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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
.
Diseases
Validation
Diseases presenting
"dyspnea"
symptom
acute rheumatic fever
allergic bronchopulmonary aspergillosis
carcinoma of the gallbladder
congenital diaphragmatic hernia
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
erdheim-chester disease
esophageal carcinoma
fabry disease
familial mediterranean fever
inclusion body myositis
liposarcoma
lymphangioleiomyomatosis
neuralgic amyotrophy
pleomorphic liposarcoma
primary effusion lymphoma
scrub typhus
systemic capillary leak syndrome
thoracic outlet syndrome
well-differentiated liposarcoma
This symptom has already been validated