CC: lcamp@BMJgroup.com
References: <001501c21099$5c8bc620$7c58d182@mfacdean1.cent.gla.ac.uk>
Greetings again Professor Farthing and BMJ,
I was curious why my small rebuttal of the article described below was not listed in this month's journal of GUT? I had thought it was going to be published, but I do not have full text access. Will it be published in the future? Regardless, I thought would pass on a more lengthy rebuttal of mine on this topic, vCJD vs sCJDs and endoscopy equipment. I don't expect it to be published, but thought you might find it interesting, i hope you don't mind and hope to hear back from someone on the questions I posed...
Here is my short submission I speak of, lengthy one to follow below that:
Date submitted: 3 Jun 2002
>> eLetter ID: gutjnl_el;21
>> >> Gut eLetter for Bramble and Ironside 50 (6): 888
>> >>Name: Terry S. Singeltary Sr. >>Email: flounder@wt.net
>>Title/position: disabled {neck injury}
>>Place of work: CJD WATCH
>>IP address: 216.119.162.85
>>Hostname: 216-119-162-85.ipset44.wt.net
>>Browser: Mozilla/5.0 (Windows; U; Win98; en-US; rv:0.9.4)
>>Gecko/20011019 Netscape6/6.2
>> >>Parent ID: 50/6/888
>>Citation:
>> Creutzfeldt-Jakob disease: implications for gastroenterology
>> M G Bramble and J W Ironside
>> Gut 2002; 50: 888-890 (Occasional viewpoint)
>> http://www.gutjnl.com/cgi/content/abstract/50/6/888
>> http://www.gutjnl.com/cgi/content/full/50/6/888
>>-----------------------------------------------------------------
>>"CJDs (all human TSEs) and Endoscopy Equipment"
>>----------------------------------------------------------------- >> >> >>
>>
regarding your article; >>
>>
Creutzfeldt-Jakob disease: implications for gastroenterology >>
>>
I belong to several support groups for victims and relatives
>>of CJDs. Several years ago, I did a survey regarding
>>endoscopy equipment and how many victims of CJDs have
>>had any type of this procedure done. To my surprise, many
>>victims had some kind of endoscopy work done on them.
>>As this may not be a smoking gun, I think it should
>>warrant a 'red flag' of sorts, especially since data now
>>suggests a substantial TSE infectivity in the gut wall
>>of species infected with TSEs. If such transmissions
>>occur, the ramifications of spreading TSEs from
>>endoscopy equipment to the general public would be
>>horrible, and could potential amplify the transmission
>>of TSEs through other surgical procedures in that
>>persons life, due to long incubation and sub-clinical
>>infection. Science to date, has well established
>>transmission of sporadic CJDs with medical/surgical
>>procedures.
Terry S. Singeltary Sr. >>CJD WATCH
Again, many thanks, Kindest regards,
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 flounder@wt.net CJD WATCH
[scroll down past article for my comments]
Subject: Creutzfeldt-Jakob disease: implications for gastroenterology & CJD 38 years after _diagnostic_ use of hGH (Iatrogenic CJDs & sporadic CJDs)
Date: Mon, 17 Jun 2002 16:46:46 -0700
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@uni-karlsruhe.de
Bovine Spongiform Encephalopathy
OCCASIONAL VIEWPOINTS
Creutzfeldt-Jakob disease' implications for gastroenterology
M G Bramble, J W Ironside
Gut 2002;50:888-890
The current clinical views regarding variant Creutzfeldt-Jakob disease, and in particular transmission via endoscopy, of those representing both gastroenterology and the Spongiform Encephalopathy Advisory Committee are presented in an attempt to guide clinicians as to "best practice" given the current state of our knowledge.
See end of article for authors' affiliations
Correspondence to: Professor MG Bramble, Endoscopy Centre, James Cook University Hospital, Marton Rd, Middlesbrough TS4 3BN, UK;
Most gastroenterologists working in the UK have been aware for some time that endoscopy may be a vector for the transmission of prions from a patient incubating, but not clinically manifesting, variant Creutzfeldt-Jakob disease (vCJD) to the next individuals undergoing the same procedure on the same list. To date there are no recorded cases of iatrogenic transmission of vCJD via endoscopy but it remains a risk which will be present for many years to come. Advice to health authorities on individual cases is through the CJD Incidents Panel. However, we are aware that advice to health professionals performing endoscopy needs to be as comprehensive as current evidence will allow, without making it impossible to perform endoscopic procedures on patients who will clearly derive long term health benefits from an accurate endoscopic diagnosis and/or treatment. This article represents the current clinical views of those representing both gastroenterology and the Spongiform Encephalopathy Advisory Com-mittee (SEAC). Both authors sit on the CJD Inci-dents Panel and have been advising the Depart-ment of Health on individual cases during the last year. It is important to note that the advice given in this article may be superseded if additional information or evidence becomes available.
CJD is a member of a group of neurological disorders known as the transmissible spongilorm encephalopathies or prion diseases, which affect both animals (such as scrapie in sheep or bovine spongiform encephalopathy (BSE) in cows) and humans. The precise nature of the transmissible agents responsible for these disorders is unknown but there is increasing evidence to support the prion hypothesis, which states that the agent is composed of an abnormally folded form of a host encoded protein, prion protein. The normal prion protein (PrPc) is expressed in many tissues but occurs at the highest levels in neurones in the central nervous system (CNS) where it may act as a copper binding protein, although its precise physiological role is unknown. The abnormal form of the protein (PrPSc) accumulates in the CNS in prion diseases; the infectious agent is remarkably resistant to most forms of degradation. The association between PrPSc and the gut has been eloquently described in a previous lead-ing article1 and gastroenterologists need to understand where we are in terms of our present day knowledge of this entity.
In humans, prion diseases occur in three major categories: sporadic, acquired, and familial. All are currently untreatable and universally fatal although recent studies have indicated that a combination of drugs may be effective in experimental prion diseases2: this approach is under consideration as a clinical trial. The sporadic form of CJD affects approximately one person per mil-lion per annum in the population on a worldwide basis. CJD has also occurred as an acquired iatrogenic disorder, transmitted to other humans through direct (inadvertent) inoculation of the brain via contaminated neurosurgical instruments, via corneal and dura mater grafts, or through administration of human pituitary ex-tracts used to treat growth hormone or gonadotrophin deficiency. Variant CJD (vCJD) is a new acquired form of CJD which was first reported in 1996 affecting mainly young adults and with a unique neuropathological phenotype.3 It is now widely accepted that bovine prions passed into the human population through consumption of BSE infected bovine tissues; the transmissible agent responsible for vCJD is identical to the BSE agent (but different from the agent in sporadic CJD). The incubation period for vCJD is likely to be lengthy and may have a mean value of 10-30 years. During this time the affected person has the potential to transmit the disease to others via surgical procedures which might result in the transfer of infected tissue into the next person operated on with the same surgical instruments.
