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[Clotting factor VIII in Sneddon syndrome].
[sneddon syndrome]
Hyperactivity
of
coagulation
factor
VIII
(
fVIII
)
marks
hypercoagulation
.
FVIII
enhances
activity
of
factor
IX
and
their
combination
activates
factor
X
,
which
is
of
primary
importance
in
prothrombin
transformation
into
thrombin
,
on
the
phospholipid
membrane
.
The
activity
of
fVIII
was
studied
in
28
patients
(
26
women
,
2
men
,
mean
age
49
.
6
+
/
-
7
.
8
years
)
with
Sneddon
's
syndrome
(
SS
)
.
SS
manifests
clinically
similarly
to
primary
antiphospholipid
syndrome
(
PAS
)
.
The
leading
of
them
are
ischemic
disorders
of
cerebral
circulation
(
IDCC
)
and
advanced
livedo
present
in
all
the
examinees
.
Hyperactivity
of
fVIII
was
registered
in
21
(
75
%
)
of
28
patients
.
Most
of
thrombosis-related
symptoms
occurred
more
frequently
in
patients
with
high
than
normal
activity
of
fVIII
:
ischemic
strokes
(
91
%
vs
57
%
,
p
>
0
.
05
)
,
repeated
strokes
(
71
%
vs
0
%
,
p
=
0
.
0014
)
,
transient
IDCC
(
76
%
vs
57
%
,
p
>
0
.
05
)
,
vascular
dementia
(
43
%
vs
0
%
,
p
>
0
.
05
)
,
ischemic
heart
disease
(
43
%
vs
0
%
,
p
>
0
.
05
)
,
thickening
of
heart
valves
according
to
echocardiography
(
91
%
vs
57
%
,
p
>
0
.
05
)
,
peripheral
venous
thromboses
(
24
%
vs
0
%
,
p
>
0
.
05
)
.
In
high
fVIII
activity
cardiolipin
antibodies
occurred
more
rarely
(
24
%
vs
43
%
,
p
>
0
.
05
)
but
lupus
anticoagulant
was
seen
more
often
(
47
%
vs
14
%
,
p
>
0
.
05
)
.
High
fVIII
activity
was
in
8
of
12
aPL-negative
patients
.
It
is
demonstrated
that
elevated
fVIII
activity
is
an
essential
mechanism
of
thrombosis
development
in
SS
.
The
cause
of
this
enhanced
activity
is
suggested
to
be
special
aPL
in
interaction
with
which
fVIII
becomes
insensitive
to
inactivation
with
protein
C
.
The
activity
of
protein
C
was
normal
in
all
the
cases
.
Diseases
Validation
Diseases presenting
"high fviii activity"
symptom
sneddon syndrome
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