21 ms·
Prions, Nearly Indestructible and Universally Lethal, Seed the Eyes of Victims
- growlist 8y agoSlightly worrying given that I had a cataract operation in my mid-30s. OTOH, I suspect I should be far more worried about the health impact of filthy diesel.
- michaelcampbell 8y agoIsn't cataract surgery removing something, only? FWIW, both my parents had cataracts removed, and my father who'd worn eyeglasses since the 1940's said he'd never seen better in his life. So, chin up.
- Invictus0 8y agoYes, but the article says that the instruments used in opthalmology can be vectors for the prions. OP is fine though assuming his operation was more than a year ago and he's not currently dead.
- armitron 8y agoPrion diseases have incubation periods that are very long (5 to 20 years). It is only after symptoms appear that the end is near (a year or less).
- Ma8ee 8y agoThe year is from the onset of the first symptoms. It said nothing about incubation period.
- erichurkman 8y agohttps://www.ncbi.nlm.nih.gov/pubmed/11721058?dopt=Abstract https://www.ncbi.nlm.nih.gov/pubmed/11721058?dopt=Abstract > we estimate that the mean duration of the incubation period is 16.7 years https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(06)69362-8/fulltext https://www.thelancet.com/journals/lancet/article/PIIS0140-6... > A comprehensive statistical analysis had earlier reported that the mean incubation period was between 10.3 and 13.2 years, but the tail of the distribution was long. Using these estimates, we calculated that the upper 0.4% quantile was 40 years, and even larger for those infected young (>50 years), very much in line with these new observations.
- arkades 8y agoNo, it quoted the speculation. > them may then become contaminated, the authors write. They recommend single-use instruments or the adoption of new decontamination procedures for opthalmological equipment with better effectiveness on prions.
- kbutler 8y agocataract surgery involves replacing the lens with a new lens, now generally an artificial lens https://www.allaboutvision.com/conditions/cataract-surgery.htm https://www.allaboutvision.com/conditions/cataract-surgery.h...
- growlist 8y agoSadly I'm blind in that eye - the operation was to allow better monitoring. Other eye is fine, fingers crossed.
- paulpauper 8y agothe odds of this are about zero. the donor would have to have CJD, which is already exceedingly rare.
- growlist 8y agoIt's the surgical instruments I was thinking of. But yes, the risk being exceedingly low was kind of my point when mentioning diesel exhaust - a single lungful of which causes my chest to tighten up!
- andrewingram 8y agoWell that's made me a bit more worried about my cornea transplant next year.
- marsrover 8y agoAs someone with anxiety and eye problems, I'm not feeling great.
- empath75 8y agoYep. I didn’t even finish reading it.
- nso95 8y agoanxeyeity
- mkagenius 8y agoThe top comment sheds some light on the rarity of the disease and the article just being some sort of fear mongering -- https://news.ycombinator.com/item?id=18648393 https://news.ycombinator.com/item?id=18648393
- crankylinuxuser 8y agoIf you don't eat brains, you're pretty much safe. If you eat brains, your risks go up by multiple magnitudes.
- gnulinux 8y agoI wonder if it's limited to brain. If a cattle has prions and if you eat its tissues other than brain, can't you still contact prions via motor neurons?
- pastor_elm 8y agoSounds like PR from the brain lobby.
- katuskoti 8y agoSame here. I've had strange eye problems that my doctor hasn't been able to explain, and I feel like my cognition is declining. It's easy to buy into unknowns like this which seem to explain everything wrong with you, but it's exceedingly unlikely. More likely is that my eye problems are from wearing contacts in for too long and staring too long at a computer in a low-humidity environment and never blinking. My cognitive issues are probably from not eating healthily, not getting enough sleep, not exercising, and from being depressed.
- barking 8y agoAbout 20 or so years ago I was feeling bad about my chances of developing the type of CJD associated with mad cow disease because of all the burgers I'd eaten over the preceding years. There were also human cases getting reported at a rate that some suggested matched the pattern for the early days of an epidemic. Luckily that never materialised. Still though having lived in the UK during a certain time period precludes one from donating blood elsewhere, to this day.
- arkades 8y ago> There were also human cases getting reported at a rate that some suggested matched the pattern for the early days of an epidemic. An epidemic is by definition a statistically significant deviation from historic baselines. Every single upswing within the normal rates of variability - all of the sin curve living above 0 - looks like “early epidemic.” We have one every month. That was scare-mongering, too. Everyone loves to report an incipient disaster, at least until people get numb and the ratings fall down.
