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New Insights Into Mad Cow Disease: Diversity Of Creutzfeldt-Jakob Disease Agents Discovered
Prion diseases are transmissible neurodegenerative disorders characterized by accumulation of an abnormal isoform (PrPSc) of a host-encoded protein (PrPC) in affected tissues. Although considered a spontaneous disorder, the clinicopathological characteristics of sporadic Creutzfeldt-Jakob disease (sCJD) are variable and substantially influenced by a particular variation in the DNA of the prion protein gene (PRNP). Due to the strong influence of host factors on the characteristics of the disease, diversity of prion agents responsible for CJD remains extremely difficult to investigate. In this study, using two new biochemical assays, the authors identified four distinct biochemical PrPSc subgroups in 41 sCJD cases. These subgroups correlate with the current sCJD subclassification. The subgroups were also found in 12 iatrogenic CJD (iCJD) cases from different countries. Iatrogenic CJD occurs following human-to-human sCJD transmission. In contrast to the sCJD cases, however, there was no particular correlation with the PRNP codon in the iCJD cases, indicating that observed biochemical properties could be specific to the prion agent. Further studies are required to confirm that the four biochemical subgroups identified correlate with distinct biological infectious agents. Journal reference: Uro-Coste E, Cassard H, Simon S, Lugan S, Bilheude J-M, et al. (2008) Beyond PrPres Type 1/Type 2 Dichotomy in Creutzfeldt-Jakob Disease. PLoS Pathog 4(3): e1000029. doi:10.1371/journal.ppat.1000029 (http://www.plospathogens.org/doi/ppat.1000029) Adapted from materials provided by Public Library of Science, via EurekAlert!, a service of AAAS.
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Although the exact cause of this trend is unclear, there are indications that continuous deep sedation may in some cases be being used as a substitute for euthanasia. Patients nearing death often experience distressing symptoms and sedating drugs can be used as an option of last resort. Sedation can be used intermittently or continuously until death, and the depth of sedation can vary from a lowered state of consciousness to unconsciousness. The most extreme use of sedation is continuous deep sedation until death, but there is a lack of large scale research on its use. In 2001, a large study in six European countries showed that continuous deep sedation was used in up to 8.5% of all deaths, among patients with cancer and other diseases, and provided in as well as outside hospital. In 2005, researchers repeated this study using a random sample of over 6,500 deaths that occurred in the Netherlands between August and November 2005. Physicians were surveyed about their medical decisions for the non-sudden deaths. The use of continuous deep sedation increased from 5.6% of deaths in 2001 to 7.1% in 2005 (an increase of 1800 cases). The increase occurred mostly in patients with cancer who were treated by a general practitioner. In contrast, the use of euthanasia decreased from 2.6% of all deaths in 2001 to 1.7% of all deaths in 2005 (a decrease of 1200 cases). In about four out of five of cases, sedation was induced by benzodiazepines, and in 94% patients were sedated for less than one week until death. Only 9% of physicians consulted a palliative expert. About one in ten patients who received continuous deep sedation had previously requested euthanasia or assisted suicide but it had not been granted. Possible explanations for these trends include increased knowledge and media attention about continuous deep sedation, say the authors. Their findings suggest that continuous deep sedation is increasingly considered part of regular medical practice in the Netherlands. They call for future research to focus on the underlying reasons for the use of continuous deep sedation. This study provides some insight into end of life management of patients with intractable suffering, say researchers in an accompanying editorial. They believe that further research must incorporate the perspectives of patients and families, as well as professionals from health care, spiritual care, social services, law and ethics. And they call for informed public debate about ethical and effective ways to alleviate persistent suffering at the end of life. Adapted from materials provided by BMJ-British Medical Journal, via EurekAlert!, a service of AAAS.
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Rob Jacobs has consulted on weight loss and nutrition for 20 years. He helps world class bodybuilders and dieters alike on how they can improve their health, and overall life. For more information on Rob's approach to health, please visit Natural Fat Burner
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"The delivery of genetic test results for a disease like cancer can trigger a range of emotions and can be more distressful than anticipated-- particularly when there's been no counseling and the results are 'positive'," explains Beth N. Peshkin, MS, CGC, senior genetics counselor at Lombardi Comprehensive Cancer Center, part of Georgetown University Medical Center, and educational director of the Jess and Mildred Fisher Center for Familial Cancer Research. "While in-person genetic counseling is ideal, it's not convenient for people who live in rural areas or don't have access to an academic center." According to Peshkin, genetic counseling and testing, particularly for adult onset conditions, is a trend that will continue to grow as additional genes are identified and as such testing diffuses into mainstream clinical care. Telephone counseling has been utilized with increased frequency despite a lack of data about its efficacy and concern about its use as a substitute for face-to-face contact with patients. "In anticipation of this increased demand, it is imperative we find alternatives to traditional genetic susceptibility counseling and that we develop and evaluate these possible options now," Peshkin explains. "A successful alternative would be one that effectively delivers information but allows greater accessibility, such as telephone counseling." To address these issues, Peshkin and her colleagues have launched a randomized clinical trial --the largest to date-- at Lombardi to evaluate telephone genetic counseling versus in person (standard) genetic counseling among women at high risk of carrying a BRCA1/2 mutation. "Many of us favor face-to-face counseling but the reality is the telephone may allow us to reach more people, more efficiently" says Peshkin. "It makes sense to develop interventions that parallel the traditional model while extending its reach and deliverability." Peshkin points to the abundant clinical data on the epidemiology of BRCA1/2 and the efficacy of various management strategies. Also, evidence attests to the efficacy of traditional genetic counseling at increasing knowledge, prospectively improving the accuracy of perceived risk, and increasing the awareness of the risks and benefits of testing. "Patients appear to be highly satisfied with the traditional format of comprehensive genetic counseling so a study among individuals undergoing BRCA1/2 testing is an ideal population on which to evaluate alternative models of counseling," Peshkin says. The study is outlined in the Spring edition of the journal Genetic Counseling (online March 17). Enrollment in the study has begun at Lombardi and the trial is expected to complete enrollment by 2010. Adapted from materials provided by Georgetown University Medical Center, via EurekAlert!, a service of AAAS.
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