The distribution of PrPSc in the body is different in sporadic and variant CJD, reflecting the differ-ent pathogenesis of the two forms. In the case ot sporadic CJD, prion infectivity is largely limited to the CNS (including the retina) and only opera-tions involving the brain and eye have resulted in iatrogenic transmission of the disease. Gastro-intestinal endoscopy is unlikely to be a vector for the transmission of sporadic CJD as infected tissue is not encountered during the procedure. No special precautions are necessary during or after the procedure and the endoscope should be cleaned and disinfected in the normal thorough way.4
"Endoscopy on patients who are incubating vCJD may result in exposure of the instrument (and particularly the biopsy forceps) to PrPsc''
In contrast, in vCJD the lymphoreticular system throughout the body contains PrPSc at the time of death, and experimental evidence suggests that the lymphoreticular system may contain significant levels of infectivity for most of the incuba-tion period.5 To support this, in vCJD abnormal prion protein was found in the germinal centres in the wall of an appendix from a vCJD patient that was removed eight months before the onset of neurological disease.6 As lymphoid follicles and germinal centres are widely distributed in the gastrointestinal tract (and are often biopsied), it is possible that endoscopy on patients who are incubating vCJD may result in exposure of the instrument (and particularly the biopsy forceps) to PrPsc. Consequently, the question now arises, how great is the risk of secondary (person to person) transmission in endoscoping a patient incubating vCJD? There are three scenarios which gastroenterologists are likely to encounter and this editorial will attempt to guide clinicians as to "best practice" given the current state of our knowledge.
UPPER GASTROINTESTINAL ENDOSCOPY
Scenario No 1
Occasionally gastroenterologists may be requested to endo-scope a patient with known or probable sporadic CJD (usually to site a PEG feeding tube). This can be carried out in the rou-tine way provided vCJD is not suspected. If inadvertently a patient with suspected vCJD is endoscoped, the instrument used should be quarantined until the postmortem diagnosis is known. If sporadic CJD is diagnosed, the endoscope can be returned to use following thorough cleaning and decontami-nation, as is normal practice. If vCJD is diagnosed the endoscope cannot be used again and should be quarantined or sent to the National CJD Surveillance Unit in Edinburgh for research purposes. The previous advice to destroy such instru-ments represents a lost opportunity to study the risks involved in more detail. It would also be good practice to inform colleagues locally that a quarantined instrument was available for use in other endoscopy units if they too had a patient with suspected vCJD requiring endoscopy.
Scenario No 2
For patients with known or probable vCJD,7 endoscopy should only be a last resort. Ultrasound guided insertion of a gastrostomy feeding tube would be preferable to a PEG feeding tube if local expertise is available. If not, endoscopy should be per-formed using an instrument already set aside for such patients. If no such instrument is available locally, one can be loaned to any hospital by the National CJD Surveillance Unit in Edinburgh (contact telephone number 0131 537 1980). If scenario No 2 becomes more common, endoscopes may need to be held regionally for this purpose.
Scenario No 3
This scenario covers patients who have been endoscoped by an instrument previously used on a patient who was not known to be incubating vCJD at the time of endoscopy but who sub-sequently went on to develop the disease. This could become the commonest scenario and it must be assumed that the patient who went on to develop vCJD was incubating the dis-ease at the time of the original endoscopy. This also means that infectious material may not have been removed completely by current methods of decontaminating endoscopes, and that subsequent patients have been exposed to the prion agent. The instrument used should therefore be quaran-tined until advice has been sought from the CJD Incidents Panel (Department of Health, Skipton House, London; contact telephone 0207 972 1761) as to the management of the situa-tion. Local infection control teams will need to be involved with contact tracing and information handling.
LOWER GASTROINTESTINAL ENDOSCOPY
It is unlikely that colonoscopy would be clinically justifiable in a patient known or strongly suspected as suffering from vCJD. However, it is quite possible that an asymptomatic patient incubating vCJD may undergo colonoscopy prior to diagnosis and this situation is essentially the same as in scenario 3. The risks of transmitting prion protein to the next patient are much greater however, due to a number of factors which relate to the amount of lymphatic tissue encountered during endos-copy and the number, site, and size of mucosal biopsies obtained by this method.
In general the risks of transmitting vCJD from one patient to another are dependent on the infectivity of the tissues involved, the amount of tissue contaminating the instrument, the effectiveness of the decontamination processes, and the susceptibility of subsequently exposed patients. Experimental studies suggest that levels of infectivity in prion diseases are highest in the CNS and retina, which are approximately two logs higher than in the tonsils and other lymphoreticular tis-sue. A recent study has also detected the abnormal form of the prion protein in rectal tissue from a patient with vCJD by western blot examination of autopsy tissues.8 The risk of transmitting vCJD through the endoscopy procedure itself is likely to be small, but contamination of the endoscope and forceps as a result of biopsy of lymphoid tissues may represent a larger (but currently unquantifiable) risk, even though only small amounts of tissue are involved.
"The risks of transmitting vCJD from one patient to another are dependent on the infectivity of the tissues involved, the amount of tissue contaminating the instrument, the effectiveness of the decontamination processes, and the susceptibility of subsequently exposed patients"
The greatest risk is undoubtedly that which ensues from biopsy of the terminal ileum where Peyer's patches may con-tain significant levels of prion protein for a patient incubating vCJD. The biopsy forceps and the colonoscope become poten-tial vectors for disease transmission under these circum-stances. Meticulous manual cleaning of the colonoscope is probably the best defence against person to person transmis-sion. The same is true of the biopsy forceps, but as disposable forceps are now available there is a strong argument for mov-ing towards the universal use of disposable biopsy forceps for mucosal samples taken at colonoscopy. Endoscopy units should now work towards a policy of using disposable biopsy forceps as the only practical way of minimising the risk which results from ileal biopsy. In addition, "random" biopsies should be kept to a minimum as lymphoid tissue is distributed widely throughout the gastrointestinal tract. Although thor-ough cleaning of flexible endoscopes ensures patient safety for "normal" pathogens, the same process may not be adequate for the PrPsc. The main benefit of the decontamination process under these circumstances is undoubtedly effective manual cleaning, as glutaraldehyde may stabilise PrPSc on the metal surface of the endoscope, with potentially adverse conse-quences. It follows that brushes used to clean the channels of the endoscope are used only once to ensure maximum efficiency and biopsy forceps should also be functioning opti-mally and discarded as soon as they appear to be under performing (tearing tissue rather than cutting it). The rubber valve protecting the biopsy channel is another item which is potentially disposable and serious consideration should be given to single use valves. Again, more research is required to determine "best practice". For rigid endoscopes, autoclaving at the recommended conditions for CJD9 is the best way of attempting decontamination.
What should endoscopists do in the short term? The answer to this question must be to ensure as far as possible that manual cleaning of endoscopes and reuseable accessories is of the highest standard. Endoscopy has a major role in patient care, and this should not be compromised unless it is absolutely unavoidable in the public interest. It is also essen-tial that endoscopes should be individually identifiable and their use traceable in any given patient population. Random biopsies should be kept to an absolute minimum (particularly of the ileum in colonoscopy) and endoscopy itself should be as atraumatic as possible, especially gastroscopy where the instrument is in contact with the mucosa covering the tonsils. Biopsy forceps should be treated as "high risk" and undergo thorough ultrasonic cleaning followed by autoclaving. As research in the UK progresses, it is likely that other procedures will be developed to inactivate prion infectivity and to remove proteins from instrument surfaces. The development of such techniques (along with more sensitive tests for prion detection) may well have an impact on future advice concern-ing endoscopy and CJD.
