Friday, December 23, 2011

Detection of PrPres in Genetically Susceptible Fetuses from Sheep with Natural Scrapie

Detection of PrPres in Genetically Susceptible Fetuses from Sheep with Natural Scrapie



María Carmen Garza1, Natalia Fernández-Borges2, Rosa Bolea1, Juan José Badiola1, Joaquín Castilla2,3, Eva Monleón1,4*


1 Centro de Investigación en Encefalopatías Espongiformes Transmisibles y Enfermedades Emergentes, Universidad de Zaragoza, Zaragoza, Spain, 2 CIC bioGUNE, Parque Tecnológico de Bizkaia, Derio, Spain, 3 IKERBASQUE, Basque Foundation for Science, Bilbao, Spain, 4 Producció Animal, Universitat de Lleida, LLeida, Spain




Scrapie is a transmissible spongiform encephalopathy with a wide PrPres dissemination in many non-neural tissues and with high levels of transmissibility within susceptible populations. Mechanisms of transmission are incompletely understood. It is generally assumed that it is horizontally transmitted by direct contact between animals or indirectly through the environment, where scrapie can remain infectious for years. In contrast, in utero vertical transmission has never been demonstrated and has rarely been studied. Recently, the use of the protein misfolding cyclic amplification technique (PMCA) has allowed prion detection in various tissues and excretions in which PrPres levels have been undetectable by traditional assays. The main goal of this study was to detect PrPres in fetal tissues and the amniotic fluid from natural scrapie infected ewes using the PMCA technique. Six fetuses from three infected pregnant ewes in an advanced clinical stage of the disease were included in the study. From each fetus, amniotic fluid, brain, spleen, ileo-cecal valve and retropharyngeal lymph node samples were collected and analyzed using Western blotting and PMCA. Although all samples were negative using Western blotting, PrPres was detected after in vitro amplification. Our results represent the first time the biochemical detection of prions in fetal tissues, suggesting that the in utero transmission of scrapie in natural infected sheep might be possible.


Citation: Garza MC, Fernández-Borges N, Bolea R, Badiola JJ, Castilla J, et al. (2011) Detection of PrPres in Genetically Susceptible Fetuses from Sheep with Natural Scrapie. PLoS ONE 6(12): e27525. doi:10.1371/journal.pone.0027525


Editor: Jason Bartz, Creighton University, United States of America


Received: July 20, 2011; Accepted: October 18, 2011; Published: December 14, 2011


Copyright: © 2011 Garza et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


Funding: This study was supported by a national grant from Spain (AGL2009-11553-C02-01), a Basque government grant (PI2010-18) and the Department of Industry, Tourism and Trade of the Government of the Autonomous Community of the Basque Country (Etortek Research Programs 2011/2013), from the Innovation Technology Department of the Bizkaia County. Dr. Garza was supported by a FPU doctoral grant from the Spanish Ministry of Education (AP2007-03842). The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.


Competing interests: The authors have declared that no competing interests exist.










Envt.18: Mother to Offspring Transmission of Chronic Wasting Disease


Candace K. Mathiason,† Amy Nalls, Kelly Anderson, Jeanette Hayes-Klug, Jenny G. Powers, Nicholas J. Haley and Edward A. Hoover


Colorado State University; Fort Collins, CO USA†Presenting author; Email: ckm@lamar.colostate.edu


We have developed a new cervid model in small Asian muntjac deer (Muntiacus reevesi) to study potential modes of vertical transmission of chronic wasting disease (CWD) from mother to offspring. Eight of eight (8/8) muntjac doe orally infected with CWD tested PrPCWD lymphoid positive by four months post infection. Ten fawns were born to these CWD-infected doe— four of the fawns were viable, five were non-viable and one was a first trimester fetus harvested from a CWD-infected doe euthanized at end-stage disease. The viable fawns have been monitored for CWD infection by immunohistochemistry and sPMCA performed on serial tonsil and rectal lymphoid tissue biopsies. PrPCWD has been detected in one fawn by IHC as early as 40 days of age. Moreover, sPMCA performed on rectal lymphoid tissue has yielded positive results on another fawn at ten days of age. In addition, sPMCA assays have demonstrated amplifiable prions in fetal placental or spleen tissue of three non-viable fawns and mammary tissue of the dams.


Additional pregnancy related fluids and tissues from the doe as well as tissue from the nonviable fawns are currently being probed for the presence of CWD. In summary, we have employed the muntjac deer model, to demonstrate for the first time the transmission of CWD from mother to offspring. These studies provide the foundation to investigate the mechanisms and pathways of maternal prion transfer.




