Evidence of
in utero transmission of classical scrapie in sheep
John
Spiropoulos⇑, Stephen A.C. Hawkins, Marion M. Simmons and Susan J. Bellworthy
+ Author
Affiliations Animal Health and Veterinary Laboratories Agency (AHVLA) Weybridge,
Addlestone, Surrey KT15 3NB, UK
ABSTRACT
Classical
scrapie is one of the Transmissible Spongiform Encephalopathies (TSE), a group
of fatal infectious diseases that affect the central nervous system (CNS).
Classical scrapie can transmit laterally from ewe to lamb perinatally, or
between adult animals. Here we report detection of infectivity in tissues of an
unborn foetus, providing evidence that in utero transmission of classical
scrapie is also possible.
FOOTNOTES
Corresponding Author: John Spiropoulos: Department of Pathology, Animal Health
and Veterinary Laboratories Agency, Woodham Lane, New Haw, Addlestone, Surrey,
KT15 3NB, Email: john.spiropoulos@ahvla.gsi.gov.uk, Tel: +44 (0) 1932 357795,
Fax: +44 (0) 1932 357805 Copyright © 2014, American Society for Microbiology.
All Rights Reserved.
Tuesday,
September 17, 2013
Mother to
Offspring Transmission of Transmissible Spongiform Encephalopathy TSE prion
disease snip... Maternal CWD infection also appears to result in lower
percentage of live birth offspring. In addition, evolving evidence from protein
misfolding cyclic amplification (PMCA) assays on fetal tissues suggest that
covert prion infection occurs in utero. Overall, our findings demonstrate that
transmission of prions from mother to offspring can occur, and may be
underestimated for all prion diseases.
snip...
Here, in an
experimental model of CWD, we have demonstrated the transmission of infectious
prions from clinical and subclinical mothers to full-term viable, nonviable and
in utero harvested offspring, revealing that the transmission of TSEs from
mother to offspring can occur and may be underestimated for all prion diseases.
snip... please see full text ; Tuesday, September 17, 2013
*** Mother
to Offspring Transmission of Transmissible Spongiform Encephalopathy TSE prion
disease ***
Friday, May
10, 2013
Evidence of
effective scrapie transmission via colostrum and milk in sheep
Tuesday,
April 30, 2013
Transmission of
classical scrapie via goat milk
Veterinary
Record2013;172:455 doi:10.1136/vr.f2613
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.
===========================
PPo3tss-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.
=========================
PPo3tss-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
Friday,
December 23, 2011
Detection
of PrPres in Genetically Susceptible Fetuses from Sheep with Natural Scrapie
http://transmissiblespongiformencephalopathy.blogspot.com/2011/12/detection-of-prpres-in-genetically.html
Monday, November 22, 2010
Monday, November 22, 2010
SHEEP WITH
MASTITIS TRANSMIT INFECTIOUS PRIONS THROUGH THE MILK
Saturday,
April 12, 2008
Evidence of
scrapie transmission via milk
[6] Date:
Fri 4 Feb 2005
From: Terry
S. Singeltary Sr. flounder@wt.net
Source:
Spongiform Encephalopathy Advisory Committee (SEAC), Position Paper, January
2005 [edited]
Position
Statement: Maternal Transmission of variant Creutzfeldt-Jakob disease Issue:
1. The
Chief Medical Officer for England asked SEAC to consider current evidence and
comment on the potential transmission of variant Creutzfeldt-Jakob disease
(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 BSE [A joint FSA/SEAC milk working group is monitoring and
providing advice on this research carried out at the Veterinary Laboratories
Agency.] 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. 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 were 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 [a chronic,
progressive, uniformly fatal nervous system disorder caused by prions,
associated with cannibalism among the Fore tribe and neighboring peoples in New
Guinea. - CopyEd.PG]. 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.
9. A
published study suggesting transmission of sCJD in colostrum (ref. 1) 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 gene (ref. 2), 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 children (ref. 3) 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.
References:
(1) Tamai Y et al. Demonstration of the transmissible agent in tissue from a
pregnant woman with CJD. New Eng J Med 1992 327, 649. (2) Wadsworth et al. Human
prion protein with valine 129 prevents expression of variant CJD phenotype.
Science. 2004 306, 1793-1796. (3) Devereux G et al. Variations in
neurodegenerative disease across the UK: findings from the national study of
Progressive Intellectual and Neurological Deterioration (PIND). Arch DisChild.