Depending on the final numbers of people infected with vCJD, we must assume that a significant number may undergo endoscopy before neurological symptoms appear10. It is there-fore up to every endoscopist to be aware of the dangers and follow the advice set out here. Further advice on specific cases and possible exposure incidents can be obtained from the CJD Incidents Panel (Department of Health, Skipton House, London; contact telephone 0207 972 1761).
Authors' affiliations M G Bramble, Endoscopy Centre, James Cook University Hospital, Marton Road, Middlesbrough TS4 3BW, UK J W Ironside, CJD Surveillance Unit, Western General Hospital, Crewe Road, Edinburgh EH4 2XU, UK
mike.bramble@stees.nhs.uk
Accepted for publication 19 November 2001
REFERENCES
1 Shmakov AN, Ghosh S. Prion proteins and the gut: une liaison dangereuse? Gut 2001;48:443-7.
2 Korth C, May BCH, Cohen FE, et al. Acridine and phenothiazine derivatives as pharmacotherapeutics for prion disease. Proc Nail Acad Sci USA 2001;98:9836-41.
3 Will RG, Ironside JW, Zeidler M, et al. A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 1996;347:921-5.
4 Report of a Working Party of the British Society of Gastroenterology Endoscopy Committee. Cleaning and disinfection of equipment for gastrointestinal endoscopy. Gut 1998;42:585-93.
5 Hill AF, Butterworth R J, Joiner S, et al. Investigation of variant Creutzfeldt-Jacob disease and other human prion diseases with tonsil biopsy samples. Lancet 1999;353:183-9.
6 Hilton DA, Fathers E, Edwards P, et al. Prion immunoreactivity in the appendix before the clinical onset of new variant Creutzfeldt-Jacob disease. Lancet 1998;352:703-4.
7 Will RG, Zeidler M, Stewart GE, et al. Diagnosis of new variant Creutzfeldt-Jakob disease. Ann Neuro12000;47:575-82.
8 Wadsworth JD, Joiner S, Hill AF, et al. Tissue distribution of protease resistant prion protein in variant Creutxfleldt-Jakob disease using a highly sensitive immunoblotting assay. Lancet 2001 ;358:171-80.
9 Dangerous Pathogens Spongiform Encephalopathy Advisory Committee. Transmissible spongiform encephalopathy agents: safe working and the prevention of infection. London: The Stationary Office, 1998.
10 Ironside JW, Hilton DA, Ghani A, et al. Retrospective study of prion protein accumulation in tonsil and appendix tissue. Lancet 2000;355:1693-94.
http://www.gutjnl.com/cgi/content/abstract/50/6/888
========================================================
Greetings List Members,
This is _very_ disturbing to me:
snip...
The distribution of PrPSc in the body is different in sporadic and variant CJD, reflecting the different pathogenesis of the two forms. In the case ot sporadic CJD, prion infectivity is largely limited to the CNS (including the retina) and only operations involving the brain and eye have resulted in iatrogenic transmission of the disease. Gastro-intestinal endoscopy is unlikely to be a vector for the transmission of sporadic CJD as infected tissue is not encountered during the procedure. No special precautions are necessary during or after the procedure and the endoscope should be cleaned and disinfected in the normal thorough way.4
snip...
i personally believe it is irresponsible for anyone to state in this day and time, that sporadic CJDs (now at 6 variants) will not transmit the disease by this route. considering infective dose cannot be quantified, only speculated, such a statement is thus, irresponsible. to hypothosize that sporadic CJD just happens spontaneously (with no scientific proof), that the PrPSc distribution in tissues of all sporadic CJDs is entirely different than that of vCJD, without being able to quantify the titre of infection, or even confirm all the different variants yet, again is _not_ based on all scientific data, then it's only a hypothosis. who is to say that some of these variants of sporadic CJD were not obtained _orally_?
also stated:
snip...
Although thorough cleaning of flexible endoscopes ensures patient safety for ''normal'' pathogens, the same process may not be adequate for the PrPSc.
snip...
The sporadic form of CJD affects approximately one person per mil-lion per annum in the population on a worldwide basis.
who is to say how much infectivity are in some of these variants of sporadic CJDs, without confirming this? if we look at the 6 different variants of sporadic CJDs, has the infective dose for all 6 _documented_ variants been quantified, and documented as being 'measurable'?
will there be more variants of sporadic CJDs, and what of the ramifications from them?
what of other strains/variants of TSE in cattle, BSE in sheep, CWD in cattle, or any of the 20+ strains of Scrapies in deer/elk? i get dizzy thinking of the different scenerio's. what would the human TSEs from these species look like and how can anyone quantify any tissue infectivity from these potential TSE transmissions to humans, and the risk scenerio described here from this potential route? could not some of these sporadic CJDs have derived directly or indirectly from one of these species, and if so, pose a risk by the route described here?
something else to consider, in the recent finding of the incubation period of 38 years from a _small_ dose of human growth hormone;
snip...
We describe the second patient with hGH related CJD in the Netherlands. The patient developed the disease 38 years after hGH injections. To our knowledge, this is the longest incubation period described for any form of iatrogentic CJD. Furthermore, our patient was _not_ treated with hGH, but only received a _low_ dose as part of a diagnostic procedure. (see full text below).
snip...
so my quesion is, how low is 'low' in quantifing the infectious dose in vCJD, comparing to _all_ sporadic CJDs, from the different potential routes, sources, and infectivity dose?
will the titre of infectivity in every tissue and organ of all sporadic CJDs stay exact or constant, no matter what the infective dose, route and species may be? this is considering you don't buy the fact that sporadic CJDs 85%+ of _all_ CJDs, are a happen stance of bad luck, happen spontaneously without cause, and are one-in-a-million world wide, with no substantial surveillance to confirm this.
Diagnosis and Reporting of Creutzfeldt-Jakob Disease T. S. Singeltary, Sr; D. E. Kraemer; R. V. Gibbons, R. C. Holman, E. D. Belay, L. B. Schonberger
http://jama.ama-assn.org/issues/v285n6/ffull/jlt0214-2.html
and what of Dr. Prusiner et al recent work about tissue infectivity;
Prions in skeletal muscle
snip...
Our data demonstrate that factors in addition to the amount of PrP expressed determine the tropism of prions for certain tissues. That some muscles are intrinsically capable of accumulating substantial titers of prions is of particular concern. Because significant dietary exposure to prions might occur through the consumption of meat, even if it is largely free of neural and lymphatic tissue, a comprehensive effort to map the distribution of prions in the muscle of infected livestock is needed. Furthermore, muscle may provide a readily biopsied tissue from which to diagnose prion disease in asymptomatic animals and even humans.
snip...
http://www.pnas.org/cgi/content/abstract/99/6/3812?maxtoshow=&HITS=10&hits= 10&RESULTFORMAT=&author1=prusiner&author2=prusiner&titleabstract=prions+meat+ tissue+mice&fulltext=prions+meat+tissue+mice&searchid=1024346978866_6016&stored_ search=&FIRSTINDEX=0&fdate=1/1/2002
can the science/diagnostic measures used to date, measure this, and at the same time guarantee that no titre of infectivity exists from sporadic CJDs (all of the variants), from this potential mode and route of transmission?
i don't think so, this is just my opinion. this is why i get paid nothing, and these scientists get the big bucks. i just hope i am wrong and the big bucks are correct in their _hypothisis_ of this potential mode/route of transmission with endoscopy equipment, from _all_ human TSEs.
i understand we have to weigh the risks of what we know to what we don't know, to the disease we _may_ catch to what we are having the procedure for, but to categorically state at this present time of scientific knowledge;
snip...
