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Clinical course and characterisation of lymphangioleiomyomatosis in a Brazilian reference centre.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
rare
disease
that
promotes
pulmonary
cystic
destruction
and
impairs
pulmonary
function
.
We
aim
to
describe
features
and
clinical
course
of
LAM
patients
from
Brazil
.
We
described
the
clinical
and
functional
features
,
performance
in
six
minute
walk
test
(
6
MWT
)
,
management
details
,
survival
and
clinical
course
of
84
LAM
patients
followed
in
a
Brazilian
reference
centre
.
All
subjects
were
women
,
the
average
age
at
onset
of
symptoms
was
38
years
,
and
the
average
at
diagnosis
was
42
years
.
The
major
symptoms
during
the
course
of
the
disease
were
dyspnoea
and
pneumothorax
.
The
patients
experienced
impaired
quality
of
life
,
with
worse
scores
in
the
physical
and
emotional
domains
.
The
most
common
abnormalities
in
pulmonary
function
tests
were
an
obstructive
pattern
and
reduced
diffusion
capacity
,
whereas
a
quarter
of
the
patients
had
normal
spirometric
results
.
In
the
6
MWT
,
although
patients
had
preserved
exercise
capacity
,
more
than
half
of
the
patients
had
significant
desaturation
.
Hormonal
blockage
and
doxycycline
were
the
most
common
treatment
modalities
employed
in
our
patients
.
The
survival
probability
from
diagnosis
was
90
%
at
5
years
,
whereas
the
mean
annual
rate
of
decline
in
FEV
1
was
60
±
78
mL
.
Clinical
and
functional
features
of
the
LAM
patients
from
our
centre
are
similar
to
those
from
other
countries
.
Our
sample
showed
preserved
exercise
capacity
,
with
desaturation
in
the
6
MWT
,
and
impaired
quality
of
life
.
Survival
was
similar
,
whereas
the
annual
rate
of
decline
of
FEV
1
was
slightly
lower
than
in
recent
studies
.
Diseases
Validation
Diseases presenting
"with worse scores in the physical and emotional domains"
symptom
lymphangioleiomyomatosis
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