2004 89, 8-12. -- Terry S. Singeltary Sr. flounder@wt.net ******
P.4.31
Prion
infectivity in milk from ARQ/ARQ sheep experimentally infected with Scrapie and
MAEDI-VISNA virus
Ciriaco
Ligios1, Maria Giovanna Cancedda1, Antonello Carta2, Cinzia Santucciu1 Caterina
Maestrale1, Francesca Demontis1, Sonia Attene1, Maria Giovanna Tilocca1,
Cristiana Patta1, Massimo Basagni5, Paola Melis1, James C. De- Martini3,
Christina Sigurdson4 1Istituto Zooprofilattico Sperimentale della Sardegna,
Italy; 2Research Unit: Genetics and Biotechnology, DIRPA, AGRIS Sardinia, Italy;
3Department of Microbiology, Immunology, and Pathology, Colorado State
University, Fort Collins, CO, USA; 4Department of Pathology, School of Medicine,
University of California San Diego, USA; 5Prion Diagnostica Rho, Italy
Background:
Scrapie in
sheep is characterized by the deposition of misfolded and aggregated prion
protein (PrPSc) in the central nervous system (CNS) and within the
lymphoreticular system (LRS). PrPSc was shown to accumulate in organs beyond the
CNS and the LRS when lymphofollicular or granulomatous inflammation was also
present.
Objectives:
Our aim was
to determine whether ectopic PrPSc accumulation in the inflamed mammary gland of
sheep with scrapie results in infectious prion secretion into the milk.
Methods:
We fed
approximately 1.1 - 2.1 L of milk from sheep with lymphofollicular mastitis and
clinical scrapie to each of 8 ARQ/ARQ lambs derived from scrapie-free flocks.
The milk donor sheep had been previously inoculated with Maedi-Visna virus (MVV)
intratracheally and intravenously and scrapie brain homogenate orally. In
addition, 3 ARQ/ARQ lambs were fed approximately 1.4 – 1.7 L of milk from
ARQ/ARQ sheep that had been experimentally infected with only scrapie.
Additional control ARQ/ARQ lambs were inoculated with scrapie brain homogenate
only, or with milk from uninfected sheep.
Results:
Two lambs
which had received milk from sheep with mastitis and scrapie developed clinical
signs of scrapie at 677 and 745 days post-inoculation. One additional clinically
healthy lamb from this group, which was sacrificed for a cause unrelated to
scrapie, was found to have PrPSc in brain and tonsil. The control lambs and
those which received milk from sheep affected only with scrapie are, to date,
clinically healthy.
Discussion:
This is the
first evidence of clinical scrapie in sheep fed milk from scrapie sick sheep.
The experiment is ongoing, however these preliminary results indicate that milk
and/or colostrum from ARQ/ARQ sheep with clinical scrapie and lymphofollicular
mastitis could contribute to scrapie transmission.
Monday,
August 03, 2009
Prions Are
Secreted in Milk from Clinically Normal Scrapie-Exposed Sheep
Journal of
Virology, August 2009, p. 8293-8296, Vol. 83, No. 16 0022-538X/09/$08.00+0
doi:10.1128/JVI.00051-09 Copyright © 2009, American Society for Microbiology.
All Rights Reserved.
TAFS
INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a
non-profit Swiss Foundation (January, 2009) TAFS1 STATEMENT ON TRANSMISSION OF
SCRAPIE VIA MILK
Prions in
Milk from Ewes Incubating Natural Scrapie
ProMED-mail
Archive
Number 20050211.0467 Published
Date
11-FEB-2005 Subject PRO/AH/EDR> CJD (new var.) update 2005 (02)
snip...
******
[3] Date:
Thu 20 Jan 2005 From: Terry S. Singeltary Sr.
Chronic
Lymphocytic Inflammation Specifies the Organ Tropism of Prions
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.
****** [5]
Date: Fri 21 Jan 2005 From: ProMED-mail 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]
Position
Statement: Maternal Transmission of variant Creutzfeldt-Jakob disease
-----------------------------------------------
Issue:
1. The
Chief Medical Officer for England asked SEAC to consider current evidence and
comment on the potential transmission of variant Creutzfeldt-Jakob disease
(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 BSE [A joint FSA/SEAC milk working group is monitoring and
providing advice on this research carried out at the Veterinary Laboratories
Agency.] 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.