"Gastro-intestinal endoscopy is unlikely to be a vector for the transmission of sporadic CJD as infected tissue is not encountered during the procedure. No special precautions are necessary during or after the procedure and the endoscope should be cleaned and disinfected in the normal thorough way.4"
snip...
but, to categorically state this, in my opinion, is not only wrong, but potentially very dangerous to the future of human health...TSS
SHORT REPORT
Creutzfeldt-Jakob disease 38 years after diagnostic use of human growth hormone
E A Croes, G Roks, G H Jansen, P C G Nijssen, C M van Duijn ...............................................................
J Neurol Neurosurg Psychiatry 2002;72:792-793
A 47 year old man is described who developed pathology proven Creutzfeldt-Jakob disease (CJD) 38 years after receiving a low dose of human derived growth hormone (hGH) as part of a diagnostic procedure. The patient presented with a cerebellar syndrome, which is compatible with iatrogenic CJD. This is the longest incubation period described so far for iatrogenic CJD. Furthermore, this is the first report of CJD after diagnostic use of hGH. Since the patient was one of the first in the world to receive hGH, other cases of iatrogenic CJD can be expected in the coming years.
Prion diseases are potentially transmissible. Human to human transmission was first reported in 1974, when a 55 year old woman was described who developed symptoms of Creutzfeldt-Jakob disease (CJD) 18 months after a corneal transplant.1 Since then, transmission has been reported after stereotactic electroencephalographic (EEG) depth recording, human growth hormone (hGH) and gonadotrophin treatment, and dura mater transplantation.2-5 More than 267 patients with iatrogenic CJD are known today and their number is growing.6 The most important iatrogenic cause of CJD is still contaminated cadaveric hGH. Exposure to contaminated hGH occurred before 1985, when recombinant growth hormone became available. In a recent study, incubation periods in 139 patients with hGH associated CJD were found to range from 5-30 years, with a median of 12 years.6 One of the factors influencing incubation time is genotype on polymorphic codon 129 of the prion protein gene.7 The incubation time is significantly shorter in people who are homozygous for either methionine or valine on this polymorphism.7
We describe the second patient with hGH related CJD in the Netherlands. The patient developed the disease 38 years after hGH injections. To our knowledge, this is the longest incubation period described for any form of iatrogenic CJD. Further-more, our patient was not treated with hGH but only received a low dose as part of a diagnostic procedure.
CASE REPORT
This patient presented at the age of 47 years with paraesthesia in both arms for six months, difficulty with walking for four weeks, and involuntary movements of mainly the upper extremities of two weeks' duration. He did not notice any change in cognitive function, although his twin sister had noticed minor memory disturbances. There was no family history of neurological disease. During childhood the patient had experienced a growth delay compared with his twin sister and with the average in the Netherlands. When he was 9 years old, a nitrogen retention test with 6 IU hGH over five days was performed to exclude growth hormone deficiency. Since the result was not decisive, a quantitative amino acid test was performed, which measures 30 amino acids during fasting and one, two, and three hours after growth hormone injection. No abnormal amino acid concentrations were found making the diagnosis of primordial dwarfism most likely. Therefore, no treatment with hGH was given.
On neurological examination we found a slight dysarthria without aphasia. Cranial nerve function was normal. Walking was unstable and wide based. During movements of the upper extremities myoclonic jerks were present. Sensation, muscle tone, and strength were normal. Co-ordination was impaired in all four limbs with a disturbed balance. Tendon reflexes were brisk at the arms and increased at the legs with a clonus in the ankle reflex. Plantar responses were both normal. On the mini mental state examination, the patient scored 30/30. Routine laboratory investigation, thyroid function, vitamin concentrations (B-1, B-6, B-12, and E), and copper metabolism were normal. Admission EEG examination showed generalised arrhythmic slow activity with diffuse spikes and spike waves. EEG examination two months later showed a further slowing of the rhythm with bilateral diphasic sharp waves but was not typical for CJD. Cerebral magnetic resonance imaging was normal. Cerebrospinal fluid examination showed 1 cell/3 µl, normal glucose and protein concentrations, and a strongly positive 14-3-3 protein test. The patient was homozygous for methionine on the PRNP codon 129 polymorphism. On clinical grounds, CJD was diagnosed. Within one month the patient's condition deteriorated rapidly and because of severe disturbances in coordination and progressive myoclonus he became bedridden. An eye movement disorder developed with slow saccadic and dysmetric eye movements. Temperature became unstable with peaks of 39°C without an infectious focus, for which a disorder of autoregulation was presumed. Until a very advanced stage, cognitive function was intact. The patient died five months after admission. The diagnosis of CJD was confirmed at necropsy. The brain weighed 990 g and showed clear cortical and cerebellar atrophy. Spongiosis, neuronal loss, and gliosis were found predominantly in the putamen, caudate nucleus, and basotemporal and cerebellar cortex; the cerebellum was the most severely affected of these. Vacuoles ranged from 2-12 µm. No amyloid or Kuru plaques were found. Immunohistochemical staining (3F4 antibody 1:1000, Senetek, USA) was clearly positive for prion protein accumulation in a "synaptic" distribution. Most deposition was found in the stratum moleculare of the cerebellum.
DISCUSSION
We describe a 47 year old patient who developed pathology proven CJD 38 years after hGH injections. The patient was never treated with hGH but received a small dose as part of a diagnostic procedure. The onset of CJD was signalled by prodromal symptoms of paraesthesia followed by a rapidly progressive ataxia. The disease presentation and course with predominantly cerebellar and eye movement disorders are compatible with iatrogenic CJD caused by hGH treatment.6 8
Growth hormone treatment was first described in 1958 but hGH was not produced on a larger scale from human pituitary glands until the beginning of the 1960s. In the Netherlands growth hormone extraction started in 1963 and was soon centrally coordinated. Until 1979 growth hormone was extracted non-commercially from pituitaries by a pharmaceutical company. In 1971 commercial products also became available. Our patient was one of the first to receive hGH in the Netherlands but the origin of this product was not recorded. A causal relation can therefore not be established with full certainty, but coincidentally receiving growth hormone and developing this very rare disease is unlikely. Since the clinical course in this relatively young patient is in accordance with an iatrogenic cause, we think the probability is high that the hGH injections explain the development of CJD in this patient.
