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Doctors later discovered that Stephen had succumbed to a new kind of killer, the prion, now known to be the cause of mad cow disease in cattle, chronic wasting disease in American deer and elk, and Creutzfeldt-Jakob disease and fatal insomnia in humans, among other exotic ailments. Doctors and researchers have been aware of some of these diseases for a century and more, but only in the last two decades have scientists even begun to understand just how the pathological protein spreads to new species and kills its victims.
In this timely and intriguing book, Philip Yam describes the history of the scientific effort to track down and understand the prion, and the medical effort, still underway, to devise treatments for those who suffer from its ravages.
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The first chapter of The Pathological Protein describes, from a very human perspective, the effects of variant Creutzfedt-Jakob disease on one victim, 19 year-old Stephen Churchill, and his family. From this tragedy, Yam then goes on to review the history of CJD and the mysterious diease 'kuru', which reached epidemic proportions amongst the Fore people of Papua-New Guinea because of their cannibalistic funerary rites. After discussing the hereditary transmissible spongiform encephalopathies (TSEs) of humans, outlines what is known of the TSEs of animals. Philip Yam's reviews of scrapie, BSE, transmissible mink encephalopathy and chronic wasting disease are up-to-date, interesting, and extremely readable.
There is an interesting episode related in the book. Carlton Gajdusek had been searching, unsuccesfully, for the cause of kuru. William Hadlow, and American scrapie researcher on a secondment to the United Kingdom, visited the Wellcome Medical Museum in London to look at a display on kuru that Gajdusek had prepared. It was Hadlow who first noticed the very close resemblance between kuru and scrapie. The similarities in epidemiologic features, general clinical pattern and the neurohistologic changes led him to the realisation that these diseases were probably mmebers of the same family. As a result of Hadlow's insight transmission experiments were started which, eventually, led to our current understanding of the TSEs
This book covers the hypotheses for the origins of BSE, the evidence for the link between BSE and vCJD, current methods and problems of diagnosis of the TSEs, and the search for cures. Philip Yam clearly is thoroughly versed in the scientific literature of the TSEs, but also interviewed a broad range of scientists, consumers advocates and regulators. So, he knows what he is writing about, and this is made evident by the clarity and accuracy of his explanations. Although there is no 'dumbing down' of a difficult and complex subject, the author has written a book which makes his subject easily accessible to the non-specialist reader. The book is referenced, well indexed, has a useful glossary and also suggests sources for further information, including the more useful web sites and organisations providing suport and help for families of CJD victims. While the book is written for the interested lay person, I would have no hesitation in recommending Philip Yam's The Pathological Protein to veterinarians and colleagues who want an interesting, thorough and current review of these fascinating diseases.
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