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From: TSS ()
Subject: Biotechnology for growth hormone deficiency CJD, FRANCE
Date: February 23, 2005 at 1:56 pm PST

-------- Original Message --------
Subject: Biotechnology for growth hormone deficiency CJD, FRANCE
Date: Wed, 23 Feb 2005 15:11:02 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy
To: BSE-L@LISTSERV.KALIV.UNI-KARLSRUHE.DE


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

Biotechnology for growth hormone deficiency
Growth hormone
Philippe Cramer, MD
France Biotech
Julien Martinet, D pham
France Biotech
Text reviewed by Pr Raja Brauner (Hôpital Saint-Joseph, Paris)
and Pr Pierre Thomopoulos (Hôpital Cochin, Paris)

N O V E M B R E 2 0 0 4

snip...

TREATMENT OF GH DEFICIENCY BEFORE
BIOTECHNOLOGY
The first replacement therapies were used in the United States in the
1950s and in
Europe at the beginning of the 1970s. At that time GH was obtained by
extraction
from the pituitary glands of human cadavers. Unlike insulin (where
insulins of porcine
and bovine origin were used until the early 1980s), it is not possible
to use a
hormone of animal origin in humans because hormones are species specific.

The only indication for this treatment was pituitary dwarfism. The
growing need for
growth hormone was such that it led to the use of pituitary glands from
various
sources. In France, for instance, Bulgarian pituitaries were imported
from 1982.
Between 1983 and1988, around half of all pituitaries were imported.
The dramatic side effect of pituitary-derived GH was the appearance of
Creutzfeldt-
Jakob disease (CJD). The first reported death from CJD in a patient
undergoing
growth hormone therapy was in 1984, but it was not until two other
deaths from CJD
in 1985 that the American FDA envisaged the possibility of a correlation
between
CJD and pituitary-derived GH (see Appendix). Approximately one hundred
cases of
CJD have been reported in France.
The first response to this infection was the treatment of pituitary
extract with urea 8M
to deactivate the prions.

snip...

BIOIMPACT
CREUTZFELDT-JAKOB DISEASE (CJD)
Creutzfeldt-Jakob disease (CJD) is a rare and fatal neuro-degenerative
disease,
described for the first time in 1920, classified amongst the
transmissible spongiform
encephalopathies which can affect several species of animal.
It leads to dementia, myoclonic jerks and ataxia. The disease normally
affects adults
between 50 and 75, but all generations are concerned when transmission is
iatrogenic. Its distribution is sporadic, with an incidence of one case
per million
inhabitants. The majority of cases are sporadic (80%), 15% are of
genetic origin,
predominantly by transmission, and 5% are iatrogenic in origin (of which
more than
90% of cases are associated with pituitary-derived growth hormone). There is
currently no cure for this disease.
According to the national network monitoring CJD in France, from January
1992 to
October 2003, 8.3% of deaths from CJD originated from growth hormone
therapy (86
cases out of 1037). Out of a total of 1361 patients studied who were
treated with
batches of pituitary-derived growth hormone suspected to be tainted, the
average
incubation time observed in the 55 patients who contracted CJD was 9 to
10 years,
with a probability that 95% of cases were declared after ages 14 to 16
years. In
addition, it was observed that 80% of patients infected were homozygous
for codon
129 of gene PRNP (met-met or val-val), and this gene is therefore
considered to
indicate predisposition to CJD. These homozygous patients present an
incubation
time which is shorter than that for heterozygous individuals with the
same gene.
A new variant of this disease (nvCJD) appeared in Britain in 1996 under
the name
mad cow disease. After having studied other possibilities, scientists
have adopted
the theory of contamination of food by the agent causing bovine spongiform
encephalopathy, BSE.

snip...

http://www.france-biotech.org/FILE_DOWNLOAD.asp?ID_DOC=1292

TSS

######### https://listserv.kaliv.uni-karlsruhe.de/warc/bse-l.html ##########






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