The first Dutch patient with hGH related CJD died in 1990. 9 During several periods from 1963 to 1969 she received intramuscular injections of hGH. During an unknown period the hGH was derived from South America. At age 39, 27 years after starting the treatment, she developed an ataxic gait, slurred speech, sensory disorders, and myoclonus, but her cognitive function remained normal. Postmortem examination of the brain confirmed the diagnosis of CJD.9 Following the identification of this patient, a retrospective study was started to trace all 564 registered hGH recipients who were treated before May 1985. Until January 1995, none of these was suspected of having CJD.10 Since 1993 prospective surveillance for all forms of human prion disease has been carried out in the Netherlands and, apart from the patient described above, a further two patients with iatrogenic CJD have been identified, who developed the disease after dura mater transplantation.11
An incubation period as long as 38 years had never been reported for iatrogenic CJD. Huillard d'Aignaux et al7 studied the incubation period in 55 patients with hGH related CJD in a cohort of 1361 French hGH recipients. The median incubation period was between 9 and 10 years. Under the most pessimistic model, the upper limit of the 95% confidence interval varied between 17 and 20 years. Although the infecting dose cannot be quantified, it can be speculated that the long incubation period in our patient is partly explained by the administration of a limited amount of hGH. This hypothesis is supported by experimental models, in which higher infecting doses usually produce shorter incubation periods.6 Since our patient was one of the first in the world to receive hGH, this case indicates that still more patients with iatrogenic CJD can be expected in the coming years. Another implication of our study is that CJD can develop even after a low dose of hGH. This case once more testifies that worldwide close monitoring of any form of iatrogenic CJD is mandatory.
ACKNOWLEDGEMENTS
We are grateful to M Jansen PhD MD for his search for the origin of the growth hormone and P P Taminiau MD. CJD surveillance in the Netherlands is carried out as part of the EU Concerted Action on the Epidemiology of CJD and the the EU Concerted Action on Neuropathology of CJD, both funded through the BIOMED II programme, and is supported by the Dutch Ministry of Health. This surveillance would not have been possible without the cooperation of all Dutch neurologists and geriatricians. ........................................
Authors' affiliations
E A Croes, G Roks*, C M van Duijn, Genetic Epidemiology Unit, Department of Epidemiology and Biostatistics, Erasmus University Medical Centre Rotterdam, PO Box 1738, 3000 DR Rotterdam, Netherlands
P C G Nijssen, Department of Neurology, St Elisabeth Hospital, PO Box 90151, 5000 LC Tilburg, Netherlands
G H Jansen, Department of Pathology, University Medical Centre Utrecht, Heidelberglaan 100, 3584 CX Utrecht, Netherlands
*Also the Department of Neurology, St Elisabeth Hospital
Correspondence to: Professor C M van Duijn, Genetic Epidemiology Unit, Department of Epidemiology and Biostatistics, Erasmus University Medical Centre Rotterdam, PO Box 1738, 3000 DR Rotterdam, Netherlands; vanduijn@epib.fgg.eur.nl
Received 27 December 2001 In revised form 1 March 2002 Accepted 12 March 2002
Competing interests: none declared
REFERENCES
1 Duffy P, Wolf J, Collins G, et al. Possible person-to-person transmission of Creutzfeldt-Jakob disease. N Engl J Med 1974;290:692-3.
2 Bernoulli C, Siegfried J, Baumgartner G, et al. Danger of accidental person-to-person transmission of Creutzfeldt-Jakob disease by surgery. Lancet 1977;i:478-9.
3 Koch TK, Berg BO, De Armond SJ, et al. Creutzfeldt-Jakob disease in a young adult with idiopathic hypopituitarism: possible relation to the administration of cadaveric human growth hormone. N Engl J Med 1985;313:731-3.
4 Cochius JI, Burns RJ, Blumbergs PC, et al. Creutzfeldt-Jakob disease in a recipient of human pituitary-derived gonadotrophin. Aust NZ J Med 1990;20:592-3.
5 Thadani V, Penar PL, Partington J, et al. Creutzfeldt-Jakob disease probably acquired from a cadaveric dura mater graft: case report. J Neurosurg 1988;69:766-9.
6 Brown P, Preece M, Brandel JP, et al. Iatrogenic Creutzfeldt-Jakob disease at the millennium. Neurology 2000;55:1075-81.
7 Huillard d'Aignaux J, Costagliola D, Maccario J, et al. Incubation period of Creutzfeldt-Jakob disease in human growth hormone recipients in France. Neurology 1999;53:1197-201.
8 Billette de Villemeur T, Deslys JP, Pradel A, et al. Creutzfeldt-Jakob disease from contaminated growth hormone extracts in France. Neurology 1996;47:690-5.
9 Roos RA, Wintzen AR, Will RG, et al. Een patiënt met de ziekte van Creutzfeldt-Jakob na behandeling met humaan groeihormoon. Ned Tijdschr Geneeskd 1996;140:1190-3.
10 Wientjens DP, Rikken B, Wit JM, et al. A nationwide cohort study on Creutzfeldt-Jakob disease among human growth hormone recipients. Neuroepidemiology 2000;19:201-5.
11 Croes EA, Jansen GH, Lemstra AF, et al. The first two patients with dura mater associated Creutzfeldt-Jakob disease in the Netherlands. J Neurol 2001;248:877-81.
re-CJD after diagnostic use of human growth hormone
from a donor sourcing aspect, seems the record keeping here has a lot to be desired for, let us hope it has improved for recipients sake.
also, they speak of 'low dose fitting long incubation'. what about KURU still existing after some 40 years exposure had ceased. i don't believe in most instances the dose with kuru is low. just something else to ponder?
TSS
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=12023427&dopt=Abstract
1: Ann Neurol 1999 Aug;46(2):224-33
Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects.
Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.
snip...
The present data demonstrate the existence of six phenotypic variants of sCJD. The physicochemical properties of PrP(Sc) in conjunction with the PRNP codon 129 genotype largely determine this phenotypic variability, and allow a molecular classification of the disease variants.
snip...
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10443888&dopt=Abstract
were not all CJDs, even nvCJD, just sporadic, until proven otherwise?
Terry S. Singeltary Sr., P.O. BOX 42, Bacliff, Texas 77518 USA
http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
Professor Michael Farthing wrote:
Louise Send this to Bramble (author) for a comment before we post. Michael
=======================================================
Subj: Reprocessing of Flexible Endoscopes and Endoscopic Accessories - an International Comparison of Guidelines Date: 9/17/02 3:28:43 AM Eastern Daylight Time
From: flounder@WT.NET (Terry S. Singeltary Sr.)
Sender: BSE-L@UNI-KARLSRUHE.DE (Bovine Spongiform Encephalopathy)
Reply-to: BSE-L@UNI-KARLSRUHE.DE (Bovine Spongiform Encephalopathy)
To: BSE-L@UNI-KARLSRUHE.DE
Bovine Spongiform Encephalopathy
Reprocessing of Flexible Endoscopes and Endoscopic Accessories - an International Comparison of Guidelines
Zeitschrift für Gastroenterologie
© Georg Thieme Verlag Stuttgart New York More about this journal
Endoscopic examinations and procedures are essential for diagnosis and treatment of gastrointestinal diseases. As a result of poor reprocessing practice microorganisms can be transmitted via endoscope. The majority of infection transmissions is due to insufficient performance of cleaning and disinfection disregarding guidelines of societies of gastrointestinal endoscopy.
A review of the literature and a comparison of European and American guidelines for reprocessing flexible endoscopes are given. Differences in the classification of endoscopic devices, on the possibility of prion transmission, recommendations on staff training and protection, quality assurance of reprocessing and evidence-based graduation of guidelines are stressed and discussed. With respect to the procedure of endoscope reprocessing, differences concerning the cleaning solution to choose, necessity of thoroughly manual cleaning and brushing of the accessible endoscope channels (even in the case of subsequent automatic reprocessing endoscopes in washers-disinfectors), disinfection solution, microbiological quality of water for final rinsing and rationale for alcohol flush of endoscope channels for better drying are mentioned.