- Grazester 8y agoI stopped eating beef from that time due to that mad cow outbreak. A few years before that I was worrying about the Ebola outbreak in Zaire. I was no where near these regions either. As kid and into my teens I was pretty paranoid about disease and death.
- dev_dull 8y agoSorry friend. Not off the hook yet: > patients with very long incubation periods of at least 34 years, and probably more than 40 years. https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(06)69362-8/fulltext https://www.thelancet.com/journals/lancet/article/PIIS0140-6...
- barking 8y agoThe uptick in cases should have started in about 2014 (if 34 years ) or 2020 (if 40 years) and start to tail off a decade later.
- 8y ago
- arkades 8y agoPrion disease is exceedingly rare. CJD is the most common, with an incidence of 1 per 1,000,000 person-years worldwide, inclusive of all transmission vectors (including medical procedures). Iatrogenic transmission is a rarer subset still: the riskiest transplants (with respect to CJD) are in the central nervous system. The UK tracked their transmission rates from 1970 to 2003 (a long period is needed because surgical transmission usually occurs in folks with long incubation periods - up to ten years or so) for dura transplants (something that goes into the CNS) and found a total of eight. This article is one good step above scare-mongering, but only one.
- fallingknife 8y agoInfectious disease is one area where fear of the unknown is rational. We don't just need to defend against existing diseases, but also diseases yet to emerge. Just because CJD is rare doesn't mean that the next prion disease will be, so it would make sense to take measures to prevent transmission of prions now.
- arkades 8y agoWe take measures now, for tissues where prions are a possible transmission risk. The article mis-represented that. But: (1) there are only so many resources to go around. Should we be devoting them to vanishingly rare things rather than things that might actually affect you? (2) prions will never be a wildfire plague. They have little or no tranmissive ability, by nature of being absolutely naked protein. One step up from that - encoding the protein for space constraints and encapsulating it - is called a virus.
- travisoneill1 8y agoThe example in the article has very little transmissive ability because it grows in the brain and eyes. If there were a respiratory prion disease that could be much different. By nature of being a protein they are simpler and harder to eliminate than bacteria or viruses: > In a particularly vexing twist, prions are also nearly impervious to destruction, even when attacked using a strenuous combination of disinfectants, heat, and pressure.
- ryanmercer 8y agoPrions are one of the things that makes me go, hey wait maybe we do live in a simulation. They're basically corrupted code (which we've yet to figure out how they get that way in the first place) that corrupts other code... what is that?!?! Reminds me a lot of the WoW 'corrupted blood incident'. I understand how something like bacteria or a virus enters a host and replicates, but an anomalous protein corrupting other protein is just so strange. I'm sure science will adequately explain it someday but until then it's a pretty WTF anomaly to me.
- rantanplan 8y agoWe do live inside a simulation... of particles, called... the Universe.
- AllegedAlec 8y agoIt's actually relatively simple. Proteins, very roughly speaking, are things that change other things into something we need. You could think of proteins required for breaking down sugar, which do more or less exactly that: they take a molecule of sugar, break a part off of it and hand it to the next protein in the chain. What makes prions so weird is that they are a protein that has been mismade (misfolded, generally). That something is being made broken isn't that weird in biology. Mostly, the body degrades them pretty quickly. However, prions, while not being able to do what they're meant to do, can take well-made versions of itself and break them in the same manner.
- ryanmercer 8y ago>What makes prions so weird is that they are a protein that has been mismade (misfolded, generally). And science doesn't know how they get misfolded in the first place then they basically go "hey other protein, boop you're like me now, let's burn this mother to the ground" and then you get something similar to the 'corrupted blood incident' https://en.wikipedia.org/wiki/Corrupted_Blood_incident https://en.wikipedia.org/wiki/Corrupted_Blood_incident except you lose your mind and die. As far as saying we know how proteins misfold, I'm going purely by what I've read both on and on wikipedia as I'm not an expert in proteins and amino acids... >It is not known what causes the normal protein to misfold Second sentence of: https://en.wikipedia.org/wiki/Prion https://en.wikipedia.org/wiki/Prion
- OldFatCactus 8y agoI had project in college on Prions. Some interesting things I remember: * Kuru (laughing disease) was transmitted from cannibalism. Women and children of the tribe were more likely to contract it because they were more often given the less palatable parts of the bodies, which included brains * Some countries do not trust American cattle because our standards for Prion screening are too low * Kentucky has issues with Prions because Squirrel brains are a popular dish Try not to eat animal or human brains and you can dramatically reduce your chances of getting Prions
- aqme28 8y agoWhy are prion diseases typically brain diseases? Or is that the only organ where they manifest in symptoms?