===========================




PPo3-18: A Possible Case of Maternal Transmission of the BSE Agent within Captive Cheetah Affected with Feline Spongiform Encephalopathy



Anna Bencsik, Sabine Debeer, Thierry Petit and Thierry Baron


Afssa; Unité ATNC; Lyon, France; Zoo de la Palmyre; Les Mathes, France


Key words: BSE, FSE, vertical transmission


Introduction. Feline spongiform encephalopathy (FSE) is considered to be related to bovine spongiform encephalopathy (BSE). It has been reported in domestic cats as well as in captive wild cats including cheetahs, first in the United Kingdom (UK) and then in other European countries. In France, several cases were described in cheetahs either imported from UK or born in France. Here we report details of two other FSE cases in captive cheetah. These cases are of particular interest since the 2nd case of FSE in a cheetah born in France, appears most likely due to maternal transmission.1


Results. Complete PrPd study showed the close likeness between the two cheetah cases. The TgOvPrP4 mouse brains infected with cattle BSE and cheetah FSE revealed similar vacuolar lesion profiles, PrPd brain mapping with occurrence of typical florid plaques.


Materials and Methods. Using immunohistochemistry (IHC), pathological form of PrP(PrPd) was analyzed in the brains and peripheral organs of these two cheetahs. Transmission studies to the TgOvPrP4 mouse line were also performed, for comparison with the transmission of cattle BSE. Lesion profiles of the infected transgenic mice were analyzed as well as type and brain distribution of PrPd.


Conclusion. Collectively, these data indicate that both FSE cases harbor the same strain of agent as the cattle BSE agent. Because this is most probably a case of maternal transmission of the disease, this new observation may have some impact on our knowledge of vertical transmission of BSE agent-linked TSEs such as in human variant Creutzfeldt Jakob disease.


References


1. Bencsik et al. PLoS One 2009; 4:6929.



=========================




PPo3-40: Mother to Offspring Transmission of Chronic Wasting Disease


Candace K. Mathiason, Amy V. Nalls, Kelly Anderson, Jeanette Hayes-Klug, Nicholas Haley and Edward A. Hoover


Colorado State University, Department of Microbiology, Immunology and Pathology, Fort Collins, CO USA


Key words: Chronic wasting disease, vertical transmission, muntjac deer


We have developed a new cervid model in small Asian muntjac deer (Muntiacus reevesi) to study potential modes of vertical transmission of chronic wasting disease (CWD) from mother to offspring. Eight of eight (8/8) muntjac doe orally infected with CWD tested PrPCWD lymphoid positive by 4 months post infection. Six fawns were born to these CWD-infected doe. Six fawns were born to 6 CWD-infected doe; 4 of the fawns were non-viable. The viable fawns have been monitored for CWD infection by immunohistochemistry and sPMCA performed on serial tonsil and rectal lymphoid tissue biopsies. PrPCWD has been detected in one fawn as early as 40 days of age. Moreover, sPMCA performed on rectal lymphoid tissue has yield positive results on another fawn at 10 days of age. In addition, sPMCA assays have also demonstrated amplifiable prions in maternal placental (caruncule) and mammary tissue of the dam.


Additional pregnancy related fluids and tissues from the doe as well as tissue from the nonviable fawns are currently being probed for the presence of CWD. In summary, we have employed the muntjac deer model, to demonstrate for the first time the transmission of CWD from mother to offspring. These studies provide the foundation to investigate the mechanisms and pathways of maternal prion transfer.


PRION 2011


landesbioscience.com


International Prion Congress: From agent to diseaseSeptember 8–11, 2010Salzburg, Austria








PRION 2010













Wednesday, December 30, 2009


Is there evidence of vertical transmission of variant CJD ?


J Neurol Neurosurg Psychiatry doi:10.1136/jnnp.2009.172148







Monday, November 22, 2010


SHEEP WITH MASTITIS TRANSMIT INFECTIOUS PRIONS THROUGH THE MILK







Monday, November 22, 2010


SHEEP WITH MASTITIS TRANSMIT INFECTIOUS PRIONS THROUGH THE MILK


J. Virol. doi:10.1128/JVI.02022-10 Copyright (c) 2010, American Society for Microbiology and/or the Listed Authors/Institutions. All Rights Reserved.







