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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...

Waardenburg syndrome
Wagner's disease
WAGR syndrome
Wallerian degeneration
Warkany syndrome
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Wegener's granulomatosis
Weissenbacher Zweymuller...
Werdnig-Hoffmann disease
Werner's syndrome
Whipple disease
Whooping cough
Willebrand disease
Willebrand disease, acquired
Williams syndrome
Wilms tumor-aniridia...
Wilms' tumor
Wilson's disease
Wiskott-Aldrich syndrome
Wolf-Hirschhorn syndrome
Wolfram syndrome
Wolman disease
Wooly hair syndrome
Worster-Drought syndrome
Writer's cramp


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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Methicillin-resistant Staphylococcus aureus toxic shock syndrome
From Emerging Infectious Diseases, 4/1/05 by Sophie Jamart

To the Editor: Toxic shock syndrome (TSS), which can be life threatening, is defined by clinical and laboratory evidence of fever, rash, desquamation, hypotension, and multiple organ failure caused by Staphylococcus aureus toxins. TSS caused by methicillin-resistant S. aureus (MRSA) strains has been found extensively in Japan (1), rarely in the United States (2), and, thus far, not in Europe.

We report a case of TSS due to an MRSA strain that produced a TSS toxin 1 (TSST-1). A 54-year-old woman was admitted to the emergency ward of Brugmann University Hospital, Brussels, with a 2-day history of myalgia, diarrhea, and vomiting. She had undergone surgery for a palate neoplasia 2 months earlier, and again 2 weeks earlier, in another hospital. After the second operation, she had been treated for a local scar infection with amoxicillin-clavulanic acid for 1 week.

On physical examination, the patient was conscious, tachypneic, pale, and sweating. Her temperature was 38.2[degrees]C and her blood pressure was 70/50 mm Hg. Abdominal examination findings were normal. The cutaneous operative wound was red and swollen. Laboratory results included the following: leukocyte count 19,830/[mm.sup.3] with 97% polynuclear neutrophils, platelets 90,000/[mm.sup.3], creatinine 2.1 mg/dL, bicarbonate 13 mEq/L, cyclic AMP receptor protein 43.7 ng/mL, creatine kinase 514 U/L. Cultures of blood, stool, and urine samples were negative for microbial agents. Puncture of the wound released 12 mL of pus; culture of the pus sample yielded an MRSA strain harboring a TSST-1 gene, detected by multiplex polymerase chain reaction as previously described (3).

By molecular typing, the strain belonged to the epidemic MRSA pulsed-field gel electrophoresis clone G10 and carried the staphylococcal chromosome cassette mec (SCCmec) type II. This clone belongs to the sequence type (ST) 5-SCCmec II clone, formerly named "NewYork/Japan clone," which has been associated with neonatal TSS-like exanthematous disease in Japanese hospitals (4-6). This epidemic clone, which is widely disseminated in the United States, Japan, and Europe, has been found in 12% of Belgian hospitals during a national survey conducted in 2001 (6).

The treatment included aggressive intravenous fluid resuscitation, administration of dopamine, and antimicrobial agent therapy with teicoplamin and clindamycin. The treatment outcome was favorable. On the second day, a diffuse cutaneous macular rash appeared. The acute renal failure and the biological abnormalities resolved. On the fifth day, the patient was transferred back to the hospital where she had undergone surgery; extensive peeling then developed on both of the patient's hands.

Our patient met the criteria of T SS: she had fever, rash, desquamation, hypotension, vomiting, diarrhea, myalgias, elevated creatine kinase, acute renal failure, and thrombocytopenia. The diagnosis of staphylococcal TSS was confirmed by bacteriologic results.

Although TSST-1 production by MRSA strains has been described in Europe (7), this case is the first of TSS due to TSST-l-producing MRSA in Europe. Recently Nathalie van der Mee-Marquet et al. (8) described the first case of neonatal TSS-like exanthematous disease due to a MRSA strain containing the TSST-1 gene in Europe. They emphasized the risk of emergence of neonatal toxic shock syndrome-like exanthematous disease outside Japan.

