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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
Watermelon stomach
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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'When all else fails, read the directions': safely using disinfectants and sterilants
From Healthcare Purchasing News, 7/1/05 by Susan Cantrell

Ask almost any expert familiar with the use of disinfectants and sterilants what they think is the most important step healthcare workers (HCWs) can take to ensure these chemicals are used safely and appropriately, and you'll almost invariably hear this answer, loud and clear: READ ... THE ... LABEL. It's a simple but crucial preliminary step. Oddly, it's not always given the attention it merits.

Jack Wagner is president of Micro-Scientific Industries Inc, Rolling Meadows, IL, a company that makes disinfectant and antimicrobial products for a number of companies in the instrument and healthcare industries. He waxes passionately on the subject, conveying a sense of urgency for the need for , users to read labels and follow directions, particularly as it pertains to potentially dangerous disinfectants and sterilants. "There's an old saying," said Wagner, "'When all else fails, read the directions.' How do you know 'when all else fails'? You have an outbreak at your facility."

Unfortunately, "all else fails" too often for Wagner's taste. According to him, too many healthcare facilities are under pressure to turn around equipment and instruments in the least amount of time possible, and they sometimes accomplish this by not following instructions on the label, which may mean not letting the product work on the equipment or instruments for the prescribed amount of time needed for killing infectious microbes.

"The problem is," said Wagner, "we are accepting the directive to move on from patient to patient because it's more cost-effective. I believe we're giving up efficacy, and we're losing the battle of infection in healthcare facilities because of that. We need to pay closer attention to our cleaning and disinfection process. The label of the disinfectant states specifically how the product is to be used to be effective. It's written on there because that's how the product was tested. I can guarantee you that many of these products were tested at lesser time periods in smaller concentrations to try to get by with as little as possible, and the time that's on the label is the minimum amount of time it takes for that disinfectant to work. If a product doesn't suit your needs because the labeled instructions don't suit your facility, then you need to find one that will. You don't change the use of products to suit your needs, you change products."

Wagner continued: "I can't stress it enough: read the label of the product that you're using. If you're using a product for only 20 minutes when the label calls for 45 minutes or 90 minutes, you're using it off-label. I don't care who the expert is, if they tell you to use a product off-label, chances are the product isn't working. The reason the label says 45 or 90 minutes is because the manufacturer has proved that their product works consistently under those conditions. If you change that, you're putting patients, yourself, and even your families in jeopardy, because you can pick up an infection and take it home."

"Do HCWs use disinfectants properly? Some do, some don't" said Wagner. Is there room for improvement? Absolutely. Read the label; use the product according to its instructions. If you don't understand it, call the company. If you have a question and aren't 100% satisfied with the answer, if it's not specifically on the label, tell the company you want their answer in writing for your records, and tell them you have to send a copy to the local Environmental Protection Agency and health departments. They'll back off if it isn't true."

Where's the 'beef'?

Unquestionably, sterile processing workers are charged with important work. Technically, they're in a position to save lives. Their work can prevent deadly infections from being transmitted from workers to patients and vice versa. So, wherein lies the failure to complete such a vital and straightforward step as reading the label and following its instructions? Why is the importance of reading labels not recognized?

Nancy Chobin has some very definite ideas about that. Chobin is the sterile processing educator/ consultant at the Saint Barnabas Health Care System, West Orange, NJ; she's also executive director for the Certification Board for Sterile Processing and Distribution, which puts her in the catbird seat for knowing what s going on in the field. Chobin observed, "I've never found sterile-processing people to be anything other than wanting to do the very best. When they don't, it's usually because they were not properly trained. I think the issue is whether they've been given the correct information." If workers have a problem in performing their work correctly, then "shame on the manager," said Chobin.

Education: make use of the manufacturer

Manufacturers are the best source of information on their products; logically, they're where the education process should start. Many of them make it a point to be up to the task.