Saturday, December 3, 2011


Isolation of Prion with BSE Properties from Farmed Goat


Volume 17, Number 12—December 2011







Prion disease spreads in sheep via mother's milk


communicated by: Terry S Singeltary Sr [ These research confirms experimentally previous observations by others (such as, Lacroux C et al: Prions in Milk from Ewes Incubating Natural Scrapie. PLoS Pathog 4(12): e1000238.doi:10.1371/journal.ppat.1000238;










SNIP...










-------- Original Message --------


Subject: POSITION STATEMENT MATERNAL TRANSMISSION OF vCJD SEAC


Date: Fri, 04 Feb 2005 09:54:58 –0600


From: "Terry S. Singeltary Sr."


To: Bovine Spongiform Encephalopathy


CC: cjdvoice@yahoogroups.com, Agriculture@mail.house.gov


1


POSITION STATEMENT MATERNAL TRANSMISSION OF vCJD




Issue




1. The Chief Medical Officer for England asked SEAC to consider current evidence and comment on the potential transmission of vCJD from mother to child via human breast milk. In utero transmission was also considered. The committee also commented on the scientific basis of a risk reduction measure for possible transmission of vCJD via banked breast milk.




Background




2. No diagnostic test is currently available for the detection of abnormal PrP in milk. Research is under way to develop tests to screen for the possible presence of abnormal prion protein (PrP) in milk samples from cattle experimentally infected with BSE1. These modified tests may also be applicable to human milk. However, it is not yet clear when/if a reliable test will be available.




3. A small number of breast milk banks in the UK supply highly vulnerable premature babies for whom no milk may be available from the mother. A model developed by the Department of Health to assess the effect of pooling breast milk from multiple donors on the possible risks of transmission of vCJD via breast milk banks was considered.




4. There is some, albeit limited, published epidemiological and experimental research on maternal transmission of prion diseases. There are also unpublished surveillance data of children born to vCJD cases from the National CJD Surveillance Unit and UK surveillance of neurological illness in children which might inform on potential risks of maternal transmission.




1 A joint FSA/SEAC milk working group is monitoring and providing advice on this research carried out at the Veterinary Laboratories Agency.




2 Breast milk banks




5. There is no evidence that vCJD infectivity has ever been transmitted through breast milk. However, a theoretical risk exists. Modelling studies clearly show that the practice of pooling breast milk increases the number of donors to which a recipient is exposed and thereby increases the potential risk of an infant receiving milk contaminated with vCJD infectivity. The theoretical risk of infection can be minimised by not pooling the milk, by the use of individual hand operated breast milk pumps for single donors, and by the use of single-use sterilised bottles for collection. In addition, available evidence suggests that infection/inflammation of the breast results in increased lymphocytes in milk and therefore increased risk of infectivity. This risk would be minimised if milk from donors showing signs of infection is not used.




6. The committee suggested that, if practicable, milk could be stored for an appropriate period of time to allow the health status of donors to be monitored, before it is released. However, information was not available to the committee on whether long-term storage of human milk is detrimental to its nutritional quality.




Maternal transmission




7. There is evidence from animal studies for low level maternal transmission of prions in cattle and sheep. This transmission may occur in utero, via milk and/or perinatally. However, the possibility that this putative maternal transmission might have been due to another mode of transmission, for example through a contaminated environment or feed, cannot be ruled out.




8. In contrast, in humans there is no evidence for maternal transmission in cases of familial prion disease, other than the transfer of a mutant form of the PrP gene, and there is no evidence of maternal transmission of Kuru. However, compared with other human prion diseases vCJD may pose a greater risk because of the greater involvement of the lymphoreticular system in vCJD pathogenesis. Although, breast tissue (and placenta) from a single vCJD case tested negative for PrPvCJD, transfer of infectivity to breast milk may depend on the physiological status of the mammary gland. Similar tests or infectivity bioassays have not been conducted on breast tissue from lactating patients with vCJD. 3




9. A published study suggesting transmission of sCJD in colostrum2 was considered unreliable because tissues not normally associated with high levels of infectivity (blood and placenta) showed equivalent infectivity to that of the brain in this study.




10. Analysis of prospective surveillance data of UK children born to mothers with, or that had subsequently developed clinical vCJD, provide no evidence for maternal transmission of vCJD. However, the number of cases is very small and the incubation period of vCJD, if transmitted from mother to child, is unknown and so the children may yet be too young to have developed symptoms.




