Disease, variant Creutzfeldt-Jakob
Disease, variant Creutzfeldt-Jakob: Abbreviated vCJD. A human disease thought due to the same infectious agent as bovine spongiform encephalopathy (BSE), or mad cow disease. Both the human and bovine disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent, a prion, resulting in the deposition of amyloid tissue that causes a breakdown of brain tissue leaving the infected brain with a “spongy” (“spongiform”) appearance. The disease in humans is sometimes called new variant Creutzfeldt-Jakob disease (nvCJD).
The BSE agent has been identified in the brain, spinal cord, retina, dorsal root ganglia (nervous tissue located near the backbone), and the bone marrow of cattle experimentally infected with this agent by the oral route. In addition to cattle, sheep are susceptible to experimental infection with the BSE agent by the oral route. Thus, in countries where flocks of sheep and goats may have been exposed to the BSE agent through contaminated feed, there exists a theoretical risk that these animals may have developed infections caused by the BSE agent and that these infections have been maintained in the flocks, even in the absence of continued exposure to contaminated feed (for example, through maternal transmission).
Everyone with vCJD appear to have eaten beef and beef products at some time during their lives (but then so have most of the population). Investigation of a cluster of cases with vCJD in the UK disclosed that most of the people were likely infected through their diet. Beef carcass meat appears to have been contaminated with the BSE agent in butchers’ shops where cattle heads were split.
Read Also:
- Disease, Vrolik's
Disease, Vrolik’s: Osteogenesis imperfecta type II, an inherited connective tissue disorder with very severe bone fragility, the lethal form of “brittle bone disease.” It is a recessive trait with males and females affected. Two copies of the mutant gene are needed to cause the disease. Clinically the disease is characterized by short limb dwarfism, thin […]
- Disease, Werner-His
Disease, Werner-His: Named for the German physician Heinrich Werner (who did not describe Werner’s premature aging syndrome) and the Swiss physician Wilhelm His, Jr. (who did describe the bundle of His in the heart), this is a louse-borne disease that was first recognized in the trenches of World War I and so was called trench […]
- Disease, Whitmore's
Disease, Whitmore’s: An infectious illness, also called Whitmore’s disease, that is most frequent in Southeast Asia and Northern Australia and is caused by a bacteria called Burkholderia pseudomallei found in soil, rice paddies and stagnant waters. Humans catch the disease by inhalation of contaminated dust or when soil contaminated by the bacteria comes in contact […]
- Disease, Wilson
Disease, Wilson: An inherited disorder in which too much copper accumulates in the body. Although the accumulation of copper begins at birth, symptoms of the disorder appear later in life, between the ages of 6 and 40. A diagnostic feature of Wilson disease is what is called a Kayser-Fleischer ring, a deep copper-colored ring around […]
- Disease-free survival
Disease-free survival: The length of time after treatment during which no disease is found. Can be reported for an individual patient or for a study population. “Disease-free survival predicts survival. It always has. And there is a very big decrease in cancer incidence so there will be a survival difference.” (Dr. Larry Norton, in The […]