医学词汇“Creutzfeldt Jakob Disease (CJD)”的翻译、意思、用法、用量、治疗方法-医学辞典

A rapidly progressive, fatal, degenerative disease in humans caused by an abnormal PRION protein. There are three aetiological forms of CJD: sporadic, IATROGENIC, and inherited. Sporadic CJD occurs randomly in all countries and has an annual incidence of one per million. Iatrogenic CJD is caused by accidental exposure to human prions through medical and surgical procedures, and cannibalism in the case of the human prion disease known as kuru that occurs in a tribe in New Guinea, where it is called the trembling disease. Inherited or familial CJD accounts for 15 per cent of human prion disease and is caused by a MUTATION in the prion protein gene. In recent years a new type of CJD has been identified that is caused by BOVINE SPONGIFORM ENCEPHALOPATHY (BSE), called variant CJD (vCJD). The incubation period for the acquired varieties ranges from four to 40 years, with an average of 10–15 years. The symptoms are dementia, seizures, focal signs in the central nervous system, MYOCLONUS, and visual disturbances.

Abnormal prion proteins accumulate in the brain and the spinal cord, damaging neurones (see NEURON(E)) and producing small cavities. Abnormal prion proteins are unusually resistant to inactivation by chemicals, heat, X-RAYS or ultraviolet light. They are resistant to cellular degradation and can convert normal prion proteins into abnormal forms. Human prion diseases, along with scrapie in sheep and BSE in cattle, belong to a group of disorders known as transmissible spongiform encephalopathies. Abnormal prion proteins can transfer from one animal species to another, and vCJD has occurred as a result of consumption of meat from cattle infected with BSE.

Examination of 30,000 appendix samples collected from patients operated on in the UK from 2000–2012 showed that 1 in 2,000 people may carry vCJD prions but only 177 cases had come to light by 2016. One precaution is that, since 2003, all surgical instruments used in brain biopsies have had to be quarantined and disposable instruments are now used in tonsillectomy, while measures have also been introduced to reduce the risk of transmission of CJD from transfusion of blood products.

单词	Creutzfeldt Jakob Disease (CJD)
释义	Creutzfeldt-Jakob Disease (CJD) A rapidly progressive, fatal, degenerative disease in humans caused by an abnormal PRION protein. There are three aetiological forms of CJD: sporadic, IATROGENIC, and inherited. Sporadic CJD occurs randomly in all countries and has an annual incidence of one per million. Iatrogenic CJD is caused by accidental exposure to human prions through medical and surgical procedures, and cannibalism in the case of the human prion disease known as kuru that occurs in a tribe in New Guinea, where it is called the trembling disease. Inherited or familial CJD accounts for 15 per cent of human prion disease and is caused by a MUTATION in the prion protein gene. In recent years a new type of CJD has been identified that is caused by BOVINE SPONGIFORM ENCEPHALOPATHY (BSE), called variant CJD (vCJD). The incubation period for the acquired varieties ranges from four to 40 years, with an average of 10–15 years. The symptoms are dementia, seizures, focal signs in the central nervous system, MYOCLONUS, and visual disturbances. Abnormal prion proteins accumulate in the brain and the spinal cord, damaging neurones (see NEURON(E)) and producing small cavities. Abnormal prion proteins are unusually resistant to inactivation by chemicals, heat, X-RAYS or ultraviolet light. They are resistant to cellular degradation and can convert normal prion proteins into abnormal forms. Human prion diseases, along with scrapie in sheep and BSE in cattle, belong to a group of disorders known as transmissible spongiform encephalopathies. Abnormal prion proteins can transfer from one animal species to another, and vCJD has occurred as a result of consumption of meat from cattle infected with BSE. Examination of 30,000 appendix samples collected from patients operated on in the UK from 2000–2012 showed that 1 in 2,000 people may carry vCJD prions but only 177 cases had come to light by 2016. One precaution is that, since 2003, all surgical instruments used in brain biopsies have had to be quarantined and disposable instruments are now used in tonsillectomy, while measures have also been introduced to reduce the risk of transmission of CJD from transfusion of blood products.
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