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Palliative management of lymphangioleiomyomatosis: Using video-assisted thoracoscopic surgery.
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
rare
,
progressive
systemic
disease
of
unknown
etiology
,
insidious
onset
,
often
fatal
,
and
underdiagnosed
.
It
is
exclusively
found
among
women
.
LAM
mainly
involves
the
lungs
where
,
as
its
name
suggests
,
lymphatic
(
lymph
)
,
blood
vessel
(
angio
)
,
and
airways
are
surrounded
by
smooth
muscle
(
leiomyoma
)
proliferation
.
It
may
be
associated
with
tuberous
sclerosis
with
clinical
manifestations
varying
from
simple
cough
to
the
development
of
recurrent
pneumothoraces
,
haemoptysis
,
and
pleural
effusions
.
There
is
currently
no
treatment
or
cure
.
We
present
a
rare
case
of
a
41
-
year
-old
female
,
who
presented
with
recurrent
pneumothoraces
,
whose
high
-resolution
computed
tomography
findings
that
were
suggestive
of
multiple
bullous
lung
disease
,
and
the
thoracoscopic
biopsy
revealed
features
of
LAM
.
She
was
offered
video-assisted
thoracoscopic
surgery
in
form
of
bullectomy
and
pleurodesis
to
reduce
the
volume
of
abnormal
tissue
and
treated
with
progesterone
hormonal
therapy
.
The
present
case
aims
to
focus
on
clinical-radiologic
and
pathologic
findings
and
the
treatment
modalities
available
in
developing
countries
like
India
where
lung
transplantation
is
seldom
performed
.
Diseases
Validation
Diseases presenting
"tuberous sclerosis with clinical manifestations varying from simple cough to the development of recurrent pneumothoraces"
symptom
lymphangioleiomyomatosis
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