Ethics in prion disease
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- 作者：Kendra Bechtel ; Michael D. Geschwind
- 关键词：CJD ; Creutzfeldt-Jakob Disease ; CSF ; University of California ; San Francisco ; RPD ; Rapidly Progressive Dementia ; TSE ; Transmissible Spongiform Encephalopathy ; sCJD ; Sporadic CJD ; gPrD ; Genetic Prion Disease ; fCJD ; Familial CJD ; vCJD ; Variant CJD ; iCJD ; Iatrogenic CJD ; BSE ; Bovine Spongiform Encephalopathy ; CWD ; Chronic Wasting Disease ; GSS ; Gerstmann&ndash ; Strä ; ussler&ndash ; Scheinker ; FFI ; Fatal Familial Insomnia ; OPRI ; Octopeptide Repeat Insertion ; FDA ; Food and Drug Administration ; WHO ; Wo
- 刊名：Progress in Neurobiology
- 出版年：November, 2013
- 年：2013
- 卷：110
- 期：Complete
- 页码：29-44
- 全文大小：681 K
文摘
This paper is intended to discuss some of the scientific and ethical issues that are created by increased research efforts towards earlier diagnosis, as well as to treatment of, human prion diseases (and related dementias), including the resulting consequences for individuals, their families, and society. Most patients with prion disease currently are diagnosed when they are about 2/3 of the way through their disease course (), when the disease has progressed so far that even treatments that stop the disease process would probably have little benefit. Although there are currently no treatments available for prion diseases, we and others have realized that we must diagnose patients earlier and with greater accuracy so that future treatments have hope of success. As approximately 15% of prion diseases have a autosomal dominant genetic etiology, this further adds to the complexity of ethical issues, particularly regarding when to conduct genetic testing, release of genetic results, and when or if to implement experimental therapies. Human prion diseases are both infectious and transmissible; great care is required to balance the needs of the family and individual with both public health needs and strained hospital budgets. It is essential to proactively examine and address the ethical issues involved, as well as to define and in turn provide best standards of care.