The need for experimental investigations of the cleaning and disinfection process is stressed. In contrast to recent guidelines of European and American societies of gastrointestinal endoscopy, the now updated recommendations of the Robert Koch-Institute for reprocessing flexible endoscopes and endoscopic accessories are evidence-based and graduated.
Original Article Z Gastroenterol 2002; 40: 531-542 DOI: 10.1055/s-2002-32807 Table of Contents Leitlinien zur Aufbereitung flexibler Endoskope und endoskopischen Zusatzinstrumentariums im internationalen Vergleich Reprocessing of Flexible Endoscopes and Endoscopic Accessories - an International Comparison of Guidelines O. Leiß1, U. Beilenhoff2, L. Bader3, M. Jung2, M. Exner4 1Fachbereich Gastroenterologie, Deutsche Klinik für Diagnostik, Wiesbaden 2St. Hildegardis-Krankenhaus, Mainz 3Max von Pettenkofer-Institut der LMU München, München 4Hygiene-Institut der Universität Bonn, Bonn
Zusammenfassung
Endoskopische Untersuchungen und Eingriffe sind für Diagnostik und Therapie gastrointestinaler Erkrankungen unverzichtbar. Durch mangelhaft aufbereitete Endoskope können Mikroorganismen übertragen werden. Die Mehrzahl der Infektionsübertragungen bei Endoskopie ist auf unzureichende Reinigungs- und Desinfektionsmaßnahmen unter Missachtung aktueller Aufbereitungsrichtlinien der Fachgesellschaften zurückzuführen.
In einer Literaturübersicht werden die Leitlinien europäischer und amerikanischer Fachgesellschaften zur Aufbereitung flexibler Endoskope verglichen. Es werden Unterschiede in der Klassifikation des endoskopischen Instrumentariums, in der Bewertung der Prionenproblematik, in den Anforderungen an Personalschulung und Personalschutz, in der Betonung qualitätssichernder Maßnahmen und in der wissenschaftlichen Untermauerung und Graduierung der ausgesprochenen Empfehlungen dargestellt und diskutiert. Zu Einzelschritten der Aufbereitung werden Unterschiede hinsichtlich der einzusetzenden Reinigungslösung, der Notwendigkeit einer manuellen Bürstenreinigung der Endoskopkanäle (auch bei nachfolgender maschineller Aufbereitung), der Wahl des Desinfektionsmittels, der mikrobiologischen Qualität des zur Schlussspülung verwendeten Wassers und der Empfehlung einer Spülung der Endoskopkanäle mit Alkohol für eine verbesserte Trocknung herausgestellt und kritisch bewertet.
Es wird offensichtlich, dass experimentelle Untersuchungen zu Einzelaspekten der Endoskop-Aufbereitung weitgehend fehlen bzw. erst in jüngster Zeit bearbeitet wurden. Im Gegensatz zu bisherigen Leitlinien europäischer und amerikanischer Fachgesellschaften zur Endoskop-Aufbereitung sind die aktualisierten Empfehlungen des Robert Koch-Instituts zur Aufbereitung flexibler Endoskope und endoskopischen Zusatzinstrumentariums mit der verfügbaren Evidenz verknüpft und graduiert. Schlüsselwörter
Flexible Endoskope - Aufbereitung - Reinigung - Desinfektion - Personalschulung - Qualitätssicherung - Mikrobiologische Prüfungen - Hygiene Abstract
http://www.thieme-connect.com/DOI/DOI?10.1055/s-2002-32807
TSS
http://mailhost.rz.uni-karlsruhe.de/warc/bse-l.html
2009
Pre-surgical risk assessment for variant Creutzfeldt-Jakob disease (vCJD) risk in neurosurgery and eye surgery unitsHospitals should already be using a questionnaire in Annex J of the ACDP TSE Working Group Infection Control guidance to find out whether any patients who are about to undergo any surgery or endoscopy may be at increased risk of being infected with CJD. If a patient is found to have an increased risk of CJD prior to their surgery or endoscopy then special infection control precautions may need to be taken. The Department of Health has carried out an analysis [1] which explores the effect of receiving a large number of blood transfusions on a patient's risk of vCJD infection. The CJD Incidents Panel reviewed this analysis and advises that patients who have received blood components from 80 or more donors may have an increased risk of variant CJD (vCJD).
Annex J of the TSE Infection Control guidance has recently been revised, and now advises that patients who are due to have high risk surgery [2 ] or neuro-endoscopy should be asked an additional question: whether they have received transfusions of blood or blood components from 80 or more donors since 1980.
On 16 July 2009 the HPA wrote to the chief executives of NHS trusts asking them to ensure that the guidance is implemented. Detailed information and tools for implementing the guidance can be downloaded from the links below.
If you have any queries about the implementation of the guidance, please contact the HPA Centre for Infections CJD Section at cjd@hpa.org.uk or on 020 8327 6074/6411.
Background information on this new pre-surgical assessment is contained in this Letter to chief executives - July 2009 (PDF, 73 KB) written to all hospitals in England.
The new version of Annex J of the TSE Infection Control Guidance contains a new question for patients undergoing high risk surgery and neuro-endoscopy. The questionnaire in Annex J should be used to assess patients' CJD risk factors.
Clinicians carrying out the new pre-surgical assessment should read Information for healthcare staff - November 2009 (PDF, 164 KB) This vCJD Algorithm for per-surgical roles - July 2009 (PDF, 28 KB) shows suggested roles and responsibilities for infection control teams, surgical teams and blood transfusion specialists.
Information on patients' transfusion histories should be collected using the Highly transfused vCJD risk assessment form - July 2009 (Word Document, 328 KB) This form is also available as a Highly transfused vCJD risk assessment form and tool - July 2009 (Excel Spreadsheet, 2.7 MB). This may help calculate the number of blood donors to a patient. The form may be posted or emailed to the HPA Centre for Infections CJD Section cjd@hpa.org.uk.
Blood transfusion laboratories may wish to use this draft Letter to other blood laboratories - July 2009 (Word Document, 31 KB) when collecting transfusion information from other hospitals.
Pre-surgical assessment teams and patients may wish to read vCJD Information for presurgical patients - July 2009 (PDF, 29 KB) about this new pre-surgical assessment.
[1] The risk of secondary vCJD infection of patients receiving a high number of blood transfusions. Department of Health, July 2009.
[2] High risk surgery is defined as surgery involving any of the following organs or tissues (high risk tissues): brain, spinal cord, cranial nerves (specifically the entire optic nerve and only the intercranial components of the other cranial nerves), cranial nerve ganglia, posterior eye (specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid, optic nerve) and pituitary gland.
