Subject: Experience With Preventive Genetic Testing of Corneal Donors in Slovakia
POST AUTHOR CORRECTIONS, 20 April 2011 doi: 10.1097/ICO.0b013e3182035ac1 Clinical Science: PDF Only
Experience With Preventive Genetic Testing of Corneal Donors in Slovakia
Mitrová, Eva MD; Cernák, Andrej MD; Slivarichová, Dana PhD; Košcová, Silvia MSc; Bernovská, Veronika MSc; Cernák, Martin MD
Purpose: The purpose of this study was (1) to detect asymptomatic carriers of the prion protein gene mutation E200K, which is associated with Creutzfeldt-Jakob disease (CJD), in corneal donors and in the general population of Slovakia and (2) to assess the genetic testing of corneal donors as an effective preventive measure against iatrogenic infection in a country with an unusually high incidence of genetic CJD.
Methods: The prion protein gene (PRNP) was analyzed in 1133 corneal donors and 970 control subjects to search for E200K mutation and to determine the genotype at codon 129.
Results: Mutation E200K was found in 2 of the 1133 donors and in 4 of the 970 control subjects. The most frequent genotype at the codon 129 polymorphic region was methionine homozygous (48% of donors and controls).
Conclusions: An E200K mutation, which confers a risk of developing genetic CJD, was detected in corneal donors and in the general population. The majority of subjects were codon 129 methionine homozygous that increases susceptibility to CJD. Genetic testing of corneal donors in Slovakia is a useful and effective preventive measure against iatrogenic CJD through human corneal transplantation in the investigated population.
(C) 2011 Lippincott Williams & Wilkins, Inc.
----- Original Message -----
From: "Terry S. Singeltary Sr."
Sent: Friday, August 07, 2009 6:50 PM
Subject: [CJD-L] CJD Human Cornea Tissue, Recall END OF ENFORCEMENT REPORT FOR AUGUST 5, 2009
PRODUCT Human Cornea Tissue, Recall # B-1129-09 CODE Units: CM109415 OS and CM109415 OD RECALLING FIRM/MANUFACTURER Illinois Eye Bank, Watson Gailey, Bloomington, IL, by telephone and letter dated on October 9, 2008. Firm initiated recall is complete. REASON Donor history indicated increased risk factors for Creutzfeldt-Jacob disease. Tissues had been distributed for transplantation. VOLUME OF PRODUCT IN COMMERCE 2 corneas DISTRIBUTION IL ___________________________________
PRODUCT Recovered Plasma, Recall # B-1280-09 CODE Unit: W127808401598 RECALLING FIRM/MANUFACTURER Tacoma Pierce County Blood Bank, Tacoma, WA, by email on March 25, 2009. Firm initiated recall is complete. REASON Blood product, collected from a donor considered to be at increased risk for variant Creutzfeldt-Jakob Disease (vCJD), was distributed. VOLUME OF PRODUCT IN COMMERCE 1 unit DISTRIBUTION Switzerland ___________________________________
PRODUCT Plasma Frozen, Recall # B-1301-09 CODE Units: F00493, F00111, R19438 RECALLING FIRM/MANUFACTURER Blood Center of Northcentral Wisconsin, Inc., Wausau, WI, by facsimile on May 12, 2009. Firm initiated recall is complete. REASON Blood products, collected from a donor who was at risk for variant Creutzfeldt-Jakob Disease (vCJD), were distributed. VOLUME OF PRODUCT IN COMMERCE 3 units DISTRIBUTION NY ___________________________________
END OF ENFORCEMENT REPORT FOR AUGUST 5, 2009
Volume 3 No 28; 17 July 2009
HPR Home | Archives | 2009 news
Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units
Pandemic flu: UK situation at 16 July 2009
Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units
Patients are routinely assessed prior to undergoing surgical or neuro-endoscopy procedures to determine whether they may have an increased risk of CJD infection. If this is the case, special infection control precautions should be followed .
This pre-surgical assessment process has been revised to fully take account of the latest knowledge of variant CJD (vCJD). Now patients undergoing high risk surgery  or neuro-endoscopy should be assessed to identify those who have received transfusions from 80 or more donors since 1980. Any patients so identified have an increased risk of vCJD, and special infection control procedures should be followed. It is estimated that around 50 patients will be identified in this way in the UK each year.
Hospitals are being asked to collate blood transfusion histories for these patients, and conduct risk assessments for patients with uncertain or incomplete transfusion histories. Patients who are found to have an increased risk of vCJD will need to be informed via the HPA Centre for Infections CJD section, which is coordinating the implementation of the revised guidance, and their GP.
The vCJD risk to these patients is uncertain, and depends on the prevalence of subclinical vCJD infection among blood donors, the infectivity in the blood of infected donors, and the number of donors the patients have received blood from.