John Kurowski, global manager, clinical education, STERIS Corporation, Mentor, OH, described his company's educational services: "STERIS educational programs focus on helping healthcare providers achieve best practices and optimal patient outcomes. Our training and education offering includes online continuing education; CD training; on-site in-servicing by account managers and clinical specialists; 1-hour continuing education programs; booth sessions at professional conferences, regionally and nationwide; instructor-led programs at STERIS headquarters; and self-study guides that provide contact hour credits when completed successfully."

Highlighting the need for ongoing education, and for keeping abreast of label instructions, Kurowski stated, "There is always room for improvement. There are so many different disinfectants and sterilants available, and there are many different label claims. Improvement must come from educating personnel through professional organizations and manufacturers, and from strict adherence to product labeling. Product labeling on disinfectants and sterilants provides a great deal of important information the user must know before mixing or using the chemical. 'Forewarned is forearmed,' as they say. Ongoing education is the key to maintaining critical knowledge that will reduce safety and infection risks. It requires management to make training a priority and to schedule the time for employees to participate, but it can help hospitals achieve and maintain best practices and can reduce risks to patients and employees."

Users of company-provided education know what they want, what works for their purpose. "I really like it when companies give me a videotape," stated Chobin. "I don't like when they come in and give a 5-minute in-service, because nobody remembers what they did. I can show a videotape, and I can repeat it. I like to get color posters for reminders, and I want the company to provide a competency assessment so that I can verify the competency of employees who are using the product. That's very important."

Loretta Litz Fanerbach, board member of the Association for Professionals in Infection Control and Epidemiology, Inc (APIC), communications team leader for APIC, and director of infection prevention and control, Shands Hospital at the University of Florida, Gainesville, FL, concurred and added: "The user greatly appreciates getting thorough and easy-to-use educational programs and modules from manufacturers that can be readily adapted to their setting. Videos, PowerPoint presentations, and self-study modules are just a few of the types of programs that are really helpful for the user: Also, a hot line number or access to a technical expert is really appreciated by healthcare providers."

Fauerbach continued: "Another good resource for information about proper use, safety, and efficacy is the "APIC Guideline on Disinfection and Sterilization, 1996," by William Rutala, PhD. APIC has partnered with the Society for Healthcare Epidemiology of America and others to commission Rutala to update that guideline, and it is pending release from the Centers for Disease Control and Prevention as a Healthcare Infection Control Practices Advisory Committee guideline. It will provide users with lots of important information."

No educational program would be complete without explaining to workers the reasons it's important to protect them selves from chemicals and to handle the chemicals per label instructions. Chobin told HPN, "I think that, very often, what is missing from education is the why. If people understand why, they're more likely to follow it," observed Chobin. "Our staff is very well educated. They understand they have to wear protective attire, and they understand why."


The reason why disinfectants and sterilants should be treated with the utmost respect is because they are chemicals with dangerous, even lethal, potential. Chobin made it crystal-clear: "Products that kill microbes can kill people."

"Don't forget," admonished Wagner, "disinfectants' primary job is to kill living cells. A chemical can't say, 'Well, that cell belongs to Jack, it's OK, but the cell next to it is a bacterial cell, Staphylococcus" aureus. 'The disinfectant can't tell the difference, so it tries to kill them both. Disinfectants are toxic. There is no such thing as a nontoxic disinfectant."

In Wagner's opinion, however, the greatest hazard from disinfectants and sterilants to HCWs is not exposure to the chemical itself but misuse of the product due to not reading labels, thereby exposing patients, themselves, and their families to infectious agents that didn't get killed during the disinfection or sterilization process. Worse yet, he said, when the microorganisms are not killed, it may set the scene for more powerful bugs to grow. "Some of the organisms are probably becoming resistant, becoming stronger," stated Wagner.