•Letter to chief executives - July 2009 (PDF, 73 KB) Added/updated: 16 July 2009
•Pre-surgical assessment Information for healthcare staff - November 2009 (Word Document, 252 KB) Added/updated: 27 November 2009
•vCJD Algorithm for per-surgical roles - July 2009 (PDF, 28 KB) Added/updated: 16 July 2009
•Highly transfused vCJD risk assessment form - July 2009 (Word Document, 328 KB) Added/updated: 16 July 2009
•Letter to other blood laboratories - July 2009 (Word Document, 31 KB) Added/updated: 16 July 2009
•vCJD Information for presurgical patients - July 2009 (PDF, 29 KB) Added/updated: 16 July 2009
•Highly transfused vCJD risk assessment form and tool - July 2009 (Excel Spreadsheet, 2.7 MB) Added/updated: 8 December 2009
http://www.hpa.org.uk/webw/HPAweb&Page&HPAwebAutoListName/Page/1247469069188
Pre-surgical assessment for vCJD risk in neurosurgery and eye surgery units. Information for clinicians
1. Introduction. 2
Box 1 High risk tissues for patients with vCJD and at risk of vCJD. 2
2. Preparation and planning. 2
Box 2. Who may be involved in assessing patients’ vCJD risks 2
3. Proposed roles and responsibilities. 3
a. Pre-surgical assessment staff 3
Box 3: Questions for patients undergoing procedures involving high risk tissues 3
b. Lead consultant for blood transfusion/consultant for the hospital blood bank. 4
c. Infection Control Doctor 5
Box 4 Infection control actions for patients with an increased risk of vCJD. 6
d. Lead consultant for vCJD risk assessment 6
e. The CJD Section at the HPA Centre for Infections 7
f. General Practitioners (or other clinician) - Informing patients 8
Box 5 Public health advice for patients 9
g. General Practitioners – action. 9
h. Health Protection Units 10
Box 6 Tissue infectivity levels for patients with, or at increased risk of, vCJD. 10
4. Evaluation. 10
5. Frequently Asked Questions. 11
a.. Which patients should be asked for their transfusion history to assess their vCJD risk? 11
b. Why not assess all patients attending for any operation? 11
c.. Should patients attending for anterior eye surgery be asked for their blood transfusion history to assess their vCJD risk? 11
d.. What should happen if a patient who has, or might have, received blood from 80 or more donors needs surgery on medium risk tissues (e.g. cataract procedure or tonsillectomy)? 11
e. What happens if a patient identified as highly transfused when assessed prior to surgery on high risk tissues or neuro-endoscopy, has an operation involving medium risk tissues at a later date? 12
f.. Should doctors try to identify prospectively all their patients who may have received blood from 80 or more donors? 12
g.. What if patients ask their doctors whether they are at increased risk because of their blood transfusion history? 12
h. How should patients who have received large numbers of transfusions, but fewer than 80, be managed? 12
i... What information should be given to patients who may receive 80 or more transfusions as part of their treatment? 13
j. Might the 80 donor exposure cut off level for highly transfused patients change? 13
k. What is the CJD Incidents Panel? 13
l... What does the CJD Incidents Panel recommend? 13
1. Introduction
This information leaflet accompanies Annex J of the ACDP TSE Working Group infection control guidance[1]. This leaflet,and related documents are available on the HPA website[2].
Annex J describes how to assess pre-surgical patients for their risk of variant Creutzfeldt-Jakob disease (vCJD) and other transmissible spongiform encephalopathies (TSE). It includes new guidance on assessing patients undergoing surgery or neuro-endoscopy on high risk tissues. This includes identifying patients who have received blood from 80 or more donors, and may have an increased risk of vCJD. This leaflet aims to give practical information on how to assess these patients, and how to manage those who have an increased vCJD risk because of their transfusion history, or have an incomplete or uncertain transfusion history.
Box 1 High risk tissues for patients with vCJD and at risk of vCJD
brain
spinal cord
dura mater
cranial nerve ganglia
cranial nerves,
specifically: the entire optic nerve
only the intracranial components of other cranial nerves
posterior eye,
specifically:
posterior hyaloid face
retina
retinal pigment epithelium
choroid
subretinal fluid
optic nerve
pituitary gland
2. Preparation and planning
--------------------------------------------------------------------------------
[1] http://www.dh.gov.uk/ab/ACDP/TSEguidance/index.htm
[2] http://www.hpa.org.uk/vCJDpresurgicalassessment
SEE FULL TEXT ;
http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469061870
Latest news 16 November 2009: Annex C: General principles of decontamination and waste disposal
Download
General principles of decontamination and waste disposal: ACDP TSE Working Group Annex C (PDF, 201K)
http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@ab/documents/digitalasset/dh_108602.pdf
12 October 2009:
Alert to urological surgeons – transrectal prostatic biopsy in men at risk of variant CJD Download alert to urological surgeons regarding the equipment used for patients at risk of vCJD requiring transrectal prostatic biopsy (PDF, 28K)
http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@ab/documents/digitalasset/dh_106909.pdf
Annex D - Transport of TSE-infected material Published: December 2003, updated: 23 January 2009
http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@ab/documents/digitalasset/dh_087484.pdf
Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J
ANNEX J
Assessment to be carried out before surgery and/or endoscopy to identify patients with, or at increased risk of, CJD or vCJD
Summary of advice (revised July 2009)
Annex J provides a clear and pragmatic way of assessing CJD and vCJD risk prior to surgery or endoscopy. Growing numbers of patients have been informed that they are at increased risk of CJD or vCJD. Therefore it is recommended that all patients about to undergo any surgery or endoscopy should be asked if they have ever been notified as at increased risk of CJD or vCJD. This recommendation is outlined in paragraphs J1 and J2. In addition, patients undergoing surgery or neuro-endoscopy which may involve contact with tissues of potentially high level TSE infectivity (“high risk tissues”) should, through a set of detailed questions, be assessed for their possible CJD/vCJD risk exposure. These questions are outlined in Table J1 and paragraphs J3 to J6.
Annex J has been revised (July 2009) to include a new question to be asked of all patients about to undergo surgery or neuro-endoscopy on high risk tissues. This new question (question 4 in Table J1) has been added to identify those patients who have received blood or blood components from 80 or more blood donors. These patients are now designated as at increased risk of vCJD by the CJD Incidents Panel. Table J1 and paragraph J6 provide guidance on how to identify those patients who have received multiple blood transfusions. An algorithm, included as Appendix A, guides health professionals through the process.
Recommendation for all surgical and endoscopy patients
J1. The CJD Incidents Panel has identified a number of individuals or groups who are at increased risk of CJD or vCJD (see paragraphs J14 – J18). At a local level arrangements should be put in place to ensure that patients who have been notified they are at increased risk of CJD/vCJD are identified before surgery or endoscopy, to allow appropriate infection control procedures to be followed.
All patients about to undergo any elective or emergency surgical or endoscopic procedure should be asked the question: “Have you ever been notified that you are at increased risk of CJD or vCJD for public health purposes?”