- cobbzilla 8y agoiirc they reproduce most rapidly in nervous tissue. thus brains have the highest concentration of them.
- SEJeff 8y agoBorn and raised in Kentucky (Lexington). I've never heard of squirrel brains as a dish. That's an interesting tidbit. Would it be fair to guess that the parts of KY this is popular are on the south eastern part of the state on the border with West Virginia? All of the normal rules of everything are different when you're in Appalachia.
- OldFatCactus 8y agoHere's some info from a quick google: http://www.mad-cow.org/~tom/victim23.html http://www.mad-cow.org/~tom/victim23.html Looks like the dish is called Burgoo > The center of the burgoo universe is Owensboro, Kentucky
- SEJeff 8y agoJust when I thought my home state couldn't embarrass me more by being backwards... Thanks!
- gumby 8y agoGiven that prions are proteins, why can't we use immunotherapy and vaccinate?
- Nasrudith 8y agoWell the immune system needs to be able to do something of use in response. Just putting it in even with an immune sensitizing agent would add another prion that it can't do anything useful about. But antibodies, if we had ones that worked for a strain and weren't essentially prions themselves we could vaccinate for it or use it to "chelate" assuming the prion grabbers wouldn't cause other unacceptable problems.
- gumby 8y agoThanks for replying (some people have downvoted my question, which is weird). I don't completely understand your answer, due to my ignorance of the immune system. I thought the immune system could target specific proteins (e.g. how snake venom vaccines work) rather than using the proteins to identify target cells. Is this correct? I as assuming such a mechanism would be able to do something useful with the target. The other part of your answer I did understand, and that was helpful, thanks!
- folli 8y agoIt's definetely being looked at, for example: https://www.ncbi.nlm.nih.gov/pubmed/18215090/ https://www.ncbi.nlm.nih.gov/pubmed/18215090/ (paywalled).
- folli 8y agoPassive immunization might indeed be a feasible option, but of course requires timely detection.
- gumby 8y agoCouldn't we immunize before exposure as we do with, say polio, TB, influenza et al? Then no detection would be needed. Or are the abnormal prions in CJD and the like not uniform enough for general prophylaxis? I really have a naive understanding of immunotherapy, so sorry if my question is dumb.
- technological 8y agoHere is excellent documentary https://www.youtube.com/watch?v=vw_tClcS6To&list=PLdO1m6dMyeyS58vB1wSpnNbc1bhv_N3jd https://www.youtube.com/watch?v=vw_tClcS6To&list=PLdO1m6dMye... anyone wants to see documentary related to Kuru (Prion Disease)
- yourapostasy 8y agoNot all prions are bad [1]. We still need to study them and their protein precursors to understand them more. [1] https://www.the-scientist.com/features/the-bright-side-of-prions-38210 https://www.the-scientist.com/features/the-bright-side-of-pr...
- paulpauper 8y agoIt would seem the solution is to treat deceased CJD victims as bio hazard like ebola
- honestoHeminway 8y agoI guess, this is the weapon of choice for any patient spy unwilling to send a message. One pinbrick, and the nuclear scientist/dissendent/oppossitional spy has half a year to write his/her memoir. Are there statistics on Creuzfeld-Jakobs in Langley?
- arbuge 8y agoThe part about the possibility of contracting these from your doctor's equipment when you go in for an eye exam is truly scary: "Undiagnosed CJD patients may seek testing. And the diagnostic equipment used to test them may then become contaminated, the authors write. They recommend single-use instruments or the adoption of new decontamination procedures for opthalmological equipment with better effectiveness on prions."
- rladd 8y agoThe fear of contracting something from equipment is one reason why I go to an eye doctor who uses contactless air-puff glaucoma diagnostic machines rather than the older style which touches the eye. In my last several visits, nothing has touched my eye at all.
- than 8y agoIf you're interested in prions and some heavy science, I would highly recommend reading the research blogs of Eric and Sonia. http://www.cureffi.org/ http://www.cureffi.org/ and http://www.prionalliance.org/blog/ http://www.prionalliance.org/blog/ > Wife-husband team Sonia Vallabh and Eric Minikel are on a very personal mission to find a cure to prion diseases. When Sonia was diagnosed as a genetic carrier of a rare disease called Fatal Familial Insomnia (FFI), she and Eric quit their regular jobs and began studying molecular biology to learn as much as they could. Theirs is an incredible story so far. https://www.newyorker.com/books/page-turner/a-prion-love-story https://www.newyorker.com/books/page-turner/a-prion-love-sto...
- theprotocol 8y agoThis is pretty inspiring. Thanks for posting it.