We would also like to emphasize the rising risk of TSS due to virulent MRSA strains outside Japan and particularly in Europe. The usual recommendations for the treatment of staphylococcal TSS do not consider this possibility and consist of a [beta]-lactamase-resistant anti-staphylococcal agent and clindamycin in some cases (to decrease the synthesis of TSST-1) (9-11).

We immediately treated our patient with teicoplanin and clindamycin because we suspected a nosocomial infection with S. aureus, possibly MRSA. The possibility of MRSA must be considered when initiating antimicrobial agents to treat TSS.


(1.) Furukawa Y, Segawa Y, Masuda K, Takahashi M, Ootsuka A, Hirai K, et al. Clinical experience of 3 cases of toxic shock syndrome caused by methicillin cephem-resistant Staphylococcus aureus (MRSA). Kansenshogaku Zasshi. 1986; 60:1147-53.

(2.) Meyer RD, Monday SR, Bohach GA, Schlievert PM. Prolonged course of toxic shock syndrome associated with methicillin-resistant Staphylococcus aureus enterotoxins G and I. Int J Infect Dis. 2001;5:163-6.

(3.) Lina G, Piemont Y, Godail-Gamot F, Bes M, Peter MO, Gauduchon V, et al. Involvement of Panton-Valentine leukocidin-producing Staphylococcus aureus in primary skin infections and pneumonia. Clin Infect Dis. 1999;29:1128-32.

(4.) Oliveira DC, Tomasz A, de Lencastre H. Secrets of success of a human pathogen: molecular evolution of pandemic clones of methicillin-resistant Staphylococcus aureus. Lancet Infect Dis. 2002;2:180-9.

(5.) Kikuchi K, Takahashi N, Piao C, Totsuka K, Nishida H, Uchiyama T. Molecular epidemiology of methicillin-resistant Staphylococcus aureus strains causing neonatal toxic shock syndrome-like exanthematous disease in neonatal and perinatal wards. J Clin Microbiol. 2003;41:3001-6.

(6.) Denis O, Deplano A, Nonhoff C, De Ryck R, de Mendonca R, Rottiers S, et al. National surveillance of methicillin-resistant Staphylococcus aureus (MRSA) in Belgian hospitals in 2001 indicates rapid diversification of epidemic clones. Antimicrob Agents Chemother. 2004;48: 3625-9.

(7.) Schmitz FJ, MacKenzie CR, Geisel R, Wagner S, Idel H, Verhoef J, et al. Enterotoxin and toxic shock syndrome toxin-I production of methicillin resistant and methicillin sensitive Staphylococcus aureus strains. Eur J Epidemiol. 1997;13: 699-708.

(8.) van der Mee-Marquet N, Lina G, Quentin R, Yaouanc-Lapalle H, Fievre C, Takahashi N, et al. Staphylococcal exanthematous disease in a newborn due to a virulent methicillin-resistant Staphylococcus aureus strain containing the TSST-1 gene in Europe: an alert for neonatologists. J Clin Microbiol. 2003;41:4883-4.

(9.) Waldvogel FA. Staphylococcus aureus. In: Mandell GL, Bennett JE, Dolin R, editors. Mandell, Douglas, and Bennett's principles and practice of infectious diseases. Philadelphia: Churchill Livingstone; 2000. p. 2069-92.

(10.) Sanford JR Gilbert DN, Moellering RC Jr, Sande MA. The Sanford guide to antimicrobial therapy. 17th ed., Belgian/ Luxemburg version. Hyde Park (VT): Antimicrobial Therapy, Inc.; 2003.

(11.) Issa NC, Thompson RL. Staphylococcal toxic shock syndrome. Postgrad Med. 2001; 110:55-62.

Sophie Jamart, * Olivier Denis, * Ariane Deplano, * Georgios Tragas, * Alexandra Vandergheynst, * David De Bels, * and Jacques Devriendt *

* Universite Libre de Bruxelles, Brussels, Belgium

Address for correspondence: Sophie Jamart, Department of intensive Care Medecine, Brugmann University Hospital, 4 Place Van Gehuchten, 1020 Brussels, Belgium; fax: 32-2-477-2631; email:

COPYRIGHT 2005 U.S. National Center for Infectious Diseases
COPYRIGHT 2005 Gale Group

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