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Outcome of 121 patients with renal amyloid a amyloidosis.
[familial mediterranean fever]
Amyloid
A
(
AA
)
amyloidosis
is
a
multisystem
,
progressive
and
fatal
disease
.
Renal
involvement
occurs
early
in
the
course
of
AA
.
We
aimed
to
investigate
the
etiology
,
clinical
and
laboratory
features
,
and
outcome
of
patients
with
biopsy-proven
renal
AA
amyloidosis
.
A
total
of
121
patients
(
male
/
female
:
84
/
37
,
mean
age
42
.
6
±
14
.
4
years
)
were
analyzed
retrospectively
between
January
of
2001
and
May
of
2013
.
Demographic
,
clinical
and
laboratory
features
and
outcomes
data
were
obtained
from
follow-up
charts
.
Familial
Mediterranean
fever
(
37
.
2
%
)
and
tuberculosis
(
24
.
8
%
)
were
the
most
frequent
causes
of
amyloidosis
.
Mean
serum
creatinine
and
proteinuria
at
diagnosis
were
2
.
3
±
2
.
1
mg
/
dL
and
6
.
7
±
5
.
3
g
/
day
,
respectively
.
Sixty
-
eight
(
56
.
2
%
)
patients
were
started
dialysis
treatment
during
the
follow-up
period
.
Mean
duration
of
renal
survival
was
64
.
7
±
6
.
3
months
.
Age
,
serum
creatinine
and
albumin
levels
were
found
as
predictors
of
end-
stage
renal
disease
.
Fifty
patients
(
%
41
.
3
)
died
during
the
follow-up
period
.
The
mean
survival
of
patients
was
88
.
7
±
7
.
8
months
(
median
:
63
±
13
.
9
)
.
1
,
2
and
5
years
survival
rates
of
patients
were
80
.
7
%
,
68
.
2
%
and
51
.
3
%
,
respectively
.
Older
age
,
male
gender
,
lower
levels
of
body
mass
index
,
estimated
glomerular
filtration
rate
,
serum
albumin
,
calcium
,
and
higher
levels
of
phosphor
,
intact
parathyroid
hormone
and
proteinuria
were
associated
with
a
higher
mortality
.
Higher
serum
creatinine
,
lower
albumin
,
dialysis
requirement
and
short
time
to
dialysis
were
predictors
of
mortality
.
The
outcome
of
patients
with
AA
amyloidosis
and
renal
involvement
is
poor
,
particularly
in
those
who
had
massive
proteinuria
,
severe
hypoalbuminemia
and
dialysis
requirement
at
the
outset
.
Diseases
Validation
Diseases presenting
"frequent causes"
symptom
acute rheumatic fever
familial mediterranean fever
hydrocephalus with stenosis of the aqueduct of sylvius
locked-in syndrome
malignant atrophic papulosis
systemic capillary leak syndrome
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