The HPA's CfI-CJD section should be informed of any patients who are identified prior to high risk surgery or neuro-endoscopy as having received blood from 80 or more donors. Health Protection Units have been asked to ensure that infection control teams are aware of the revised guidance.
Information for healthcare workers and patients is available at:
1. Department of Health. "Assessment to be carried out before surgery and/or endoscopy to identify patients with, or at increased risk of, CJD or vCJD" (Annex J of ACDP TSE Working Group guidance).
2. High risk surgery is defined as surgery involving any of the following organs or tissues (high risk tissues): brain, spinal cord, dura mater, cranial nerves (specifically the entire optic nerve and only the intracranial components of the other cranial nerves), cranial nerve ganglia, posterior eye (specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid, optic nerve) and pituitary gland.
Pandemic flu: UK situation at 16 July 2009
The latest HPA Weekly Pandemic Flu Update  notes the following developments as at 16 July:
GP consultation rates in England for individuals presenting with flu-like illness showed increased rates, well above the threshold level for normal seasonal flu activity; the under-fives and 5-14 year olds are the age groups predominantly affected; the majority of cases continue to be mild, but there had been 26 deaths in England as at 16 July; and HPA estimated that there were 55,000 new cases of swine flu during the previous week (within a possible range of 30,000 to 85,000). Following the move away from laboratory testing for confirmation of swine flu cases to clinical diagnosis , the level of influenza in the community is being monitored using a range of surveillance mechanisms. One of these is the collaborative project between the HPA and the University of Nottingham Division of Primary Care known as QSurveillance®. This is a not-for-profit network over 3,300 general practices covering a total population of almost 22 million patients (more than 25% of the UK population). In the week-ending 12 July, the flu-like illness weekly consultation rate recorded by QSurveillance® reached 86.8 per 100,000, higher than the peak activity in winter 08/09 (see figure 2 in the HPA Weekly Pandemic Flu Update for 16 July ).
Given that laboratory testing is no longer routinely recommended, figure 1 (below) graphically illustrates how primary care surveillance such as QSurveillance® is providing a good comparator to laboratory confirmation, showing daily reporting of laboratory confirmed cases alongside daily primary care surveillance reports for flu-like illness.
Figure 1: Comparison of daily laboratory confirmed H1N1v case reports and daily QSurveillance® reports of influenza-like illness, UK*
* Data source: QSurveillance (daily data). Database version 1. Copyright QRESEARCH 2009.
Department of Health guidance: the National Pandemic Flu Service
In view of the very high demand for primary care, NHS Direct and A and E services in many areas, the Department of Health announced plans to activate, subject to rigorous testing, the National Pandemic Flu Service before the end of July .
1. "Weekly pandemic flu update (16 July 2009)", (HPA press release of 16 July 2009). HPA website: National Press Releases.
2. "Treatment approach announced for pandemic flu", Health Protection Report [serial online] 2009; 3 (26): news. Available at:
3. Department of Health. "National Pandemic Flu Service", 17 July 2009
Saturday, May 23, 2009
Latest results of HPA study on vCJD-related abnormal prion proteins in extracted tonsil
Wednesday, August 20, 2008
Tonometer disinfection practice in the United Kingdom: A national survey
Saturday, July 26, 2008
Creutzfeldt-Jakob Disease in Recipients of Corneal Transplants
Eye procedure raises CJD concerns November 19, 2004
United Press International by STEVE MITCHELL
A New York man who died from a rare brain disorder similar to mad cow disease in May underwent an eye procedure prior to his death that raises concerns about the possibility of transmitting the fatal disease to others, United Press International has learned. The development comes on the heels of the announcement Thursday by U.S. Department of Agriculture officials of a possible second case of mad cow disease in U.S. herds.
Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt Jakob disease, an incurable brain-wasting illness that strikes about one person per million.
Richard's wife Ann Marie Da Silva told UPI he underwent a check for the eye disease glaucoma in 2003, approximately a year before his death. The procedure involves the use of a tonometer, which contacts the cornea -- an eye tissue that can contain prions, the infectious agent thought to cause CJD.
Ann Marie's concern is that others who had the tonometer used on them could have gotten infected.
A 2003 study by British researchers suggests her concerns may be justified. A team led by J.W. Ironside from the National Creutzfeldt-Jakob Disease Surveillance Unit at the University of Edinburgh examined tonometer heads and found they can retain cornea tissue that could infect other people -- even after cleaning and decontaminating the instrument.
"Retained corneal epithelial cells, following the standard decontamination routine of tonometer prisms, may represent potential prion infectivity," the researchers wrote in the British Journal of Ophthalmology last year. "Once the infectious agent is on the cornea, it could theoretically infect the brain."
Prions, misfolded proteins thought to be the cause of mad cow, CJD and similar diseases, are notoriously difficult to destroy and are capable of withstanding most sterilization procedures.
Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University, agreed with the British researchers that tonometers represent a potential risk of passing CJD to other people.