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 were 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 [a chronic,
progressive, uniformly fatal nervous system disorder caused by prions,
associated with cannibalism among the Fore tribe and neighboring peoples in New
Guinea. - CopyEd.PG]. 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.
9. A
published study suggesting transmission of sCJD in colostrum (ref. 1) 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 gene (ref. 2), 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 children (ref. 3) 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.
References:
(1) Tamai Y
et al. Demonstration of the transmissible agent in tissue from a pregnant woman
with CJD. New Eng J Med 1992 327, 649.
(2)
Wadsworth et al. Human prion protein with valine 129 prevents expression of
variant CJD phenotype. Science. 2004 306, 1793-1796.
(3)
Devereux G et al. Variations in neurodegenerative disease across the UK:
findings from the national study of Progressive Intellectual and Neurological
Deterioration (PIND). Arch DisChild. 2004 89, 8-12.
-- Terry S.
Singeltary Sr.
******
snip...
ProMED-mail
promed@promedmail.org
******[6]Date: Fri 4 Feb 2005
From: Terry S. Singeltary Sr.
Source: Spongiform Encephalopathy Advisory Committee
(SEAC), Position Paper, January 2005 [edited]
snip...
******[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 ;
p.s. ProMed archives are pay-per-view now. ...tss
SEE FULL TEXT ;
p.s. ProMed archives are pay-per-view now. ...tss
SNIP...SEE FULL TEXT ;
cjd mother to child transmission ???
Mother
passes on CJD to unborn baby Sun, 17 Sep 2000 Telegraph By Rajeev Syal, Jenny
Booth and Chris Hastings
Dr Will
offers me a tour of the laboratories. As we are getting up to go, I broach
something that has been bothering me. Does he think the victims will get any
younger?
'Well, we
now have a 12-year-old.'
A
12-year-old?
'That's
what I said.' He looks almost ashamed.
Girl or
boy?
'I can't
say.'
But if the
incubation period is at least 10 years, then the child was barely eating solid
food when it contracted the infection. 'I'm not saying anything,' the
neurologist says wearily. 'You've got small children of your own, Allison. You
do the maths.'
The child
has been ill since she was born but tests to pinpoint the cause of the problem
have so far proved inconclusive INCONCLUSIVE. What does not put an end to a
thing. Inconclusive presumptions are those which may be overcome by opposing
proof; for example, the law presumes that he who possesses personal property is
the owner of it, but evidence is allowed to contradict this presumption, and
show who is . At birth the baby, who cannot be named for legal reasons, could
not swallow and was unable to gain weight.
Wednesday,
December 30, 2009
Is there
evidence of vertical transmission of variant CJD ?
J Neurol
Neurosurg Psychiatry doi:10.1136/jnnp.2009.172148
Is there
evidence of vertical transmission of variant CJD?
Katy Murray
(kmurray12@doctors.org.uk) + Author Affiliations
NationalCJD
Surveillance Unit, United Kingdom James Peters (jimmypeters1980@yahoo.co.uk) +
Author Affiliations
NationalCJD
Surveillance Unit, United Kingdom Lesley Stellitano
(lesley.stellitano@addenbrookes.nhs.uk) + Author Affiliations
Addenbrooke's
Hospital, United Kingdom Annemarie Winstone
(annemarie.winstone@addenbrookes.nhs.uk) + Author Affiliations
Addenbrooke's
Hospital, United Kingdom Christopher Verity
(christopher.verity@addenbrookes.nhs.uk) + Author Affiliations
Addenbrooke's
Hospital, United Kingdom Robert Will (r.g.will@ed.ac.uk) + Author
Affiliations
NationalCJD
Surveillance Unit, United Kingdom Published Online First 27 April 2009 Abstract
Objectives: The possibility of vertical transmission of variant CJD (vCJD) has
been raised, because of the widespread distribution of infectivity in vCJD and
the demonstration that this condition can be transmitted through blood
transfusion. The aim is to search for evidence of this type of transmission of
vCJD.
Methods: A
national surveillance system for CJD has been established in the UK since 1990.
Through this register details were extracted of all children born to vCJD cases
up to March 2009. This list was checked against the CJD register and cases
identified through the UK study of progressive intellectual and neurological
deterioration in children (PIND) to determine whether any of the children of
vCJD cases had themselves developed a progressive neurological disorder or
vCJD.
