Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2006 Jun;113(6):1010-3.
doi: 10.1016/j.ophtha.2006.02.030.

Ophthalmic manifestations of Danon disease

F Ryan Prall  1 , Arlene Drack, Matthew Taylor, Lisa Ku, Jeffrey L Olson, Darren Gregory, Luisa Mestroni, Naresh Mandava
Affiliations

Ophthalmic manifestations of Danon disease

F Ryan Prall et al. Ophthalmology. 2006 Jun.

Abstract

Purpose: To describe the ophthalmic findings in patients with Danon disease, an X-linked condition causing cardiomyopathy in males and females.

Design: Retrospective case series.

Participants: Patients with genetically proven Danon disease.

Methods: Retrospective chart review of complete eye examinations including electroretinogram, visual fields, and fluorescein angiography.

Results: Five females (4 affected) and 2 affected males were examined. The 4 affected females demonstrated a peripheral pigmentary retinopathy. Lens changes, myopia, abnormal electroretinogram and visual fields were also found. The males demonstrated a near-complete loss of pigment in the retinal pigment epithelium.

Conclusion: We report the first description of a characteristic retinopathy in patients with Danon disease and the first extracardiac manifestations in affected females. Retinopathy potentially could be used to identify asymptomatic carriers.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

MeSH terms

Substances