J2. The actions to take following the patient’s response to the above question are:
SEE FULL TEXT ;
http://www.dh.gov.uk/prod_consum_dh/groups/dh_digitalassets/@dh/@ab/documents/digitalasset/dh_102856.pdf
Update: Creutzfeldt-Jakob Disease Associated with Cadaveric Dura Mater Grafts --- Japan, 1979--2003 MMWR Weekly December 5, 2003 / 52(48);1179-1181
http://www2.niddk.nih.gov/AboutNIDDK/CommitteesAndWorkingGroups/HGH_CJD_Dec_11_2003_TAB_A.htm
Thursday, October 23, 2008
Creutzfeldt-Jakob Disease Associated with Cadaveric Dura Mater Grafts - Japan, 1979-2008 : UPDATE
http://creutzfeldt-jakob-disease.blogspot.com/2008/10/creutzfeldt-jakob-disease-associated.html
http://www.wellsphere.com/cjd-article/medical-procedures-and-risk-for-sporadic-creutzfeldt-jakob-disease-japan-1999-2008-warning-to-neurosurgeons-and-ophthalmologi/641229
Thursday, January 29, 2009
Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research
http://creutzfeldt-jakob-disease.blogspot.com/2009/01/medical-procedures-and-risk-for.html
Sunday, May 10, 2009
Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)
http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html
Monday, August 17, 2009
Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/transmissible-spongiform-encephalopathy.html
Friday, July 17, 2009
Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/07/revision-to-pre-surgical-assessment-of.html
Tuesday, August 12, 2008
Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)
http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html
Thursday, July 23, 2009
UW Hospital warning 53 patients about possible exposure to rare brain disease
http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-warning-53-patients-about.html
Tuesday, April 21, 2009
Doctor Antonio Ruiz Villaespesa, pathologist and CJD researcher deceased because of Creutzfeldt-Jakob Disease SPAIN
http://cjdusa.blogspot.com/2009/04/doctor-antonio-ruiz-villaespesa.html
Br J Ophthalmol 2005;89:1131-1138 doi:10.1136/bjo.2004.063495 Clinical science Scientific reports Abnormal prion protein in the retina of the most commonly occurring subtype of sporadic Creutzfeldt-Jakob disease M W Head1, A H Peden1, H M Yull1, D L Ritchie1, R E Bonshek2, A B Tullo2 and J W Ironside1 + Author Affiliations
1National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, Edinburgh EH4 2XU, UK 2Academic Department of Ophthalmology, Manchester Royal Eye Hospital, Manchester M13 9WH, UK Correspondence to: Dr M W Head National CJD Surveillance Unit, Bryan Matthews Building, Western General Hospital, Edinburgh EH4 2XU, UK; m.w.head@ed.ac.uk Accepted 7 March 2005 Abstract Background: Involvement of the eye has been reported in patients with variant Creutzfeldt-Jakob disease (vCJD), but there is disagreement on whether retinal involvement occurs in sporadic Creutzfeldt-Jakob disease (sCJD).
Methods: Western blotting, paraffin embedded tissue blotting, and immunohistochemistry were used to test whether the abnormal form of the prion protein (PrPSc) accumulates to detectable levels in the eye in a case of the most common subtype of sCJD (MM1).
Results: Low levels of PrPSc were detectable in the retina, localised to the plexiform layers of the central retina. PrPSc was not detectable in other ocular tissues.
Conclusions: The abnormal form of the prion protein is present in the retina in the most common sCJD subtype (MM1), albeit at levels lower than those found previously in vCJD and in sCJD of the VV2 subtype.
http://bjo.bmj.com/content/89/9/1131.abstract
Friday, August 07, 2009
CJD Human Cornea Tissue, Recall END OF ENFORCEMENT REPORT FOR AUGUST 5, 2009
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/cjd-human-cornea-tissue-recall-end-of.html
Wednesday, August 12, 2009
Unique clinicopathological features and PrP profiles in the first autopsied case of dura mater graft-associated Creutzfeldt-Jakob disease
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/unique-clinicopathological-features-and.html
Tuesday, August 11, 2009
Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants
http://creutzfeldt-jakob-disease.blogspot.com/2009/08/characteristics-of-established-and.html
Monday, July 27, 2009
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?
WHY DID THIS VIDEO NOT SHOW ON EVERY NEWS CHANNEL IN THE U.S.A. $$$
IT IS A DAMNING VIDEO !!!
I WATCHED THIS RECENTLY, and had never seen it. i was so mad, i was spitting nails out faster than a framing gun.
WHY DID THE CANADIAN MEDIA ONLY PRESENT THIS TO THE U.S.A. PUBLIC (thank you very much though), and why has the U.S.A. MEDIA FAILED US ???
SNIP...
SEE DAMNING VIDEO AT BOTTOM OF BLOG HERE ;
Monday, July 27, 2009
U.S.A. HIDING MAD COW DISEASE VICTIMS AS SPORADIC CJD ?
http://creutzfeldt-jakob-disease.blogspot.com/2009/07/usa-hiding-mad-cow-disease-victims-as.html
NOW, AFTER SEEING THAT VIDEO, (first watch the video) lets go back in mad cow time here in the USA, shall we.
THERE must be an independent review of this cover-up, and the infamous ENHANCED BSE SURVEILLANCE AND TESTING OF 2004, that was nothing but a cover-up, and blundered at that, and plus a REDO of the testing of no less than 1 million head of cattle tested each year, for five years, with scientist from the EU overseeing the testing protocols, surveillance, and confirmation of all cases.
this video states the 'USA had to _admit_ a home grown case of mad cow in TEXAS." fact was, they did not finally admit anything, IT TOOK AN ACT OF CONGRESS, THE HONORABLE PHYLISS FONG AND THE O.I.G., TO MAKE THEM RETEST AND CONFIRM! that my friend is fact. and only after Prof. Aguzzi, Dr. Jean-Philippe Deslys, Dr. Collinge et al slammed them over the testing of that animal. and then we had the mad cow in Texas that they just refused to test, and was sent to a pet food rendering plant. NO TEST AT ALL. you must realize, when other officials, doctors, and such from other country's confront issues about things in the USA, they must walk on ice when doing so. read inbetween the lines here ;
i wrote all these scientist and doctors and the OIG about that damn texas cow, gave them the evidence i had from TAHC. so did others, but finally fong et al did something.
a bit of history for you mel. file this away. ...
Tuesday, July 28, 2009
MAD COW COVER-UP USA MASKED AS SPORADIC CJD
http://madcowtesting.blogspot.com/2009/07/mad-cow-cover-up-usa-masked-as-sporadic.html
The most recent assessments (and reassessments) were published in June 2005 (Table I; 18), and included the categorisation of Canada, the USA, and Mexico as GBR III. Although only Canada and the USA have reported cases, the historically open system of trade in North America suggests that it is likely that BSE is present also in Mexico.
http://www.oie.int/boutique/extrait/06heim937950.pdf
Tuesday, July 14, 2009
U.S. Emergency Bovine Spongiform Encephalopathy Response Plan Summary and BSE Red Book Date: February 14, 2000 at 8:56 am PST
WHERE did we go wrong $$$
http://madcowtesting.blogspot.com/2009/07/us-emergency-bovine-spongiform.html
Saturday, January 2, 2010
Human Prion Diseases in the United States January 1, 2010 ***FINAL***
http://prionunitusaupdate2008.blogspot.com/2010/01/human-prion-diseases-in-united-states.html
my comments to PLosone here ;
http://www.plosone.org/annotation/listThread.action?inReplyTo=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd&root=info%3Adoi%2F10.1371%2Fannotation%2F04ce2b24-613d-46e6-9802-4131e2bfa6fd
TSS
Labels: atypical CJD, BLOOD, CJD, ENDOSCOPY, iCJD, PRION, tissue