- nkurz 8y agoIn related prion news, does anyone have insight into the recent studies suggesting that Chronic Wasting Disease (CWD, increasingly prevalent in North American deer and elk) may be transmissible from infected animals to humans via cooked muscle meat? In 2017, there was a Canadian study that seemed to show that feeding macaque monkeys raw muscle meat from infected deer was capable of transmitting the disease. I don't think the study has been published yet, but it's described in detail in this Norwegian report (https://vkm.no/download/18.25950e0715e84a118a6ec492/1506925175221/Statement%20CWD.pdf https://vkm.no/download/18.25950e0715e84a118a6ec492/15069251...) and forms the basis of an advisory by the Canadian government (https://www.thetyee.ca/Documents/2017/06/24/Risk-Advisory-Opinion-CWD-2017.pdf https://www.thetyee.ca/Documents/2017/06/24/Risk-Advisory-Op...) suggesting that consumption of all potentially infected meat be avoided. Then in 2018, a separate seemingly very similar American study was published (http://sci-hub.tw/10.1128/JVI.00550-18 http://sci-hub.tw/10.1128/JVI.00550-18) , saying that no evidence was found for transmission to macaques, even after direct cerebral exposure. The NIH issued a press release (https://www.nih.gov/news-events/news-releases/nih-study-finds-no-chronic-wasting-disease-transmissibility-macaques https://www.nih.gov/news-events/news-releases/nih-study-find...) with the headline "NIH study finds no chronic wasting disease transmissibility in macaques". Suffice it to say, there seem to be contradictory messages here. Is there some overview that would help to clear things up?
- ff7f00 8y agoI don't have studies but I did email the National Prion Disease Pathology Surveillance Center asking "is there a breakdown of cases of the individual Animal Prion Diseases by location and possibly by year (i.e. cases of human prion disease acquired by eating contaminated meat)?" and here was their response: > To date, in the US, there have been no known cases of variant CJD, or human prion disease acquired by eating contaminated beef. Here at the NPDPSC, we have diagnosed 4 cases, but they were all acquired out side the US; 1 case in 2044 acquired in UK, 2 cases in 2006 acquired in Saudi Arabia, and 1 case in 2014 acquired in the middle east. > In several western US states, Canada, and now South Korea and Norway, there is concern that chronic wasting disease (CWD), the prion disease of elk and deer, may be transmissible to people who hunt, butcher, or eat the affected animals. Although transmission of CWD from animals to humans is unlikely, recent data indicate that the barriers between species may be weakened when CWD has been transmitted from animal to animal several times (as may happen in the wild). The NPDPSC, along with the CDC, Department of Health, and the Game and Wildlife Administration, is carefully monitoring any possible case of acquired CWD in humans. Asked for extra clarification, "So although there is a concern with CWD crossing that barrier, there still haven't been any recorded cases?" > That is correct.
- chc4 8y agoA few months ago I was talking at a family gathering and learned that one of my distant relatives has CJD. They're a high-ranking company executive, but their cognition is rapidly wasting away and will be dead within a few years. Another family member that was a nurse in Kentucky(?) said they have a high rate of prion disease due to farmers inhaling contaminated dirt for years. There are a few comments here about how talking about prion diseases are basically fearmongering. It shook me a bit, though. I always thought of CJD as one of those freak diseases that you hear about in the news, but is such low likely hood that it's basically non-existent. Not so much anymore.
- stef25 8y ago> A few months ago I was talking at a family gathering and learned that one of my distant relatives has CJD. They're a high-ranking company executive, but their cognition is rapidly wasting away and will be dead within a few years Are you sure it's CJD? Article says 95% of CJD patients won't live 12 months after diagnosis. > Another family member that was a nurse in Kentucky(?) said they have a high rate of prion disease due to farmers inhaling contaminated dirt for years. What kind of prion disease and what kind of dirt?
- chc4 8y agoI was told mad cow disease and CJD interchangably, which looking it up seems to be slightly different. I don't know the difference between CJD and vCJD and Wikipedia isn't helping much. Honestly, I don't know how fast it's progressing, so it may in fact be months. They may already be dead for all I know, but it sounded like they were diagnosed several months before - they don't know how long ago she got it though, due to the long incubation. The family member mentioned mood swings, improper outbursts in conversation, and memory degeneration and how they're nearly bedbound now due to loss of balance. The nurse family member said cow farmers, so I assume cow feces contaminated dirt, and that their brains looked "like Swiss cheese". This was also in the context of mad cow disease. I checked my notes from the day and it was North Carolina, not Kentucky.
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