11. The phenotype of BSE infection in humans expressing PrP genotypes other than M/M at codon 129 is not known. Given recently published studies in mice expressing the human PrP gene3, which suggest that the human PrP genotype may affect disease phenotype, the committee considered it very important that undiagnosed neurological diseases be carefully monitored. In this respect, amongst others, it is recommended that the careful monitoring of neurological illnesses through the PIND surveillance of children4 continue.




Conclusions




12. In summary, there is currently no epidemiological evidence for maternal transmission of vCJD, including transmission via breast milk. However, there is a hypothetical risk. Although available evidence is limited and mostly indirect rather than direct, this risk, if any, appears to be low. As a risk cannot be excluded, a watching brief should be maintained.




SEAC




January 2005




2 Tamai Y et al. Demonstration of the transmissible agent in tissue from a pregnant woman with CJD. New Eng J Med 1992 327, 649.




3 Wadsworth et al. Human prion protein with valine 129 prevents expression of variant CJD phenotype. Science. 2004 306, 1793-1796.




4 Devereux G et al. Variations in neurodegenerative disease across the UK: findings from the national study of Progressive Intellectual and Neurological Deterioration (PIND). Arch Dis Child. 2004 89, 8-12.








Date: Thu 20 Jan 2005 From: Terry S. Singeltary Sr.




Chronic Lymphocytic Inflammation Specifies the Organ Tropism of Prions




----------------------------------------------------------------------[




The following is the summary of a paper by Mathias Heikenwalder and 8 others, published in Science online, 10.1126/science.1106460, Thu 20 Jan 2005






This paper describes work that illustrates that chronic inflammatory conditions may affect and expand the natural and iatrogenic transmission of prions - Mod.CP]Prions typically accumulate in nervous and lymphoid tissues. Because proinflammatory cytokines and immune cells are required for lymphoid prion replication, we tested whether inflammatory conditions affect prion pathogenesis. We administered prions to mice with 5 inflammatory diseases of kidney, pancreas or liver. In all cases, chronic lymphocytic inflammation enabled prion accumulation in otherwise prion-free organs. Inflammatory foci consistently correlated with lymphotoxin upregulation and ectopic induction of PrPC-expressing FDC-M1+ cells, whereas inflamed organs of mice lacking lymphotoxin-alpha or its receptor accumulate neither PrPSc nor infectivity upon prion inoculation. By expanding the tissue distribution of prions, chronic inflammatory conditions may act as modifiers of natural and iatrogenic prion transmission.******[4]Date: Thu 20 Jan 2005


From: Terry S. Singeltary Sr.


Source: Reuters News Agency, Thu 20 Jan 2005 [edited]









Study Finds that Illness May Promote Spread of Mad Cow Prion


------------------------------------------------------------




The agent that transmits mad cow disease and related diseases may spread further in the body of an animal suffering from certain illnesses, scientists said on Thu 20 Jan 2005. Their finding raises the question of whether measures aimed at curbing the spread of mad cow disease, or bovine spongiform encephalopathy (BSE), are adequate, the researchers said.Tests on mice showed that prions, the protein-like fragments that transmit BSE and related diseases [e.g. variant Creutzfeldt-Jakob disease in humans], can show up in organs they are not supposed to if the mouse has an inflammatory condition. Scientists have believed that BSE-causing prions are limited to the brain, spleen, spinal cord and lymph tissue, although some tests have suggested blood and muscle tissue may also harbor the prions. The latest study, published in the journal Science, suggests prions may also sometimes be found in the kidney, pancreas and liver. "We administered prions to mice with 5 inflammatory diseases of kidney, pancreas or liver," wrote the researchers, led by top prion expert Dr. Adriano Aguzzi of the University Hospital of Zurich in Switzerland.Aguzzi and colleagues in Britain and the United States inoculated specially bred mice with prions and checked to see if the prions spread in their bodies when the mice had an inflammatory condition. This is because other studies had suggested that prions might be attracted to immune system inflammatory cells. "In all cases, chronic lymphocytic inflammation enabled prion accumulation in otherwise prion-free organs," the researchers wrote.BSE peaked in British cattle herds in the mid-1990s, and a few cases have been reported in other countries. Canada reported its 3rd case this month. People who eat BSE-infected beef products can develop a related human brain disease called variant Creutzfeldt-Jakob disease or vCJD. There is no treatment or cure. [As of 4 Feb 2005, so far in the UK for the year 2005 there have 8 referrals of suspected CJD; and there have been 8 deaths from sporadic CJC, one from GSS and none from familial, iatrogenic or variant CJD. - Mod.CP]. It has killed 148 Britons, and 5 [now 6] Britons are alive with the disease, according to the British Department of Health's monthly report on the disease. The World Health Organization says it has reports of 6 cases in France, one in Ireland, one in Italy, one in Canada and one in the United States [and one in Japan: see; ProMED-mail post "CJD (new var.) - Japan: death 20050204.0381" - Mod.CP] Experts believed BSE first appeared when cattle were fed improperly rendered remains of sheep infected with scrapie, a related disease. In 1997, the United States and Canada imposed animal feed bans, and have mandated the removal of materials believed to carry infectious prions. These include the skull, brain, nerves attached to the brain, eyes, tonsils, spinal cord and attached nerves, plus a portion of the small intestine. The study suggests that even symptom-free animals may also have prions in their liver, kidney, and pancreas.--



