Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Experiment Videos

Contrast sensitivity function in nephropathic cystinosis.

B Katz1, R B Melles, J A Schneider

  • 1Department of Ophthalmology, University of California, San Diego, La Jolla.

Archives of Ophthalmology (Chicago, Ill. : 1960)
|December 1, 1987
PubMed
Summary
This summary is machine-generated.

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Microbial Indicators of Dental Health, Dysbiosis, and Early Childhood Caries.

Journal of dental research·2023
Same author

Therapeutic drug monitoring and TB treatment outcomes in patients with diabetes mellitus.

The international journal of tuberculosis and lung disease : the official journal of the International Union against Tuberculosis and Lung Disease·2023
Same author

Improving Caries Risk Prediction Modeling: A Call for Action.

Journal of dental research·2020
Same author

Maturation of the Oral Microbiome in Caries-Free Toddlers: A Longitudinal Study.

Journal of dental research·2019
Same author

Biomarkers of inflammation - LBP and TLR- predict progression of knee osteoarthritis in the DOXY clinical trial.

Osteoarthritis and cartilage·2018
Same author

Fluoride Use in Health Care Settings: Association with Children's Caries Risk.

Advances in dental research·2018

Patients with infantile-onset nephropathic cystinosis exhibit significantly reduced spatial contrast sensitivity, particularly at higher frequencies. This visual impairment is likely linked to corneal disease, with potential contributions from retinal and central nervous system issues.

Area of Science:

  • Ophthalmology
  • Genetics
  • Metabolic Disorders

Background:

  • Cystinosis is a rare autosomal recessive metabolic disorder characterized by lysosomal cystine accumulation.
  • Ocular manifestations include iridescent crystals and pigmentary retinopathy.
  • Infantile-onset nephropathic cystinosis is the most severe form.

Purpose of the Study:

  • To measure and compare spatial contrast sensitivity in patients with infantile-onset nephropathic cystinosis and healthy controls.
  • To investigate the impact of cystinosis on visual function.

Main Methods:

  • Spatial contrast sensitivity function was measured in seven patients with infantile-onset nephropathic cystinosis.
  • Data were compared with ten age-matched healthy controls.

Related Experiment Videos

Main Results:

  • Patients with cystinosis showed significantly lower spatial contrast sensitivity compared to controls.
  • The loss of contrast sensitivity was more pronounced at higher spatial frequencies in the patient group.
  • Corneal disease is speculated as the primary cause, with secondary retinal and CNS involvement.

Conclusions:

  • Spatial contrast sensitivity is impaired in infantile-onset nephropathic cystinosis.
  • Visual dysfunction in cystinosis may stem from ocular and central nervous system abnormalities.