Manuelidis told UPI she has been voicing her concern about the risks of corneas since 1977 when her own study, published in the New England Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.
At the time the procedure was done on Richard Da Silva, about a year before he died, she said it was "absolutely" possible he was infectious.
The CJD Incidents Panel, a body of experts set up by the U.K. Department of Health, noted in a 2001 report that procedures involving the cornea are considered medium risk for transmitting CJD. The first two patients who have a contaminated eye instrument used on them have the highest risk of contracting the disease, the panel said.
In 1999, the U.K. Department of Health banned opticians from reusing equipment that came in contact with patients' eyes out of concern it could result in the transmission of variant CJD, the form of the disease humans can contract from consuming infected beef products.
Richard Da Silva was associated with a cluster of five other cases of CJD in southern New York that raised concerns about vCJD.
None of the cases have been determined to stem from mad cow disease, but concerns about the cattle illness in the United States could increase in light of the USDA announcement Thursday that a cow tested positive on initial tests for the disease. If confirmed, this would be the second U.S. case of the illness; the first was detected in a Washington cow last December. The USDA said the suspect animal disclosed Thursday did not enter the food chain. The USDA did not release further details about the cow, but said results from further lab tests to confirm the initial tests were expected within seven days.
Ann Marie Da Silva said she informed the New York Health Department and later the eye doctor who performed the procedure about her husband's illness and her concerns about the risk of transmitting CJD via the tonometer.
The optometrist -- whom she declined to name because she did not want to jeopardize his career -- "didn't even know what this disease was," she said.
"He said the health department never called him and I called them (the health department) back and they didn't seem concerned about it," she added. "I just kept getting angrier and angrier when I felt I was being dismissed."
She said the state health department "seems to have an attitude of don't ask, don't tell" about CJD.
"There's a stigma attached to it," she said. "Is it because they're so afraid the public will panic? I don't know, but I don't think that the answer is to push things under the rug."
New York State Department of Health spokeswoman Claire Pospisil told UPI she would look into whether the agency was concerned about the possibility of transmitting CJD via tonometers, but she had not called back prior to story publication.
Disposable tonometers are readily available and could avoid the risk of transmitting the disease, Ironside and colleagues noted in their study. Ann Marie Da Silva said she asked the optometrist whether he used disposable tonometers and "he said 'No, it's a reusable one.'"
Ironside's team also noted other ophthalmic instruments come into contact with the cornea and could represent a source of infection as they are either difficult to decontaminate or cannot withstand the harsh procedures necessary to inactivate prions. These include corneal burrs, diagnostic and therapeutic contact lenses and other coated lenses.
Terry Singletary, whose mother died from a type of CJD called Heidenhain Variant, told UPI health officials were not doing enough to prevent people from being infected by contaminated medical equipment.
"They've got to start taking this disease seriously and they simply aren't doing it," said Singletary, who is a member of CJD Watch and CJD Voice -- advocacy groups for CJD patients and their families.
U.S. Centers for Disease Control and Prevention spokeswoman Christine Pearson did not return a phone call from UPI seeking comment. The agency's Web site states the eye is one of three tissues, along with the brain and spinal cord, that are considered to have "high infectivity."
The Web site said more than 250 people worldwide have contracted CJD through contaminated surgical instruments and tissue transplants. This includes as many as four who were infected by corneal grafts. The agency noted no such cases have been reported since 1976, when sterilization procedures were instituted in healthcare facilities.
Ironside and colleagues noted in their study, however, many disinfection procedures used on optical instruments, such as tonometers, fail. They wrote their finding of cornea tissue on tonometers indicates that "no current cleaning and disinfection strategy is fully effective."
Singletary said CDC's assertion that no CJD cases from infected equipment or tissues have been detected since 1976 is misleading.
"They have absolutely no idea" whether any cases have occurred in this manner, he said, because CJD cases often aren't investigated and the agency has not required physicians nationwide report all casesof CJD.
"There's no national surveillance unit for CJD in the United States; people are dying who aren't autopsied, the CDC has no way of knowing" whether people have been infected via infected equipment or tissues, he said.
Ann Marie Da Silva said she has contacted several members of her state's congressional delegation about her concerns, including Rep. Sue Kelly, R-N.Y., and Sen. Charles Schumer, D-N.Y.
"Basically, what I want is to be a positive force in this, but I also want more of a dialogue going on with the public and the health department," she said.
Cadaver corneal transplants -- without family permission Houston, Texas channel 11 news 28 Nov 99 Reported by Terry S. Singeltary Sr.son of CJD victim
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
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TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY EXPOSURE SPREADING VIA HOSPITALS AND SURGICAL PROCEDURES AROUND THE GLOBE
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27 U.S. Senators want to force feed Japan Highly Potential North America Mad Cow Beef TSE PRION CJD
March 8, 2011
President Barack Obama The White House
1600 Pennsylvania Avenue, W Washington, DC 20500
Dear President Obama: