Rare Diseases Symptoms Automatic Extraction
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[Retroperitoneal lymphangioleiomyomatosis - a case reports].
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
rare
progressive
disease
affecting
women
of
childbearing
age
.
The
disease
is
characterised
by
an
abnormal
proliferation
of
immature
smooth
muscle
cells
predominantly
in
the
lung
.
It
gradually
leads
to
respiratory
failure
,
and
it
frequently
result
in
death
.
Extrapulmonary
LAM
typically
presents
with
abdominal
mass
,
abdominal
pain
and
chylous
ascites
.
In
the
case
reports
we
describe
two
cases
of
premenopausal
females
with
extrapulmonary
LAM
.
In
both
cases
they
occur
in
pelvic
location
in
the
obturator
fossa
and
around
the
external
iliac
artery
.
After
surgical
procedures
patients
were
primary
treated
with
progesterone
.
Sirolimus
was
second
-line
drugs
.
Diseases
Validation
Diseases presenting
"rare progressive disease"
symptom
lymphangioleiomyomatosis
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