De eerste patiënt in Nederland met de nieuwe variant van de ziekte van Creutzfeldt-Jakob
De eerste patiënt in Nederland met de nieuwe variant van de ziekte van Creutzfeldt-Jakob
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荷兰的一名患者遇到了新的变种 van de ziekte van Creutzfeldt-Jakob
DOI：
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发表时间：
2005
期刊：
影响因子：
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通讯作者：
C. Duijn
中科院分区：
文献类型：
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作者：
Casper Jansen;M. Houben;J. I. Hoff;Pascual Sanchez;A. J. Rozemuller;C. Duijn
一名荷兰妇女因新的克雅氏病变种(VCJD)在患病18个月后去世，享年26岁。她从未去过联合王国，也没有潜在的医源性接触史。然而，她之前在餐饮和食品生产行业工作了6年，经常食用生肉。病程显示典型的vCJD临床表现，并经尸检证实。与经典的散发性CJD相反，这种变异型疾病的患者通常更年轻，主要表现为精神症状。像疼痛和感觉困难这样的感觉主诉通常很快就会出现。直到后来，这些症状才会伴随着迅速进展的神经症状和体征。到目前为止，所有患者的基因分型都是Pron蛋白基因I29密码子上的蛋氨酸纯合子。由于该病可能通过血液和组织传播，因此对该病的识别尤为重要。对于有快速进展的精神症状和无法解释的神经体征的患者，尤其是感觉性主诉，必须考虑vCJD的可能性。
A Dutch woman died at the age of 26 years, after a disease duration of I8 months, due to the new variant of Creutzfeldt-Jakob's disease (vCJD). She had never travelled to the United Kingdom and there was no history of potential iatrogenic exposure. However, she had worked in the catering and food production industry for the previous 6 years and had frequently consumed raw meat. The disease course showed the classical clinical picture of vCJD, which was confirmed by post-mortem examination of the brain. Contrary to classical sporadic CJD, patients with the variant disease are usually younger and present predominantly with psychiatric symptoms. Sensory complaints like pain and dysaesthesiae usually follow soon. Only later are these symptoms followed by rapidly progressive neurological symptoms and signs. All patients genotyped so far are homozygous for methionine on codon I29 of the prion protein gene. Recognition of the disease is of particular importance because of possible transmission via blood and tissues. In patients with rapidly progressive psychiatric symptoms and unexplained neurological signs, particularly sensory complaints, one must consider the possibility ofvCJD.