From: TSS (216-119-143-35.ipset23.wt.net)
Subject: Advancing Prion Science: Guidance for the National Prion Research
Date: September 1, 2004 at 11:13 am PST
Advancing Prion Science: Guidance for the National Prion Research
Human TSEs in the United States are underrecognized and underreferred
for definitive diagnosis, the committee found.
The most effective strategy for managind the threat of TSEs is to avoid
preventable exposure to the infectious agent, the committee concluded.
U.S. mesures to prevent the tissue of cattle infected with the agent of BSE
from entering the food chain are not foolproof, however, the committee
SURVEILLANCE FOR TSEs IN THE UNITED STATES
Surveillance for TSEs in humans and animals can permit the detection of
outbreaks of known or new TSEs and the monitoring of those TSEs known to
occur in the United States: CWD and scrapie in animals, and sCJD,
iatrogenic CJD (iCJD), familial CJD, familial fatal insomnia, sporadic
fatal insomnia, and Gerstmann-Straussler-Scheinker disease in humans.
Surveillance for TSEs in Humans The Centers for Disease Control and
Prevention (CDC) monitors the U.S. population for human TSEs. It also
conducts surveillance specifically for vCJD (Belay et al., 2003), even
though there has never been a reported case of BSE in the United States.
Human TSEs are reportable in 12 states. Identification and Autopsy of
TSE Cases The diagnosis of TSE in humans almost always requires a
neuropathological examination. These examinations are conducted for the
U.S. population by the National Prion Disease Pathology Surveillance
Center (NPDPSC). Yet at least half of the estimated total number of
deaths caused by TSE in the United States are not autopsied and
confirmed by laboratory examination (Gambetti, 2002; Holman et al.,
1996. The committee concluded that the large percentage of undiagnosed
TSE cases represents a major obstacle to thorough U.S. surveillance for
SUMMARY 25 The committee determined that the U.S. military is at an
increased risk for acquiring vCJD as a result of its deployment to Europe.