Results:
125 children have been born to parents with a diagnosis of vCJD. Nine of these
children were born to females with vCJD who were symptomatic at conception,
birth or within a year of clinical onset. Only one woman was known to have
breast fed her child. None of the children of vCJD cases have been referred to
the NCJDSU as suspected vCJD and none have been classified as suffering from a
progressive neurodegenerative disorder through the PIND study. One of the
children has been investigated by the National Prion Unit (see accompanying case
report).
Interpretation:
To date there is no evidence of vertical transmission of vCJD. However, the
incubation period through this mechanism might be prolonged and it will be many
years before observational data can exclude this possibility.
snip...see
more here ;
Wednesday,
December 30, 2009
Is there evidence of vertical transmission of variant CJD ?
http://creutzfeldt-jakob-disease.blogspot.com/2009/12/is-there-evidence-of-vertical.html
Thursday, January 23, 2014
Is there evidence of vertical transmission of variant CJD ?
http://creutzfeldt-jakob-disease.blogspot.com/2009/12/is-there-evidence-of-vertical.html
Thursday, January 23, 2014
Medical
Devices Containing Materials Derived from Animal Sources (Except for In Vitro
Diagnostic Devices) [Docket No. FDA–2013–D–1574]
2013
Tuesday, September 17, 2013
*** Mother
to Offspring Transmission of Transmissible Spongiform Encephalopathy TSE prion
disease ***
To date,
125 children have been born to women who later developed CJD [11]. This is
concerning because PrPCJD has been detected in the fetal and pregnancy related
tissues of a woman infected with CJD [12]. Although decades may pass before the
onset of clinical effects associated with such transmission due to a long
subclinical carrier state, the probability that these individuals harbor
infectious prions remains high.
Sunday,
August 25, 2013
Prion2013
Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and
mother to offspring transmission
snip...
Oral.08:
Mother to offspring transmission of chronic wasting disease in Reeve's Muntjac
deer Amy Nalls,1 Erin McNulty,1 Jenny Powers,2 Davis Seelig,1 Clare Hoover,1
Nicholas Haley,1 Jeanette Hayes-Klug,1 Kelly Anderson,1 Paula Stewart,3 Wilfred
Goldmann,3 Edward A. Hoover1 and Candace K. Mathiason1 1Colorado State
University; Fort Collins, CO USA; 2National Park Service; Fort Collins, CO USA;
3The Roslin Institute and Royal School of Veterinary Studies; Edinburgh, UK To
investigate the role mother to offspring transmission plays in chronic wasting
disease (CWD), we have developed a cervid model employing the Reeve's muntjac
deer (Muntiacus reevesi). Eight muntjac doe were orally inoculated with CWD and
tested PrPCWD lymphoid positive by 4 mo post infection. Fourteen fawns were born
to these eight CWD-infected doe-3 were born viable, 6 were born non-viable and 5
were harvested as fetuses from early or end-stage CWD-infected doe. All three
viable fawns have demonstrated CWD IHC lymphoid biopsy positivity between 43 d
post birth and 11 mo post birth. Two of these three CWD positive viable
offspring have developed clinical signs consistent with TSE disease (28-33 mo
post birth). Moreover, CWD prions have been detected by sPMCA in 11 of 16
tissues harvested from 6 full-term non-viable fawns and in 7 of 11 fetal tissues
harvested in utero from the second and third trimester fetuses. Additional
tissues and pregnancy related fluids from doe and offspring are being analyzed
for CWD prions. In summary, using the muntjac deer model we have demonstrated
CWD clinical disease in offspring born to CWD-infected doe, and in utero
transmission of CWD from mother to offspring. These studies provide basis to
further investigate the mechanisms of maternal transfer of prions.
snip...
Sunday,
August 25, 2013
Prion2013
Chronic Wasting Disease CWD risk factors, humans, domestic cats, blood, and
mother to offspring transmission
>>>
Here, in an experimental model of CWD, we have demonstrated the transmission of
infectious prions from clinical and subclinical mothers to full-term viable,
nonviable and in utero harvested offspring, revealing that the transmission of
TSEs from mother to offspring can occur and may be underestimated for all prion
diseases. <<<
2014
Sunday,
January 19, 2014
National
Prion Disease Pathology Surveillance Center Cases Examined1 as of January 8,
2014
Thursday,
January 23, 2014
Medical
Devices Containing Materials Derived from Animal Sources (Except for In Vitro
Diagnostic Devices) [Docket No. FDA–2013–D–1574]
Terry S.
Singeltary Sr.
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