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[Lung transplantation in sporadic lymphangioleiomyomatosis: study of 7 cases].
[lymphangioleiomyomatosis]
Sporadic
lymphangioleiomyomatosis
(
S-
LAM
)
is
a
rare
disease
that
affects
only
women
.
It
is
characterized
by
an
abnormal
proliferation
of
immature
smooth
muscle
cells
(
LAM
cells
)
that
grow
in
an
aberrant
manner
in
the
airway
,
parenchymal
lung
lymph
and
blood
vessels
,
determining
the
onset
of
pulmonary
cystic
lesions
.
The
disease
has
no
treatment
,
progressing
to
respiratory
failure
,
and
lung
transplantation
(
LT
)
may
be
a
treatment
option
at
this
stage
.
Our
goal
was
to
study
7
patients
undergoing
LT
for
S-
LAM
.
We
studied
a
series
of
clinical
and
demographic
characteristics
,
diagnostic
modality
and
post-transplant
outcomes
.
We
performed
a
descriptive
analysis
of
the
series
.
The
Kaplan-
Meier
method
was
used
to
estimate
survival
.
The
mean
age
of
onset
of
symptoms
was
35
years
,
the
diagnosis
of
37
years
and
that
of
LT
38
years
.
The
most
common
symptom
was
dyspnea
.
Four
patients
had
a
history
of
pneumothorax
and
pleural
effusion
.
The
mean
forced
expiratory
volume
in
one
second
was
32
.
7
%
and
the
diffusing
capacity
for
carbon
monoxide
was
29
%
.
All
patients
were
subjected
to
LT
and
survival
was
100
,
85
.
7
and
57
.
1
%
at
one
,
3
and
5
years
,
respectively
.
Three
died
of
bronchiolitis
obliterans
and
2
necropsies
did
not
show
evidence
of
disease
recurrence
.
LT
is
a
therapeutic
option
in
patients
with
S-
LAM
with
an
advanced
respiratory
functional
impairment
.