all types of EDS due to biomechanical risks.
    • Advanced corneal topography and pachymetry, like Orbscan, are valuable tools for assessing corneal structure in EDS.
    • Understanding corneal morphology in EDS is crucial for managing ocular health and surgical candidacy.