Variant Creutzfeldt-Jakob Disease Literature: Commentary by Dr. John G. Bartlett -- April 2007

John G. Bartlett, MD


May 03, 2007

Homepage: CJD Update 2007 ProMED-AHEAD Digest V2007:44:1 : The International Society for Infectious Disease has provided the following update on variant Creutzfeldt-Jakob disease (vCJD):

Global experience: Table 1 summarizes the total cases, number of persons living with vCJD, the number with residence in the United Kingdom for more than 6 months, and the number of cases implicating blood transfusions for the 4 countries with the most cases.

Cases in United Kingdom: The total number of deaths from definite vCJD in the United Kingdom is 158 and the number of probable cases in persons still alive is 7; the total is 165 through February, 2007. The initial cases were reported in 1995, the zenith was 28 cases in 2000 and the number has subsequently decreased, suggesting the cases are on the wane. These data are summarized in Table 2.

Fourth case of transfusion-associated vCJD: The fourth case is a patient in whom symptoms developed 8.5 years after receiving a transfusion of red blood cells (RBCs) from a donor in whom the disease developed 17 months after the blood donation. All 4 cases have involved non-leukocyte depleted RBC transfusions between 1996 and 1999. Leukocyte depletion has been standard practice in the United Kingdom since October, 1999. All 4 cases are patients who are methionine homozygote on codon 129 of the prion protein gene. Persons who have received vCJD-implicated blood transfusions in the United Kingdom have been warned of this exposure and advised to take the precautions to prevent further transmission by donating blood.

Engineered resistance in cattle: The report indicates that scientists in the United States and Japan had successfully used genetic engineering to produce cattle that are resistant to bovine spongiform encephalopathy (BSE).[1] The method was 'gene targeting' with a knockout of the prion protein gene. It was shown that this protein is not necessary for the normal development and survival of cattle.


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