Rare Diseases Symptoms Automatic Extraction
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Cardiovascular outcomes in Fabry disease are linked to severity of chronic kidney disease.
[fabry disease]
Assess
the
impact
of
end-
stage
renal
disease
(
chronic
kidney
disease
stage
5
(
CKD
5
)
)
on
cardiovascular
outcomes
in
patients
with
Fabry
disease
on
enzyme
replacement
therapy
.
Fabry
disease
,
an
X-
linked
lysosomal
storage
disease
,
causes
hypertrophic
cardiomyopathy
and
cardiovascular
dysfunction
.
Cardiac
and
renal
function
of
25
male
patients
with
Fabry
disease
were
analysed
at
0
,
1
,
2
,
5
,
7
and
10
years
after
initiation
of
treatment
.
Patients
were
grouped
at
baseline
into
those
with
CKD
5
(
n
=
10
)
and
those
without
(
n
=
15
)
.
ECG
and
echocardiography
were
performed
6
and
12
monthly
,
respectively
,
while
renal
function
was
measured
yearly
.
After
10
years
of
treatment
,
cardiac
and
renal
function
in
non-
CKD
5
patients
remained
unchanged
.
In
contrast
,
CKD
5
was
associated
with
worse
baseline
cardiac
parameters
and
progressive
LV
hypertrophy
.
LV
mass
index
grew
by
35
.
4
±
31
.
8
g
/
m
(
2
.
7
)
in
CKD
5
versus
5
.
7
±
7
.
9
g
/
m
(
2
.
7
)
,
p
=
0
.
044
in
non-
CKD
5
,
predominantly
due
to
increased
interventricular
septal
wall
thickness
(
7
.
7
±
5
.
5
mm
vs
1
.
3
±
1
.
7
mm
,
p
=
0
.
003
)
.
Cardiovascular
events
,
including
sudden
death
,
arrhythmia
and
pacing
device
insertion
,
occurred
in
100
%
patients
with
CKD
5
(
21
events
)
and
26
%
non-
CKD
5
patients
(
7
events
)
,
p
<
0
.
0001
.
Additionally
,
estimated
LV
filling
pressure
(
E
/
Ea
)
was
significantly
higher
in
patients
having
cardiovascular
events
(
21
.
1
±
7
.
7
vs
12
.
5
±
4
.
5
,
p
=
0
.
008
)
irrespective
of
renal
function
.
End-
stage
renal
disease
was
the
strongest
indicator
of
cardiovascular
disease
progression
in
Fabry
disease
.
Enzyme
replacement
initiated
prior
to
CKD
5
was
associated
with
stability
in
cardiac
and
renal
disease
while
patients
with
CKD
5
showed
ongoing
deterioration
.
Additionally
,
E
/
Ea
≥
15
may
predict
risk
of
cardiac
events
.
Diseases
Validation
Diseases presenting
"renal disease while patients with ckd5 showed ongoing deterioration"
symptom
fabry disease
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