Terry S. Singeltary Sr.flounder@wt.net



****** [5]Date: Fri 21 Jan 2005From: ProMED-mail promed@promedmail.org



Souce: New York Times, Fri 21 Jan 2005 [edited]






Study Finds Broader Reach for Mad Cow Proteins



----------------------------------------------



Mad cow disease has long been thought to occur in just the brains and nervous systems of infected animals. But scientists are reporting today that the proteins thought to cause the disease can travel to other organs as well. The research is based on experiments with mice, but if it is borne out in other species, it may suggest that no part of an infected animal is safe to eat. The disease leads to a fatal brain disease in humans [variant Creutzfeldt-Jakob disease].In the mouse experiments, reported in the journal Science [see [3] above], researchers in Switzerland found that prions, proteins that are the infectious agent in mad cow disease, follow immune cells, called lymphocytes, in the body. When mice were given chronic infectious diseases of the liver, kidney and pancreas and then inoculated with prions, the prions made their way to the infected organs. Dr. Adriano Aguzzi, a neuropathologist at the University Hospital in Zurich, who led the experiments, said this meant that cows and sheep infected with prions could harbor the disease in any inflamed organ.But Dr. David R. Smith, a veterinarian at the University of Nebraska, said the research did not raise alarms about American beef. For one thing, he said, livestock with obvious signs of systemic infection, like a fever, are not allowed into the food supply. And most American cattle are slaughtered while they are young and at reduced risk of infection. Many countries, including the United States, require the removal of skulls, brains, eyes, spinal cords and other nervous tissues from slaughtered animals because prions are known to accumulate in those tissues. Even in countries with mad cow disease, mainly in Europe, meat is considered safe if those tissues are removed, Dr. Aguzzi said. But the disease could spread more readily if infections are not obvious or if inspections are sloppily done, he said.[



Byline: Sandra Blakeslee]--ProMED-mail


******[6]Date: Fri 4 Feb 2005


From: Terry S. Singeltary Sr.


Source: Spongiform Encephalopathy Advisory Committee (SEAC), Position Paper, January 2005 [edited]






SNIP...SEE FULL TEXT ;






-------- Original Message --------


Subject: re-Mother passes on CJD to unborn baby SUNDAY TELEGRAPH Sun, 17


Sep 2000 10:09:01 –0700


Date: Sun, 26 Dec 2004 11:32:26 –0600


From: "Terry S. Singeltary Sr."


Reply-To: BSE-L




Greetings list members,


> re-Mother passes on CJD to unborn baby SUNDAY TELEGRAPH Sun, 17 Sep


> 2000 10:09:01 –0700




WHY is it we have heard nothing else about this case?


WHAT is the latest about this child that was supposedly to have been infected with nvCJD via his mother from birth, after which the mother passed on with confirmed nvCJD ???


thank you, kind regards,


Terry




Date: Sun, 17 Sep 2000 10:09:01 –0700


Reply-To: BSE-L


Sender: BSE-L


From: "Terry S. Singeltary Sr."




