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Wagner's disease

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. more...

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History

In 1938 Hans Wagner described 13 members of a Canton 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 authors 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.

Current Developments

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.

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

Also Known As

Wagner’s hyaloid retinal degeneration
Wagner’s vitreoretinal herdodegeneration

Reference Links

Wagner's disease and erosive vitreoretinopathy
University of Ottawa Eye Institute

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Brother bare: Son Frere director Patrice Chereau may be the coolest queer filmmaker you've never heard of
From Advocate, The, 4/13/04 by David Ehrenstein

"It's a dream," says Patrice Chereau of his new film Son Frere. "It's about two brothers who don't have a relationship with one another, then one becomes ill. Suddenly he discovers that he doesn't have the strength to resist and fight the disease. So he asks his brother to help him." Sounds like a standard-issue AIDS drama, doesn't it? But no, this time it's the straight brother (Bruno Todeschini) who's dying of a mysterious blood disease and the gay one (Eric Caravaca) who offers him support. The result is the simplest and most direct work by an openly gay artist long celebrated in Europe but barely known to most U.S. audiences, save for his performance as a French officer in The Last of the Mohicans.

In France, Patrice Chereau has been a name to reckon with since the '60s and '70s, when his physically forceful renditions of plays by Ibsen, Marlowe, Marivaux, and, above all, Genet's The Screens made Iris reputation in theater. Then in the 1980s his productions of Berg's Lulu and Wagner's Ring Cycle galvanized the world of opera.

But it's as a film director where Chereau has really shaken things up, with films like L'Homme Blesse, his 1983 study of a gay youth's painful coming-out; Queen Margot, his lavish version of Alexandre Dumas's violent Historical drama; and Those Who Love Me Can Take the Train, his modern classic about a funeral and its aftermath, where gay, bisexual, and transgendered characters take center stage while the straights have supporting roles. His fearlessness in diving right off into the deep end makes such seemingly radical gay filmmakers as Almodovar, Ozon, and Van Sant seem timid by comparison.

"It's a film about a man who had two families," notes Chereau, regarding Those Who Love Me, during a recent visit to Los Angeles. "There's a biological family that's not very interesting. And then there's this other family--the gay family. That's what happened to me. When I was around 16 or 20 I discovered that it was possible to have family of another kind, a family of friends.

"The thing about Son Frere is, I don't have a relationship with my older brother. The fact that I'm gay stopped everything. He would never talk about it. So we don't talk about anything anymore. If he won't talk about me and the people I love, I'm not interested in talking with him about his children--my nephews, I'm not interested in talking about his wife. Sometimes the family is the place where nobody talks."

But in Chereau's films everybody talks, and if things work out, that talk may shortly be on this side of the Atlantic. There's a film about Napoleon that Chereau may shortly direct with Al Pacino starring. "And I'm fascinated by Los Angeles," he reveals. "I'm trying to figure it out."

Ehrenstein is the author of Open Secret: Gay Hollywood, 1928-2000.

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