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What is Variant Creutzfeldt-Jakob Disease?

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How is variant CJD different than classic CJD?
asked May 9, 2012 in Glossary by pnutts (1,820 points)
retagged Jun 4, 2012 by LarryEitel

2 Answers

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Variant CJD was first described in 1996 in the United Kingdom. It has different clinical and pathologic characteristics from classic CJD. Each disease also has a particular genetic profile of the prion protein gene. (See table below). The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD. The median duration of illness for vCJD is 14 months, compared to 5 months for classic CJD.

answered May 9, 2012 by pnutts (1,820 points)
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Variant Creutzfeldt-Jakob disease (vCJD) may be transmissible by blood. To prevent secondary transmission through blood components, several countries have started to exclude as donors persons who have received a blood transfusion. We investigated the effectiveness of this measure by using a dynamic age-structured model. It is the first such model based on epidemiologic data: 1) blood donor activities, 2) a case-control study on CJD, 3) age distribution of recipients, and 4) death of recipients of blood transfusions. The model predicts that an infection like vCJD, which has been introduced into the population by the alimentary route, could not become endemic by transfusion alone and that <1% of cases would be avoided by excluding from blood donation those persons who have received a transfusion.



Modeling the Infection

Usually the incubation period refers to the time between the infection and disease. In the context of CJD, however, disease can refer to onset, diagnosis, or death. Like Bacchetti, we also focused on death rates (810). The incubation period is assumed to be γ distributed with a mean duration of 16 years and a standard deviation of 4 years, which conforms to estimates of Valleron et al. and Ghani et al. (5,6). Because of great uncertainty about the length of the incubation time, we also considered a much higher value of 50 years in the absence of the competing risk for death. The coefficient of variation is assumed to be the same, such that the standard deviation is 12.5 years. Because of competing risks, the actual sojourn in the incubation period is 15.3 for an incubation period of 16 years and 34.0 years for an incubation period of 50 years. The proportions of infected persons who would die with disease symptoms are 79% and 37% for the incubation periods of 16 and 50 years, respectively. This means that for an incubation time of 50 years, nearly two thirds would die without disease symptoms. Hereafter we refer to these values of 15 and 50 years as short and long incubation periods.



answered May 9, 2012 by Hatice Simsek MD (3,070 points)



Blood Transfusion and Spread of Variant Creutzfeldt-Jakob Disease

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