Subject: Mother passes on CJD to unborn baby SUNDAY TELEGRAPH


Bovine Spongiform Encephalopathy


ISSUE 1941 Sunday 17 September 2000 Mother passes on CJD to unborn baby By Rajeev Syal, Jenny Booth and Chris Hastings Scientists shocked as disease reveals new deadly traits Tragic inheritance of baby 'born with CJD' BSE report DOCTORS believe that a baby girl has been born with new variant Creutzfeldt-Jakob disease, the human form of mad cow disease. Her mother died of the illness earlier this year. Four specialists who have examined the 11-month-old girl believe that she is exhibiting the symptoms of vCJD and that she contracted the condition in the womb. The Telegraph knows the identity of the child, but cannot name her for legal reasons. The specialists have passed on their findings to the child's grandmother after tests failed to detect any other ailment in the girl. Only a post-mortem examination, however, can offer conclusive proof of vCJD. If confirmed, this would be the first known example of vCJD being transmitted from mother to child, and will heighten fears that the disease can be transmitted through blood. One leading microbiologist believes that some of the 67 people who have already died of vCJD may have inherited it from their mothers, rather than contracting it from eating infected meat. The baby's 50-year-old grandmother, who is now her legal guardian, said the doctors suspected that prions - the infectious agents believed to cause the disease - had been passed on to the baby in the womb and had given her brain damage. She said: "They don't know if it's gone into incubation. If so, it could be years before we can finally confirm the disease." The health of the child has been the subject of speculation since her mother died of vCJD in May, seven months after giving birth. The girl was found to have brain damage and has been suffering from fits and convulsions. Doctors have said that she is growing at half the normal rate for a child of her age and suffers from poor sight and abnormally stiff limbs. Her appendix has been examined by doctors looking for signs of vCJD, but the tests proved inconclusive. She will undergo further brain scans later this year. On Friday, The Lancet reported research by scientists at the Institute for Animal Health confirming for the first time that BSE can be transmitted in sheep by infected blood transfusions. The finding increases the likelihood that a vCJD-infected mother could pass on the disease to her baby. Richard Lacey, the emeritus professor of medical microbiology at Leeds University, said that it was "inevitable" that infected mothers would pass on vCJD through the placenta. He said: "The only thing that is uncertain is the scale on which it is happening." New variant CJD, a fatal disease of the brain and nervous system, is believed to have been transmitted to humans through eating beef infected with bovine spongiform encephalopathy. The Ministry of Agriculture admitted in 1996 that a pregnant cow could pass BSE to its unborn calf. Maternal transmission also occurs in sheep, rats and mice. Scientists have observed that offspring often develop the disease more virulently than the parent and after a much shorter incubation period. Until now, researchers have been baffled at the youth of the 67 people known to have died of vCJD. Their average age is 27. Most victims were aged 18 to 40. Dr Rob Will, of the National CJD Surveillance Unit in Edinburgh, has suggested that the victims caught the disease through eating cheap mechanically recovered meat used in school meals or even baby food. Dr Lacey however suspects that some may have contracted the illness from their mothers. The Telegraph has also learnt that the Department of Health is now considering the use of disposable surgical instruments throughout the NHS because of growing concern that blood and tissue from vCJD carriers could remain infectious even after sterilisation. As this newspaper revealed four years ago, tissue from such patients is capable of passing ordinary CJD to healthy patients, yet current standards for sterilising equipment are not adequate to destroy the prions.








SSUE 1941 Sunday 17 September 2000



Tragic inheritance of baby 'born with CJD' By Rajeev Syal, Chris Hastings and Jenny Booth



