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Recovery of chylous pulmonary congestion in tuberous sclerosis complex-associated lymphangioleiomyomatosis.
[lymphangioleiomyomatosis]
Chyloptysis
and
chylous
pulmonary
congestion
are
extremely
rare
complications
of
lymphangioleiomyomatosis
(
LAM
)
.
We
report
a
case
of
a
50
-
year
-old
woman
with
tuberous
sclerosis
complex
-associated
LAM
,
who
presented
with
expectorating
milky-
white
bronchial
casts
.
She
was
diagnosed
with
chyloptysis
and
chylous
pulmonary
congestion
by
sputum
analysis
.
Her
symptoms
and
lung
infiltration
were
improved
by
oral
sirolimus
therapy
;
moreover
,
serum
Krebs
von
den
Lungen-
6
(
KL
-
6
)
levels
paralleled
the
symptoms
and
lung
infiltration
of
these
complications
.
We
suggest
that
serum
KL
-
6
may
be
a
useful
monitoring
biomarker
of
chyloptysis
and
chylous
pulmonary
congestion
in
LAM
.
Diseases
Validation
Diseases presenting
"chyloptysis"
symptom
lymphangioleiomyomatosis
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