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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

Read more at Wikipedia.org


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Portrait of the artist as an antisemite: many highly regarded writers and artists held deep antipathy towards Jews and incorporated anti-Jewish themes
From New Internationalist, 10/1/04

DESPITE his belief in 'redemption' and 'Christian love', Fyodor Dostoevsky developed fervent antisemitic views that were explicitly revealed in his popular diary but also in his later masterpieces such as the House of the Dead and The Brothers Karamazov. These works draw upon negative stereotypes of Jews as moneylenders, opportunists and deceivers: Old Karamazov 'made the acquaintance at first, in his own words, of a lot of low Jews, Jewesses and Jewkins ... and developed a peculiar faculty for making and hoarding money.' He also promoted the myth of Jewish blood rituals. Mikhail Bulgakov's classic. The Master and Margarita, plays on a number of fantasies of clandestine Jewish power, conspiracy and treachery. Such themes were a major current of Russian mystical literature from the early 20th century, coinciding with the publication of the Czarist hoax, The Protocols of the Elders of Zion.

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Geoffrey Chaucer's The Prioress' Tale, one of the celebrated Canterbury Tales, indulges in rhapsodic Jew-hatred in its retelling of the classic blood-libel charge of Jews murdering Christian children. Shakespeare's villain, Shylock, in The Merchant of Venice helped give credence to 16th-century antisemitism. Shylock's infamous demand for a 'pound of flesh' has served to perpetuate a combination of traditional fantasies of Jewish ritual killing and usury. Shylock would come to typify the 'stage Jew'. Christopher Marlowe's depiction of Barabas in the Jew of Malta, is similarly loaded with antisemitic confectionery. Although Dickens denied Judeophobic influences in his portrait of Fagin in Oliver Twist, the characterization is deeply disturbing. He is ugly, twisted, greedy and constantly referred to as 'the Jew'. He is a resonant embodiment of terrifying evil and villainy, which would influence the representation of the Child-Catcher (replete with huge nose and dark Orthodox-style clothing and hat) in the Roald Dahl story-turned-film Chitty Chitty Bang Bang. Dahl once defended himself against accusations of antisemitism by saying: 'Even a stinker like Hitler didn't just pick on them for no reason.'

HG Wells described Zionism in The Anatomy of Frustration as 'an expression of Jewish refusal to assimilate. If Jews have suffered it is because they have regarded themselves as a chosen people.' He later wrote: 'A careful study of antisemitism prejudice and accusations might be of great value to many Jews, who do not adequately realize the irritations they inflict.'

Mark Twain wrote of Jews as 'simple, superstitious, disease-tortured creatures' who could only understand a transcendental idea 'if it was written on their skins'. TS Eliot and Ezra Pound drank from the same racist wellspring. Eliot wove hateful antisemitic skeins into his work even after the Second World War. In Dirge, verses that would be excluded from The Waste Land, he revealed his low esteem of Jews: 'The rats are underneath the piles! The Jew is underneath the lot.' He often used 'the Jew' as a symbol of the decay of Western Culture, one who: '... squats on the window sill / the owner / Spawned in some estaminet of Antwerp / Blistered in Brussels / patched and peeled in London'. At a time when persecutions in Nazi Germany were under way, Eliot claimed that 'reasons of race and religion combine to make any large number of free-thinking Jews undesirable'. Pound's close friend Ernest Hemingway was a far-out Jew-hater who ranted about 'kikes' in his letters. This often translated into less than flattering 'Jew' characters such as Robert Cohn in The Sun Also Rises and self-indulgent tirades against Jews in stories such as Fifty Grand.

In the world of German culture, few have had as much impact as Richard Wagner. The renowned 19th-century composer, music theorist and essayist, who broke traditional conventions and developed new dramatic forms of opera will be infamously remembered as Hitler's favourite composer. An association captured by Woody Allen: 'I can't listen to that much Wagner. I start getting the urge to conquer Poland.' Wagner's frothingly antisemitic tract Judaism in Music described Jews as freaks of nature: 'The Jew ... no matter to what European nationality we belong has something disagreeably foreign to that nationality: instinctively we wish to have nothing in common with a man who looks like that.' As a representative of 'high culture', Wagner gave Jew-hatred respectability.

Modern cultural representations of Jews, in the West at least, have improved dramatically since the Second World War. Yet some old stereotypes continue to hold currency, such as George Lucas' portrayal of the bearded, miserly, slave-owning mercantile character of Watto in the latest Star Wars films replete with hooknose, beady eyes, black hat and coarse gravelly Yiddish accent. A caricature that would not be out of place in the pages of the Nazi propaganda rag Der Sturmer.

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COPYRIGHT 2004 New Internationalist Magazine
COPYRIGHT 2004 Gale Group

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