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Evaluation of the Extent of Pulmonary Cysts and Their Association with Functional Variables and Serum Markers in Lymphangioleiomyomatosis (LAM).
[lymphangioleiomyomatosis]
Although
computed
tomography
(
CT
)
has
been
used
previously
to
assess
disease
severity
in
lymphangioleiomyomatosis
(
LAM
)
,
the
associations
between
the
extent
of
pulmonary
cysts
on
CT
and
six
-
minute
walk
test
(
6
MWT
)
,
matrix
metalloproteinases
(
MMPs
)
and
vascular
endothelial
growth
factor
(
VEGF-D
)
are
not
well
established
.
We
performed
a
cross-sectional
study
to
quantify
the
extent
of
pulmonary
cysts
in
CT
and
to
establish
their
correlations
with
pulmonary
function
tests
(
PFTs
)
,
6
MWT
results
,
including
a
composite
index
(
desaturation-distance
ratio
,
DDR
)
,
and
levels
of
VEGF-D
and
MMPs
in
LAM
.
Twenty
-
three
LAM
patients
underwent
CT
scanning
to
automatically
quantify
the
extent
of
pulmonary
cysts
and
performed
PFTs
and
6
MWT
.
Serum
levels
of
MMP-
2
,
MMP-
9
,
and
VEGF-D
were
also
measured
.
The
severity
of
pulmonary
cystic
involvement
was
mild
(
the
extent
of
cysts
was
6
.
8
%
)
and
correlated
best
with
FEV
1
/
FVC
(
r
=
-
0
.
84
)
,
residual
volume
(
r
=
0
.
66
)
,
DLCO
(
r
=
-
0
.
82
)
,
the
DDR
index
(
r
=
0
.
77
)
,
and
desaturation
during
the
6
MWT
(
r
=
-
0
.
81
)
.
There
was
a
weak
correlation
with
VEGF-D
(
r
=
0
.
45
)
,
but
no
association
was
found
with
MMP-
2
and
MMP-
9
.
The
severity
of
pulmonary
cystic
involvement
was
mild
in
this
sample
of
LAM
patients
and
correlated
best
with
airway
obstruction
,
air
trapping
,
reduced
DLCO
,
the
DDR
index
,
and
desaturation
during
the
6
MWT
.
Serum
VEGF-D
can
not
be
completely
defined
as
a
valuable
marker
of
disease
severity
and
there
may
be
a
mechanism
independent
of
MMPs
to
explain
the
formation
of
pulmonary
cysts
.
Diseases
Validation
Diseases presenting
"vascular endothelial growth factor"
symptom
adrenal incidentaloma
aniridia
coats disease
dedifferentiated liposarcoma
esophageal squamous cell carcinoma
hodgkin lymphoma, classical
inclusion body myositis
krabbe disease
liposarcoma
lymphangioleiomyomatosis
pleomorphic liposarcoma
primary effusion lymphoma
severe combined immunodeficiency
systemic capillary leak syndrome
von hippel-lindau disease
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