Mother passes on CJD to unborn baby THE dark-haired baby attracts admiring glances wherever she goes. She has her mother's striking blue eyes, says her adoring grandmother. Medical experts believe, however, that she is the first child to inherit the disease which killed her mother, variant Creutzfeldt-Jakob disease, while still in the womb. The child's 50-year-old grandmother, who has to feed the 11-month-old girl through a tube, said: "Every time I look at her, I see the agony that my daughter endured in her last days. Seeing my own child die in agony nearly killed me and now I am terrified that I will also see my grandchild die in the same way." For months she suspected that her granddaughter was suffering from the symptoms of vCJD. Over recent weeks, her worst fears have been confirmed by doctors from the leading London hospital that is treating her grandchild. The tragedy began in July 1998, when the woman's 22-year-old daughter - who ran her own catering business - became moody, tired and constantly tearful. Her daughter's frequent outbursts of temper were untypical; the two normally lived harmoniously together in a semi-detatched home in a Warwickshire village. By February last year, the young woman had become pregnant. She developed severe back trouble and, five months into her pregnancy, had to give up work. She could hardly move her arms and legs and had to be helped around the house. Other symptoms included pins and needles in her legs and swollen and sore lips. Doctors were mystified as to the cause of the illness. In October, to try to protect mother and child, the baby girl was delivered by Caesarean section weighing 6lb 4oz. Immediately, however, the doctors were aware that the little girl had difficulties swallowing and she was placed in a special baby care unit. The family was told by doctors two days after the birth that the baby probably had brain damage. The specialists decided to conduct a series of tests on the child. The family had begun to guess the truth. The dead woman's mother recalled: "I had spoken to someone who told me that their relative had died of CJD, and I had seen the news reports on television about cows. Then it dawned on me; my daughter's moods and the jerky movements she had begun to suffer were similar. It was an awful moment." By January, vCJD was confirmed in the mother by a biopsy on her tonsils - a procedure that is 98 per cent accurate.After this she was warned that her baby may also have contracted the disease.The young woman was virtually confined to a wheelchair and her memory was so bad that she sometimes failed to recognise her mother and her child. The baby's father had moved away from the area. Doctors tried to discover if her baby also had vCJD, but because the case was unique, and hampered by the poor health of the child, they were not sure how to reach a diagnosis. In May, the child's mother died after months of suffering. In the same month, doctors removed the little girl's appendix and took samples of her lymph tissue in the hope that an analysis would show whether she was carrying the prions - aberrant proteins - that caused the disease that killed her mother. According to the baby's grandmother, the tests carried out by the doctors were necessarily inconclusive because vCJD infection can only be finally confirmed after death through a post-mortem. They nevertheless believe that her granddaughter has been suffering from the effects of vCJD from the time of her conception. Caring for her dead daughter's child has now become the focus of the woman's life, and it is proving to be a daunting task. Her granddaughter's eyesight has been affected, and it is impossible to know how much she can see. Her limbs are stiff and she needs physiotherapy. She sleeps a lot of the time, as her mother did when she was ill. Last week she was suffering fits and convulsions, and doctors have said that she is growing at half the normal rate. She is to undergo further examinations by vCJD specialists later this year. Her grandmother said: "The appalling thing is that I am watching my granddaughter die while the Government fails to warn others that they may be passing the disease on. I want this baby's case highlighted because no other family should go through what we have been through,"








ISSUE 1941 Sunday 17 September 2000



Scientists shocked as disease reveals new deadly traits



By Robert Matthews



THE growing concern over the health of the baby born to a mother with variant CJD and the new evidence, announced last week, that the disease may have spread through blood transfusions, highlight the disturbing ability of vCJD to surprise the experts. A conclusive diagnosis of vCJD in the 11-month-old child would imply a far shorter incubation period than many scientists thought possible. It would also mean that the disease can be passed down the generations, not merely acquired through contact with infected tissue. Scientists have yet to pin down the likely size of the vCJD epidemic, with estimates for Britain ranging from about 100 cases to more than 100,000; so far there have been only 82 probable or confirmed cases. The case of the baby girl means that scientists are now admitting that many predictions may have to be rethought. According to Dr Neil Ferguson of the University of Oxford, a leading expert on the mathematics of epidemics, so-called vertical transmission down the generations has, at least until now, been thought to be relatively unimportant. Dr Ferguson said: "It very much depends on the probability of it taking place. If a woman has to be very sick and symptomatic in order to give it to her baby, then the number of cases it creates are going to be very small, because she is unlikely to get pregnant." The mother of the baby at the centre of the current concern is, however, understood to have been showing only minor symptoms of the disease at the time of conception; only later in her pregnancy did she develop the classic symptoms of vCJD from which she died in May this year. Richard Lacey, an emeritus professor of medical microbiology at Leeds University, said that the Telegraph story had wider implications than its obvious medical significance. Prof Lacey said: "The only thing that is certain is the scale on which it is happening. That will take decades to find out. I am aware of other cases where maternal transmission could be an issue; this isn't the only one. "This poses difficult ethical problems: to what extent should individuals be told of the risk? Should such a person be told not to give blood when he or she is old enough to do so? What effect will this knowledge have on that person's way of life, on their emotions? There needs to be some sort of discussion about how we cope with it, but at the moment there is nothing." Epidemiologists are puzzled by the fact that vCJD is predominantly affecting young people, while classic CJD is a disease of the old. Prof Lacey suggested that one possible explanation is that the teenagers and young people who have died from vCJD may have contracted the disease from their mothers while in the womb. He added that it is a recognised clinical syndrome that infectivity accumulates when it is passed on from one generation to the next. Dr Stephen Dealler, an expert on BSE and vCJD at Burnley Hospital, said that it had been believed that mother-to-offspring infection could take place in cows, but only with a considerable incubation period. "The thinking is that it may take place in cows, but the incubation period is still three to five years," he said. "This would lead us to expect an incubation period of around seven years in humans. If the baby does have vCJD, that's a very fast incubation period." Last week's revelation resulting from animal experiments that the disease may be transferred through blood transfusions from infected people with no symptoms was being played down by government scientists, who said that all British blood is screened to remove cells that may harbour vCJD. The Telegraph has learnt, however, that the Department of Health is seriously considering the use of disposable surgical instruments throughout the National Health Service. This follows growing concern that blood and tissue from asymptomatic carriers of the disease could remain infectious even after sterilisations.