Exposure to a hazardous chemical is a genuine threat, and certain controls should be in place for worker and patient safety. Fauerbach explained: "The HCW must be trained properly to know what precautions to take and how to handle the disinfectant or sterilant product. To protect the HCW, when using any product, there must be a current and thorough procedure and policy on how to use the product appropriately. Every product has a Material Safety Data Sheet (MSDS), which lists safety precautions, such as appropriate rinsing, for the product to be used safely. There are warnings, about contact with mucous membranes or skin for some products. If the product has requirements for area ventilation, the HCW must be given the correct room with ventilation controls to work with the product. In some cases, the MSDS indicates that workers need to be monitored for potential exposure, and the employer should set up a safety monitoring system if that is required."

"Detergents and disinfectants can damage skin and eyes, perhaps even causing loss of vision," reiterated Chobin. "Yon have to follow the MSDS in terms of protective attire. MSDSs should be updated every couple of years, because the information does change. There are people still using vinyl gloves for handling glutaraldehyde, which is totally inappropriate because they absorb the glutaraldehyde. If you don't read the MSDS, you're not going to know that you need special gloves for handling this chemical; it really is critical. Most people do not read labels," Chobin said. "Even at home you need to follow this principle. There's a lot of information on labels; that's why it's there."

Aside from worker and patient safety, many other factors need to be considered for safe handling of disinfectants and sterilants. Such information can be found, no big surprise by now, on the product's label. Chobin outlined what sorts of necessary and important information about products appear on their labels, including how to store the chemical, how long it can be stored safely, and whether the longevity of the product is affected by opening the bottle; whether the chemical is affected by temperature and humidity; how to mix it and what is the correct concentration; whether there are any adverse effects with materials, and what material compatibility studies have been done; contact time: how long the disinfectant should remain on the product for the disinfectant to work; microbiocidal activity; pH; interactions with other chemicals; water claims: whether it should be used only with distilled water or if it can be used with tap water. 'All of that information is absolutely critical, and that's just for starters," exclaimed Chobin.

Fauerbach emphasized the need to be particularly concerned with mixing, concentration, and adequate contact time: "Disinfectant products must be used according to the manufacturer's directions on the label. I am afraid that, quite often, anyone fixing a solution to use in cleaning might not mix correctly, either by not measuring or by thinking 'if one squirt is good, two might be better.' Products function best when used at the correct use dilution. Another faulty practice in our busy society is not to allow the proper amount of contact time for the disinfectant to work. Some manufacturers list that a 10-minute exposure time is required; but, in the environment, workers may 'swipe and go,' and adequate cleaning and disinfection may not occur."

In Wagner's opinion, "That's where people are making their mistakes. They're not following labeled instructions because they're in a hurry. In the name of time and saving money, it's 'let's get to the next patient and move on'. You can't do that. We're not cleaning Hula Hoops here. We're cleaning medical equipment that's going from room to room and patient to patient."

"In the fast-paced sterile-processing environment, it's easy to take shortcuts," commented Kurowski. "Employees should be fully aware of the consequences of taking safety shortcuts, and they should not let the pace dictate their practice."

Disinfection and sterilization products are wonderful, even amazing, provisions in the battle against nosocomial infection, but they're only one of the components in the disinfection and sterilization process. The most important component is human. It's up to humans to use the products safely and effectively. If labels aren't read and the instructions aren't employed correctly, the product likely won't be effective, said Wagner: "You may as well use water."

The healthcare industry is constantly searching for ways to make the process faster, more efficient, and safer. Fauerbach noted, "We are all looking for the magic bullet. But until that time, elbow grease used to assure proper and thorough cleaning, adherence to use dilution and contact-time recommendations, and following safety guidelines are the safety keys for everyone."

When handling the responsibility of disinfection and sterilization, knowledge truly is power, the power to improve or possibly even save lives. Who knows ... the life you save may be your own.

COPYRIGHT 2005 Healthcare Purchasing News
COPYRIGHT 2005 Gale Group

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