Wagner's disease

Jump to navigation Jump to search

WikiDoc Resources for Wagner's disease

Articles

Most recent articles on Wagner's disease

Most cited articles on Wagner's disease

Review articles on Wagner's disease

Articles on Wagner's disease in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Wagner's disease

Images of Wagner's disease

Photos of Wagner's disease

Podcasts & MP3s on Wagner's disease

Videos on Wagner's disease

Evidence Based Medicine

Cochrane Collaboration on Wagner's disease

Bandolier on Wagner's disease

TRIP on Wagner's disease

Clinical Trials

Ongoing Trials on Wagner's disease at Clinical Trials.gov

Trial results on Wagner's disease

Clinical Trials on Wagner's disease at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Wagner's disease

NICE Guidance on Wagner's disease

NHS PRODIGY Guidance

FDA on Wagner's disease

CDC on Wagner's disease

Books

Books on Wagner's disease

News

Wagner's disease in the news

Be alerted to news on Wagner's disease

News trends on Wagner's disease

Commentary

Blogs on Wagner's disease

Definitions

Definitions of Wagner's disease

Patient Resources / Community

Patient resources on Wagner's disease

Discussion groups on Wagner's disease

Patient Handouts on Wagner's disease

Directions to Hospitals Treating Wagner's disease

Risk calculators and risk factors for Wagner's disease

Healthcare Provider Resources

Symptoms of Wagner's disease

Causes & Risk Factors for Wagner's disease

Diagnostic studies for Wagner's disease

Treatment of Wagner's disease

Continuing Medical Education (CME)

CME Programs on Wagner's disease

International

Wagner's disease en Espanol

Wagner's disease en Francais

Business

Wagner's disease in the Marketplace

Patents on Wagner's disease

Experimental / Informatics

List of terms related to Wagner's disease

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Wagner’s hyaloid retinal degeneration; Wagner’s vitreoretinal herdodegeneration; Wagner's syndrome.

Overview

Wagner's Disease is a familial eye disease of the connective tissue in the eye that causes blindness. Wagner's disease was originally described in 1938. This disorder is frequently confused with Stickler's syndrome, but lacks the systemic features and high incidence of retinal detachments. Inheritance is autosomal dominant.

It is a disorder involving the association of hypertension, arteriosclerosis, thickened heart muscle and severe neuroretinitis.

Historical Perspective

In 1938 Hans Wagner described 13 members of a Canton of Zurich family with a peculiar lesion of the vitreous and retina. Ten additional affected members were observed by Boehringer et al. in 1960 and 5 more by Ricci in 1961. In Holland Jansen in 1962 described 2 families with a total of 39 affected persons. Alexander and Shea in 1965 reported a family. In the last report, characteristic facies (epicanthus, broad sunken nasal bridge, receding chin) was noted. Genu valgum was present in all. In addition to typical changes in the vitreous, retinal detachment occurs in some and cataract is another complication.

Wagner's syndrome has been used as a synonym for Stickler's syndrome. Since there may be more than one type of Wagner syndrome, differentiation from Stickler's syndrome is difficult, and doctors disagree as to whether these are the same entity. It may be that Wagner has skeletal effects, but not the joint and hearing problems of Stickler's syndrome. Blair et al. in 1979 concluded that the Stickler and Wagner syndromes are the same disorder. However, retinal detachment, which is a feature of Stickler' syndrome, was not noted in any of the 28 members of the original Swiss family studied by Wagner in 1938 and later by Boehringer in 1960 and Ricci in 1961.

Pathophysiology

An exhaustive genetics study of blood from 54 patients found everyone with Wagner's disease has the same eight "markers," a genetic fingerprint that sets them apart from those with healthy eyes.

The gene involved helps regulate how the body makes collagen, a sort of chemical glue that holds tissues together in many parts of the body. This particular collagen gene only becomes active in the jelly-like material that fills the eyeball; in Wagner's disease this "vitreous" jelly grabs too tightly to the already weak retina and pulls it away.

Treatment

Most people with the disease need laser repairs to the retina, and about 60 per cent need further surgery.

References

Template:WikiDoc Sources