TSS


Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518



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Subject: mother-to-child CJD (more downplaying or just hope?)


Date: Thu, 21 Sep 2000 20:56:41 –0700


From: "Terry S. Singeltary Sr."


Reply-To: Bovine Spongiform Encephalopathy


To: BSE-L@uni-karlsruhe.de


######### Bovine Spongiform Encephalopathy #########


The first case of mother-to-child transmission of vCJD?




Lancet 2000; 356: 1085 - 1092 Download PDF (109 Kb)




Last week UK newspapers reported that four UK doctors had revealed that they suspected an 11-month-old girl had variant Creutzfeldt-Jakob disease (vCJD), which she may have contracted from her mother, who died from the disease in May.




The child has brain damage, is unable to swallow, and has convulsions and stiff limbs. Doctors have not been able to give another explanation for her signs and tests for the abnormal prion protein in appendix and lymph tissue have been inconclusive. Proof of vCJD in adults requires examination of brain tissue at necropsy or by brain biopsy.




Prof James Ironside, a neuropathologist from the National Creutzfeldt-Jakob Disease Surveillance Unit, Edinburgh, UK, says "the position we are in just now is suspicion, as far as I am aware, and it is not proven that this child has the disease". In occasional cases of iatrogenic CJD occurring in younger patients during pregnancy there has been no evidence of maternal transmission, adds Ironside.




Chris Verity, a paediatric neurologist from Addenbrooke's Hospital, Cambridge, UK, who is part of a national paediatric vCJD surveillance team, stresses the need for caution before drawing conclusions from this case. "We cannot comment on individual cases . . . and there are a whole lot of reasons why children may have problems with development".




Haroon Ashraf








so, what about sutures ???


Other US BSE risks: the imported products picture 24 Jul 00 Trade Statistics: UK to US Compiled by Terry S. Singeltary Sr of Bacliff, Texas


[Opinion (webmaster): The US has focused for years on tracing, containing, and eradicating live animal imports from the UK or other countries with acknowledged BSE like Belgium, including some 499 cattle and the Vermont sheep. This strategy does not acknowledge imports of rendered bovine products from England during the BSE period nor secondary products such as surgical catgut, which is to say surgical cowgut, or dairy cattle embryos, vaccines for veterinarian and human medicines. What has become of these?


Mr. Singeltary, who lost his mother to CJD of unexplained origin a few years back and went on to became a well-known TSE activist, has tracked down voluminous pertinent import data through correspondence with UK officials and searches of government web sites. Imports of such products are frequently cited by Europeans in rating BSE risks in the US and in shutting out US exports.


Many people's eyes glaze over when reviewing reams of sometimes older trade statistics. There is no proof that any of the imported products was contaminated with BSE nor if so, any evidence that any BSE product lead to infection in US livestock, surgical patients, or what not. Nonetheless, the data obtained by Mr. Singeltary establish that an appalling variety and tonnage of products that were imported by the US from the UK and othr BSE-affected countries during the peak of the BSE epidemic years.


10 January 1990 COMMERCIAL IN CONFIDENCE


NOT FOR PUBLICATION


COMMITTEE ON SAFETY OF MEDICINES WORKING PARTY ON BOVINE SPONGIFORM ENCEPHALOPATHY


SURGICAL CATGUT SUTURES 2.1 At the first meeting of the Working Party on Bovine Spongiform Encephalopathy on 6 September 1989, detailed consideration was given to XXXXX Surgical Catgut. This arose from the Company's response to the Letter to Licence Holders, indicating that the bovine small intestine source material was derived from UK cattle, unlike 8 other licenced catgut sutures. In contrast XXXXX Surgical Catgut was stated to hold over 90% share of the market for catgut sutures, and to constitute approximately 83% of all sutures used in U.K. IMPORTS OF SUTURES FROM THE KNOWN BSE COUNTRY;









see full text ;








Tuesday, February 8, 2011


U.S.A. 50 STATE BSE MAD COW CONFERENCE CALL Jan. 9, 2001







http://cjdtexas.blogspot.com/

TSS
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